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1
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Czarnywojtek A, Pietrończyk K, Thompson LDR, Triantafyllou A, Florek E, Sawicka-Gutaj N, Ruchała M, Płazinska MT, Nixon IJ, Shaha AR, Zafereo M, Randolph GW, Angelos P, Al Ghuzlan A, Agaimy A, Ferlito A. IgG4-related sclerosing thyroiditis (Riedel-Struma): a review of clinicopathological features and management. Virchows Arch 2023; 483:133-144. [PMID: 37204493 PMCID: PMC10412505 DOI: 10.1007/s00428-023-03561-2] [Citation(s) in RCA: 4] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/30/2023] [Revised: 05/03/2023] [Accepted: 05/14/2023] [Indexed: 05/20/2023]
Abstract
We present a thorough review of the literature on Riedel thyroiditis (RT) with emphasis on aetiology, diagnosis and management, using the PubMed, Sinomed, and China National Knowledge Infrastructure databases. Although the exact aetiology of RT remains obscure, the histopathological features are consistent with a localized form of IgG4-related systemic disease (IgG4-RSD). Nevertheless, IgG4-RSD as a systemic fibroinflammatory disorder per se rarely affects the thyroid in the context of multiorgan manifestations. The initial diagnosis of RT is based on clinical history and imaging, but confirmation by histopathological examination is mandatory. In contrast to the historical surgical approach, glucocorticosteroid therapy is currently considered first line therapy, in line with the RT currently being viewed as a manifestation of, or analogous to, IgG4-RSD. For disease relapse, immunomodulatory agents (azathioprine, methotrexate, rituximab) can be used.
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Affiliation(s)
- Agata Czarnywojtek
- Department of Pharmacology, Poznan University of Medical Sciences, 60-806 Poznan, Poland
- Chair and Department of Endocrinology, Metabolism and Internal Medicine, Poznan University of Medical Sciences, 60-355 Poznan, Poland
| | | | | | - Asterios Triantafyllou
- Department of Pathology, Liverpool Clinical Laboratories, School of Dentistry, University of Liverpool, Liverpool, L3 5PS UK
| | - Ewa Florek
- Laboratory of Environmental Research, Department of Toxicology, Poznan University of Medical Sciences, Dojazd 30 Street, 60-631 Poznan, Poland
| | - Nadia Sawicka-Gutaj
- Chair and Department of Endocrinology, Metabolism and Internal Medicine, Poznan University of Medical Sciences, 60-355 Poznan, Poland
| | - Marek Ruchała
- Chair and Department of Endocrinology, Metabolism and Internal Medicine, Poznan University of Medical Sciences, 60-355 Poznan, Poland
| | | | - Iain J. Nixon
- Department of Otorhinolaryngology Head and Neck Surgery, NHS Lothian, Edinburgh, EH8 9YL UK
| | - Ashok R. Shaha
- Head and Neck Service, Memorial Sloan-Kettering Cancer Center, New York, NY 10065 USA
| | - Mark Zafereo
- Department of Head & Neck Surgery, MD Anderson Cancer Center, Houston, TX 77005 USA
| | - Gregory William Randolph
- Department of Otolaryngology Head and Neck Surgery, Harvard Medical School, Boston, MA 02114 USA
| | - Peter Angelos
- Section of General Surgery and Surgical Oncology, Department of Surgery, The University of Chicago, Chicago, Illinois IL 60637 USA
| | - Abir Al Ghuzlan
- Department of Biology and Pathology, Gustave Roussy Cancer Campus, University Paris-Saclay, 91190 Villejuif, France
| | - Abbas Agaimy
- Institute of Pathology, University Hospital Erlangen, Friedrich-Alexander University Erlangen-Nürnberg (FAU), 91054 Erlangen, Germany
| | - Alfio Ferlito
- Coordinator of the International Head and Neck Scientific Group, 35100 Padua, Italy
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2
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Jayachamarajapura Onkaramurthy N, Suresh SC, Theetha Kariyanna P, Jayarangaiah A, Prakash G, Raju B. IgG4 related disease and aortitis: an up-to-date review. Scand J Rheumatol 2023; 52:306-316. [PMID: 36763458 DOI: 10.1080/03009742.2022.2145744] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/11/2023]
Abstract
Aortic involvement in immunoglobulin G4-related disease (IgG4-RD) is extremely rare and is often overlooked during the aortitis work-up. IgG4-related aortitis differs from non-IgG4-related aortitis in its histopathological features, site of involvement, laboratory markers, and treatment options. The histopathological examination of the vessel walls characteristically reveals adventitial thickening with intimal sparing, typically affecting the infrarenal abdominal aorta. In addition, inadequate knowledge about the disease often leads to delayed or missed diagnosis and undermanagement of a potentially treatable condition. Hence, in this paper, we review the unique clinical manifestations, laboratory markers, diagnostic features, current treatment strategies, and novel experimental therapeutic options in the management of IgG4-related aortitis.
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Affiliation(s)
| | - S C Suresh
- Department of Internal Medicine, SUNY Downstate Medical Center, Brooklyn, NY, USA
| | - P Theetha Kariyanna
- Division of Interventional Cardiology, Marshfield Clinic Health System, Marshfield, WI, USA
| | - A Jayarangaiah
- Department of Hematology and Oncology, Prevea Cancer Center at HSHS Sacred Heart Hospital, Eau Claire, WI, USA
| | - G Prakash
- Department of Hepatobiliary Surgery, New Jersey Medical College, Newark, NJ, USA
| | - B Raju
- Department of Neurosurgery, Rutgers-Robert Wood Johnson Medical School & University Hospital, New Brunswick, NJ, USA
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3
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Vergoossen DLE, Ruiter AM, Keene KR, Niks EH, Tannemaat MR, Strijbos E, Lipka AF, van der Zijde ECJ, van Tol MJD, Bakker JA, Wevers BA, Westerberg E, Borges LS, Tong OC, Richman DP, Illa I, Punga AR, Evoli A, van der Maarel SM, Verschuuren JJ, Huijbers MG. Enrichment of serum IgG4 in MuSK myasthenia gravis patients. J Neuroimmunol 2022; 373:577978. [PMID: 36240543 DOI: 10.1016/j.jneuroim.2022.577978] [Citation(s) in RCA: 6] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/20/2022] [Revised: 09/26/2022] [Accepted: 10/05/2022] [Indexed: 11/05/2022]
Abstract
Muscle-specific kinase (MuSK) myasthenia gravis (MG) is a neuromuscular autoimmune disease belonging to a growing group of IgG4 autoimmune diseases (IgG4-AIDs), in which the majority of pathogenic autoantibodies are of the IgG4 subclass. The more prevalent form of MG with acetylcholine receptor (AChR) antibodies is caused by IgG1-3 autoantibodies. A dominant role for IgG4 in autoimmune disease is intriguing due to its anti-inflammatory characteristics. It is unclear why MuSK autoantibodies are predominantly IgG4. We hypothesized that MuSK MG patients have a general predisposition to generate IgG4 responses, therefore resulting in high levels of circulating IgG4. To investigate this, we quantified serum Ig isotypes and IgG subclasses using nephelometric and turbidimetric assays in MuSK MG and AChR MG patients not under influence of immunosuppressive treatment. Absolute serum IgG1 was increased in both MuSK and AChR MG patients compared to healthy donors. In addition, only MuSK MG patients on average had significantly increased and enriched serum IgG4. Although more MuSK MG patients had elevated serum IgG4, for most the IgG4 serum levels fell within the normal range. Correlation analyses suggest MuSK-specific antibodies do not solely explain the variation in IgG4 levels. In conclusion, although serum IgG4 levels are slightly increased, the levels do not support ubiquitous IgG4 responses in MuSK MG patients as the underlying cause of dominant IgG4 MuSK antibodies.
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Affiliation(s)
- Dana L E Vergoossen
- Department of Human Genetics, Leiden University Medical Centre LUMC, Einthovenweg 20, 2300 RC Leiden, the Netherlands
| | - Annabel M Ruiter
- Department of Neurology, Leiden University Medical Centre LUMC, Albinusdreef 2, 2333 ZA Leiden, the Netherlands
| | - Kevin R Keene
- Department of Neurology, Leiden University Medical Centre LUMC, Albinusdreef 2, 2333 ZA Leiden, the Netherlands
| | - Erik H Niks
- Department of Neurology, Leiden University Medical Centre LUMC, Albinusdreef 2, 2333 ZA Leiden, the Netherlands
| | - Martijn R Tannemaat
- Department of Neurology, Leiden University Medical Centre LUMC, Albinusdreef 2, 2333 ZA Leiden, the Netherlands
| | - Ellen Strijbos
- Department of Neurology, Leiden University Medical Centre LUMC, Albinusdreef 2, 2333 ZA Leiden, the Netherlands
| | - Alexander F Lipka
- Department of Neurology, Leiden University Medical Centre LUMC, Albinusdreef 2, 2333 ZA Leiden, the Netherlands
| | - Els C Jol van der Zijde
- Willem-Alexander Children's Hospital, Leiden University Medical Centre LUMC, Albinusdreef 2, 2333 ZA Leiden, the Netherlands
| | - Maarten J D van Tol
- Willem-Alexander Children's Hospital, Leiden University Medical Centre LUMC, Albinusdreef 2, 2333 ZA Leiden, the Netherlands
| | - Jaap A Bakker
- Department of Clinical Chemistry, Leiden University Medical Centre LUMC, Albinusdreef 2, 2333 ZA Leiden, the Netherlands
| | - Brigitte A Wevers
- Department of Clinical Chemistry, Leiden University Medical Centre LUMC, Albinusdreef 2, 2333 ZA Leiden, the Netherlands
| | - Elisabet Westerberg
- Clinical Neurophysiology, Department of Medical Sciences, Uppsala University, Akademiska sjukhuset, Ingång 85, 3rd floor, 751 85 Uppsala, Sweden
| | - Lúcia S Borges
- Department of Neurology, University of California Davis, 1515 Newton Court, Davis, CA 95618, USA
| | - Olivia C Tong
- Department of Neurology, University of California Davis, 1515 Newton Court, Davis, CA 95618, USA
| | - David P Richman
- Department of Neurology, University of California Davis, 1515 Newton Court, Davis, CA 95618, USA
| | - Isabel Illa
- Neuromuscular diseases Neurology department, Hospital Sant Pau UAB, Avenida Pare Claret N° 167, Barcelona 08025, Spain
| | - Anna Rostedt Punga
- Clinical Neurophysiology, Department of Medical Sciences, Uppsala University, Akademiska sjukhuset, Ingång 85, 3rd floor, 751 85 Uppsala, Sweden
| | - Amelia Evoli
- Department of Neurology, Università Cattolica del Sacro Cuore, Largo A. Gemelli 1, 00168 Rome, Italy
| | - Silvère M van der Maarel
- Department of Human Genetics, Leiden University Medical Centre LUMC, Einthovenweg 20, 2300 RC Leiden, the Netherlands
| | - Jan J Verschuuren
- Department of Neurology, Leiden University Medical Centre LUMC, Albinusdreef 2, 2333 ZA Leiden, the Netherlands
| | - Maartje G Huijbers
- Department of Human Genetics, Leiden University Medical Centre LUMC, Einthovenweg 20, 2300 RC Leiden, the Netherlands; Department of Neurology, Leiden University Medical Centre LUMC, Albinusdreef 2, 2333 ZA Leiden, the Netherlands.
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Lorenzon M, Spina E, Tulipano Di Franco F, Giovannini I, De Vita S, Zabotti A. Salivary Gland Ultrasound in Primary Sjögren’s Syndrome: Current and Future Perspectives. Open Access Rheumatol 2022; 14:147-160. [PMID: 36072437 PMCID: PMC9444027 DOI: 10.2147/oarrr.s284763] [Citation(s) in RCA: 16] [Impact Index Per Article: 5.3] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/17/2022] [Accepted: 08/24/2022] [Indexed: 12/12/2022] Open
Affiliation(s)
- Michele Lorenzon
- Institute of Radiology, Department of Medicine, University of Udine, Udine, Italy
- Correspondence: Michele Lorenzon, Institute of Radiology, Department of Medicine, University of Udine, Udine, Italy, Tel +39 0432 559232, Email
| | - Erica Spina
- Institute of Radiology, Department of Medicine, University of Udine, Udine, Italy
| | | | - Ivan Giovannini
- Rheumatology Clinic, Department of Medical and Biological Sciences, Azienda Sanitaria Universitaria Friuli Centrale c/o University of Udine, Udine, Italy
| | - Salvatore De Vita
- Rheumatology Clinic, Department of Medical and Biological Sciences, Azienda Sanitaria Universitaria Friuli Centrale c/o University of Udine, Udine, Italy
| | - Alen Zabotti
- Rheumatology Clinic, Department of Medical and Biological Sciences, Azienda Sanitaria Universitaria Friuli Centrale c/o University of Udine, Udine, Italy
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Koneczny I, Tzartos J, Mané-Damas M, Yilmaz V, Huijbers MG, Lazaridis K, Höftberger R, Tüzün E, Martinez-Martinez P, Tzartos S, Leypoldt F. IgG4 Autoantibodies in Organ-Specific Autoimmunopathies: Reviewing Class Switching, Antibody-Producing Cells, and Specific Immunotherapies. Front Immunol 2022; 13:834342. [PMID: 35401530 PMCID: PMC8986991 DOI: 10.3389/fimmu.2022.834342] [Citation(s) in RCA: 11] [Impact Index Per Article: 3.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/13/2021] [Accepted: 02/28/2022] [Indexed: 12/24/2022] Open
Abstract
Organ-specific autoimmunity is often characterized by autoantibodies targeting proteins expressed in the affected tissue. A subgroup of autoimmunopathies has recently emerged that is characterized by predominant autoantibodies of the IgG4 subclass (IgG4-autoimmune diseases; IgG4-AID). This group includes pemphigus vulgaris, thrombotic thrombocytopenic purpura, subtypes of autoimmune encephalitis, inflammatory neuropathies, myasthenia gravis and membranous nephropathy. Although the associated autoantibodies target specific antigens in different organs and thus cause diverse syndromes and diseases, they share surprising similarities in genetic predisposition, disease mechanisms, clinical course and response to therapies. IgG4-AID appear to be distinct from another group of rare immune diseases associated with IgG4, which are the IgG4-related diseases (IgG4-RLD), such as IgG4-related which have distinct clinical and serological properties and are not characterized by antigen-specific IgG4. Importantly, IgG4-AID differ significantly from diseases associated with IgG1 autoantibodies targeting the same organ. This may be due to the unique functional characteristics of IgG4 autoantibodies (e.g. anti-inflammatory and functionally monovalent) that affect how the antibodies cause disease, and the differential response to immunotherapies of the IgG4 producing B cells/plasmablasts. These clinical and pathophysiological clues give important insight in the immunopathogenesis of IgG4-AID. Understanding IgG4 immunobiology is a key step towards the development of novel, IgG4 specific treatments. In this review we therefore summarize current knowledge on IgG4 regulation, the relevance of class switching in the context of health and disease, describe the cellular mechanisms involved in IgG4 production and provide an overview of treatment responses in IgG4-AID.
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Affiliation(s)
- Inga Koneczny
- Division of Neuropathology and Neurochemistry, Department of Neurology, Medical University of Vienna, Vienna, Austria
| | - John Tzartos
- Neuroimmunology, Tzartos NeuroDiagnostics, Athens, Greece
- 2nd Department of Neurology, “Attikon” University Hospital, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece
| | - Marina Mané-Damas
- Research Group Neuroinflammation and Autoimmunity, Department of Psychiatry and Neuropsychology, Faculty of Health, Medicine and Life Sciences, Maastricht University, Maastricht, Netherlands
| | - Vuslat Yilmaz
- Department of Neuroscience, Aziz Sancar Institute of Experimental Medicine, Istanbul University, Istanbul, Turkey
| | - Maartje G. Huijbers
- Department of Human Genetics, Leiden University Medical Center, Leiden, Netherlands
- Department of Neurology, Leiden University Medical Center, Leiden, Netherlands
| | - Konstantinos Lazaridis
- Department of Immunology, Laboratory of Immunology, Hellenic Pasteur Institute, Athens, Greece
| | - Romana Höftberger
- Division of Neuropathology and Neurochemistry, Department of Neurology, Medical University of Vienna, Vienna, Austria
| | - Erdem Tüzün
- Department of Neuroscience, Aziz Sancar Institute of Experimental Medicine, Istanbul University, Istanbul, Turkey
| | - Pilar Martinez-Martinez
- Research Group Neuroinflammation and Autoimmunity, Department of Psychiatry and Neuropsychology, Faculty of Health, Medicine and Life Sciences, Maastricht University, Maastricht, Netherlands
| | - Socrates Tzartos
- Neuroimmunology, Tzartos NeuroDiagnostics, Athens, Greece
- Department of Neurobiology, Hellenic Pasteur Institute, Athens, Greece
| | - Frank Leypoldt
- Neuroimmunology, Institute of Clinical Chemistry and Department of Neurology, UKSH Kiel/Lübeck, Kiel University, Kiel, Germany
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6
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Vargas C, Velásquez C, Mancilla E, Aracena J. IgG4-related lung disease as a differential diagnosis of a lymphoproliferative disease: atypical presentation of an atypical disease. BMJ Case Rep 2022; 15:15/1/e247295. [PMID: 35039375 PMCID: PMC8768867 DOI: 10.1136/bcr-2021-247295] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/13/2023] Open
Abstract
IgG4-related disease (IgG4-RD) is an immunomodulated inflammatory disease that usually affects the pancreas and parotid glands. Although lung involvement is rare, it has been recently reported and could mimic various other diagnoses. We present a case of IgG4-RD whose symptoms and images raised the suspicion of a malignant lymphoproliferative lung neoplasm. It is imperative to differentiate both diseases, since their treatment and prognosis vary.
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Affiliation(s)
- Camilo Vargas
- Department of Radiology, Universidad de Antofagasta, Antofagasta, Chile
| | | | - Edgardo Mancilla
- Department of Pathology, Hospital Regional Dr Leonardo Guzmán de Antofagasta, Antofagasta, Chile
| | - Joaquín Aracena
- Department of Radiology, Universidad de Antofagasta, Antofagasta, Chile
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7
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Endmayr V, Tunc C, Ergin L, De Rosa A, Weng R, Wagner L, Yu TY, Fichtenbaum A, Perkmann T, Haslacher H, Kozakowski N, Schwaiger C, Ricken G, Hametner S, Klotz S, Dutra LA, Lechner C, de Simoni D, Poppert KN, Müller GJ, Pirker S, Pirker W, Angelovski A, Valach M, Maestri M, Guida M, Ricciardi R, Frommlet F, Sieghart D, Pinter M, Kircher K, Artacker G, Höftberger R, Koneczny I. Anti-Neuronal IgG4 Autoimmune Diseases and IgG4-Related Diseases May Not Be Part of the Same Spectrum: A Comparative Study. Front Immunol 2022; 12:785247. [PMID: 35095860 PMCID: PMC8795769 DOI: 10.3389/fimmu.2021.785247] [Citation(s) in RCA: 14] [Impact Index Per Article: 4.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/28/2021] [Accepted: 12/15/2021] [Indexed: 12/18/2022] Open
Abstract
Background IgG4 is associated with two emerging groups of rare diseases: 1) IgG4 autoimmune diseases (IgG4-AID) and 2) IgG4-related diseases (IgG4-RLD). Anti-neuronal IgG4-AID include MuSK myasthenia gravis, LGI1- and Caspr2-encephalitis and autoimmune nodo-/paranodopathies (CNTN1/Caspr1 or NF155 antibodies). IgG4-RLD is a multiorgan disease hallmarked by tissue-destructive fibrotic lesions with lymphocyte and IgG4 plasma cell infiltrates and increased serum IgG4 concentrations. It is unclear whether IgG4-AID and IgG4-RLD share relevant clinical and immunopathological features. Methods We collected and analyzed clinical, serological, and histopathological data in 50 patients with anti-neuronal IgG4-AID and 19 patients with IgG4-RLD. Results A significantly higher proportion of IgG4-RLD patients had serum IgG4 elevation when compared to IgG4-AID patients (52.63% vs. 16%, p = .004). Moreover, those IgG4-AID patients with elevated IgG4 did not meet the diagnostic criteria of IgG4-RLD, and their autoantibody titers did not correlate with their serum IgG4 concentrations. In addition, patients with IgG4-RLD were negative for anti-neuronal/neuromuscular autoantibodies and among these patients, men showed a significantly higher propensity for IgG4 elevation, when compared to women (p = .005). Last, a kidney biopsy from a patient with autoimmune paranodopathy due to CNTN1/Caspr1-complex IgG4 autoantibodies and concomitant nephrotic syndrome did not show fibrosis or IgG4+ plasma cells, which are diagnostic hallmarks of IgG4-RLD. Conclusion Our observations suggest that anti-neuronal IgG4-AID and IgG4-RLD are most likely distinct disease entities.
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Affiliation(s)
- Verena Endmayr
- Division of Neuropathology and Neurochemistry, Department of Neurology, Medical University of Vienna, Vienna, Austria
| | - Cansu Tunc
- Division of Neuropathology and Neurochemistry, Department of Neurology, Medical University of Vienna, Vienna, Austria
| | - Lara Ergin
- Division of Neuropathology and Neurochemistry, Department of Neurology, Medical University of Vienna, Vienna, Austria
| | - Anna De Rosa
- Department of Clinical and Experimental Medicine, Neurology Unit, University of Pisa, Pisa, Italy
| | - Rosa Weng
- Department of Neurology, Medical University of Vienna, Vienna, Austria
| | - Lukas Wagner
- Division of Neuropathology and Neurochemistry, Department of Neurology, Medical University of Vienna, Vienna, Austria
| | - Thin-Yau Yu
- Division of Neuropathology and Neurochemistry, Department of Neurology, Medical University of Vienna, Vienna, Austria
| | - Andreas Fichtenbaum
- Department of Laboratory Medicine, Medical University of Vienna, Vienna, Austria
| | - Thomas Perkmann
- Department of Laboratory Medicine, Medical University of Vienna, Vienna, Austria
| | - Helmuth Haslacher
- Department of Laboratory Medicine, Medical University of Vienna, Vienna, Austria
| | | | - Carmen Schwaiger
- Division of Neuropathology and Neurochemistry, Department of Neurology, Medical University of Vienna, Vienna, Austria
| | - Gerda Ricken
- Division of Neuropathology and Neurochemistry, Department of Neurology, Medical University of Vienna, Vienna, Austria
| | - Simon Hametner
- Division of Neuropathology and Neurochemistry, Department of Neurology, Medical University of Vienna, Vienna, Austria
| | - Sigrid Klotz
- Division of Neuropathology and Neurochemistry, Department of Neurology, Medical University of Vienna, Vienna, Austria
| | - Lívia Almeida Dutra
- Department of Neurology and Neurosurgery, Hospital Israelita Albert Einstein, São Paulo, Brazil
| | - Christian Lechner
- Division of Neuropathology and Neurochemistry, Department of Neurology, Medical University of Vienna, Vienna, Austria
- Pediatric Neurology, Department of Pediatric and Adolescent Medicine, Medical University of Innsbruck, Innsbruck, Austria
| | - Désirée de Simoni
- Division of Neuropathology and Neurochemistry, Department of Neurology, Medical University of Vienna, Vienna, Austria
- Department of Neurology, University Hospital St. Poelten, St. Poelten, Austria
| | - Kai-Nicolas Poppert
- Department of Neurology, Christian Doppler University Hospital, Paracelsus Medical University, Salzburg, Austria
| | - Georg Johannes Müller
- Department of Neurology and Karl Landsteiner Institute for Neuroimmunological and Neurodegenerative Disorders, Klinik Donaustadt, Vienna, Austria
| | - Susanne Pirker
- Department of Neurology, Klinik Hietzing, Vienna, Austria
| | - Walter Pirker
- Department of Neurology, Klinik Ottakring, Vienna, Austria
| | | | - Matus Valach
- Department of Pathology, Klinik Landstrasse, Vienna, Austria
| | - Michelangelo Maestri
- Department of Clinical and Experimental Medicine, Neurology Unit, University of Pisa, Pisa, Italy
| | - Melania Guida
- Department of Clinical and Experimental Medicine, Neurology Unit, University of Pisa, Pisa, Italy
| | - Roberta Ricciardi
- Department of Clinical and Experimental Medicine, Neurology Unit, University of Pisa, Pisa, Italy
| | - Florian Frommlet
- Center for Medical Statistics, Informatics and Intelligent Systems, Section for Medical Statistics, Medical University of Vienna, Vienna, Austria
| | - Daniela Sieghart
- Division of Rheumatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria
| | - Miklos Pinter
- Wiener Privatklinik – Health Center, Vienna, Austria
| | - Karl Kircher
- Department of Ophthalmology, Medical University of Vienna, Vienna, Austria
| | - Gottfried Artacker
- Department of Pediatrics and Adolescent Medicine, Klinik Donaustadt, Vienna, Austria
| | - Romana Höftberger
- Division of Neuropathology and Neurochemistry, Department of Neurology, Medical University of Vienna, Vienna, Austria
| | - Inga Koneczny
- Division of Neuropathology and Neurochemistry, Department of Neurology, Medical University of Vienna, Vienna, Austria
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Khoo V, Khoo HSJ, Goh LC. Nasolacrimal Duct Malignancy or IgG4- Related Disease? A Curious Case Report of a Nasal Vestibular Mass and Review of the Literature. Medeni Med J 2021; 36:281-286. [PMID: 34915688 PMCID: PMC8565585 DOI: 10.5222/mmj.2021.80445] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/05/2022] Open
Abstract
IgG4-related disease (IgG4-RD) is a fibro-inflammatory condition associated with tumefactive lesions at multiple sites. IgG4-RD was initially recognized in 2001 in a case of autoimmune pancreatitis. However, the disease was not limited to the pancreas but involved other organs such as the bile ducts, lacrimal glands, lymph nodes and salivary glands. IgG4-RD is rarely seen with an estimated incidence of 0.2 to 1/100.000 as reported in Japan, but with minimal to no incidence data have been published in Western countries. We hereby report a case of an IgG4-related mass arising from the nasolacrimal duct, masquerading as a sinonasal mass.
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Affiliation(s)
- Veejie Khoo
- Hospital Sultanah Aminah, Department of Otorhinolaryngology, Johor Bahru, Malaysia
| | | | - Liang Chye Goh
- Hospital Sultanah Aminah, Department of Otorhinolaryngology, Johor Bahru, Malaysia
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9
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Ramdin N, Orde M, O’Neill SB, Lai C, Pors JD, Multan M, Chen LYC, Carruthers MN. Hidden IgG4-Related Coronary Disease. Am J Clin Pathol 2021; 156:471-477. [PMID: 33738467 DOI: 10.1093/ajcp/aqaa258] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/13/2023] Open
Abstract
OBJECTIVES We present a full autopsy with a focused radiology and pathologic review of the coronary arteries. We hope that the results described in this article will help create better diagnostic measures and prevent future coronary artery vasculitis misdiagnosis. METHODS A full autopsy was performed on the body of Dr Myung Choong Yoon, with full consent from the family, within the department of pathology and laboratory medicine at Vancouver General Hospital. Tissue samples from the heart, brain, lungs, and spinal cord were submitted to specialist pathologists for histologic processing. RESULTS Cardiac gated computed tomography coronary angiography suggested periarteritis. Coexistent calcified coronary atherosclerosis with linear calcifications was present along the luminal wall, along with coronary artery ectasia. Histologic assessment confirmed features of dense adventitial fibrosis around the coronary arteries, with an exuberant lymphoplasmacytic infiltrate and numerous plasma cells consistent with IgG4-related disease. The media of the coronary arteries was markedly attenuated or completely absent, which likely contributed to the coronary arterial ectasia noted microscopically. These findings confirmed IgG4-related coronary arteritis. CONCLUSIONS Coronary periarteritis is an uncommon manifestation of IgG4-related disease established radiographically and later by autopsy.
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Affiliation(s)
| | - Matthew Orde
- Department of Pathology and Laboratory Medicine, Vancouver General Hospital and University of British Columbia, Vancouver, Canada
| | - Siobhán B O’Neill
- Department of Radiology, University of British Columbia, Vancouver, Canada
| | - Chi Lai
- Department of Pathology and Laboratory Medicine, St Paul’s Hospital and University of British Columbia, Vancouver, Canada
| | - Jennifer D Pors
- Department of Pathology and Laboratory Medicine, Vancouver General Hospital and University of British Columbia, Vancouver, Canada
- Department of Pathology, Stanford University, Palo Alto, CA, USA
| | - Michael Multan
- Department of Pathology and Laboratory Medicine, Vancouver General Hospital and University of British Columbia, Vancouver, Canada
| | - Luke Y C Chen
- Department of Medicine, Division of Hematology, University of British Columbia, Vancouver, Canada
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Zhu K, Yang J, Chen YZ, Zhang XR, Yu XH, Wang J, Zhang R, Liu C. Differences in Clinical Features and Diagnostic Strategies Between IgG4-Related Autoimmune Cholangitis and Cholangiocarcinoma. Front Oncol 2021; 11:540904. [PMID: 33816216 PMCID: PMC8012807 DOI: 10.3389/fonc.2021.540904] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/06/2020] [Accepted: 01/18/2021] [Indexed: 11/13/2022] Open
Abstract
IgG4-related autoimmune cholangitis (IgG4-AIC) is often difficult to distinguish from cholangiocarcinoma (CCA). This study aimed to determine a practical clinical strategy for distinguishing between IgG4-AIC and CCA to avoid unnecessary surgical resection. We retrospectively collected and compared the clinicopathological data between IgG4-AIC and CCA patients, including the clinical, serological, and radiological characteristics, to follow up on these patients to investigate the prognosis. Among the 377 patients who received surgical resection for suspecting CCA at the Sun Yat-Sen Memorial Hospital between June 2004 and June 2014, 14 patients were diagnosed as IgG4-AIC through histochemistry after surgery. Immunohistochemistry revealed that IgG4 was up-regulated in the plasma cells of IgG4-AIC tissues in 13 out of 14 patients. The serum CA19-9 level was significantly lower than in the CCA group. Patients with IgG4-AIC can only see slight or no enhancement under the contrast enhancement CT scan, while there are no signs of ring-like or delayed enhancement that is unique to CCA. Thirteen patients were followed up, and the time was 12 to 92 months. Three of them were regularly treated with prednisone after surgery, and original symptoms disappeared. Our study demonstrated that the combination of imaging with serum CA19-9 could improve the preoperative diagnostic value and reduce the rate of unnecessary resection.
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Affiliation(s)
- Ke Zhu
- Guangdong Provincial Key Laboratory of Malignant Tumor Epigenetics and Gene Regulation and Department of Biliary-Pancreatic Surgery, Sun Yat-sen Memorial Hospital, Sun Yat-sen University, Guangzhou, China
| | - Jin Yang
- Department of Hepatobiliary Surgery, The Affiliated Hospital, Southwest Medical University, Luzhou, China
| | - Ying-Zhen Chen
- Department of Anesthesiology, Sun Yat-sen Memorial Hospital, Sun Yat-sen University, Guangzhou, China
| | - Xue-Rong Zhang
- Department of Anesthesiology, Sun Yat-sen Memorial Hospital, Sun Yat-sen University, Guangzhou, China
| | - Xian-Huan Yu
- Guangdong Provincial Key Laboratory of Malignant Tumor Epigenetics and Gene Regulation and Department of Biliary-Pancreatic Surgery, Sun Yat-sen Memorial Hospital, Sun Yat-sen University, Guangzhou, China
| | - Jie Wang
- Guangdong Provincial Key Laboratory of Malignant Tumor Epigenetics and Gene Regulation and Department of Biliary-Pancreatic Surgery, Sun Yat-sen Memorial Hospital, Sun Yat-sen University, Guangzhou, China
| | - Rui Zhang
- Guangdong Provincial Key Laboratory of Malignant Tumor Epigenetics and Gene Regulation and Department of Biliary-Pancreatic Surgery, Sun Yat-sen Memorial Hospital, Sun Yat-sen University, Guangzhou, China
| | - Chao Liu
- Guangdong Provincial Key Laboratory of Malignant Tumor Epigenetics and Gene Regulation and Department of Biliary-Pancreatic Surgery, Sun Yat-sen Memorial Hospital, Sun Yat-sen University, Guangzhou, China
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11
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Ramachandran PV, Healy CM, Lambert EM, Guerra D, Curry CV, Vogel TP. Case Report: Hyper IgE, but Not the Usual Suspects-Kimura Disease in an Adolescent Female. Front Pediatr 2021; 9:674317. [PMID: 34354968 PMCID: PMC8329340 DOI: 10.3389/fped.2021.674317] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/01/2021] [Accepted: 06/21/2021] [Indexed: 01/13/2023] Open
Abstract
Elevated immunoglobulin E (IgE) levels can be associated with infectious, allergic and inflammatory disorders, and rarely as a manifestation of an inborn error of immunity. Here we report the case of an adolescent female who presented with a gradually enlarging neck mass, lymphadenopathy, eosinophilia and highly elevated IgE levels. Laboratory and histopathologic evaluation revealed an unlikely diagnosis of Kimura Disease. We discuss the differential diagnosis of a neck mass with prominent eosinophils on histology, and review support for T-helper type 2 (Th2) cell activation and hyper-IgE in Kimura Disease.
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Affiliation(s)
- Prasanna Venkatesh Ramachandran
- Department of Pediatrics, Baylor College of Medicine, Houston, TX, United States.,Pediatrician-Scientist Program, Baylor College of Medicine, Houston, TX, United States.,Texas Children's Hospital, Houston, TX, United States
| | - C Mary Healy
- Texas Children's Hospital, Houston, TX, United States.,Division of Infectious Disease, Department of Pediatrics, Baylor College of Medicine, Houston, TX, United States
| | - Elton M Lambert
- Texas Children's Hospital, Houston, TX, United States.,Division of Otolaryngology, Department of Pediatrics, Baylor College of Medicine, Houston, TX, United States
| | | | - Choladda V Curry
- Texas Children's Hospital, Houston, TX, United States.,Department of Pathology and Immunology, Baylor College of Medicine, Houston, TX, United States
| | - Tiphanie P Vogel
- Texas Children's Hospital, Houston, TX, United States.,Division of Rheumatology, Department of Pediatrics, Baylor College of Medicine, Houston, TX, United States
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12
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Abid H, Alaoui MEHHBE, Lamrani MYA, Figuigui M, Ahmed BC, Lahmidani N, Yousfi ME, Benajah DA, Maaroufi M, Abkari ME, Ibrahimi SA, Aqodad N. [IgG4-related disease: about 3 cases]. Pan Afr Med J 2020; 36:364. [PMID: 33235641 PMCID: PMC7666702 DOI: 10.11604/pamj.2020.36.364.24835] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/05/2020] [Accepted: 07/29/2020] [Indexed: 01/11/2023] Open
Abstract
IgG4-Related disease (IgG4-RD), formerly known as IgG4-related autoimmune polyexocrinopathy, is a new condition including Plasminogen Activator Inhibitor-1 (PAI-1). It can affect different organs (central nervous system, salivary glands, thyroid, lungs, pancreas, bile ducts, liver, digestive tract, kidneys, prostate, etc.) with symptoms depending on the organ that is affected. It is more common in men older than 50 years of age. Its incidence and prevalence are poorly known because it is an uncommon disease. It is most common in Asia, accounting for only 20-30% of PAI in the Western world. Diagnosis is based on histological examination which shows dense lymphoplasmocytic infiltration in the organ affected associated with IgG4-positive plasma cells (immunohistochemistry), organ fibrosis and obliterating venulitis, all this in the context of increased serum IgG4 levels in more than 80% of cases. Patients are sensitive to corticosteroid therapy, with a high risk of relapse after discontinuation of corticosteroid therapy. This leads to the use of immunomodulators, mainly: thiopurines (azathioprine or 6-mercaptopurine), methotrexate and more recently rituximab, which can also be used as induction therapy. Given recent advances, accurate histological and clinical criteria are currently known to limit inappropriate management such as surgery. However, knowledge gaps remain concerning: pathophysiology, identification of specific biomarkers other than IgG4, natural history of the disease and long-term cancer risk assessment, performances of diagnostic tools such as endoscopic ultrasound-guided pancreatic biopsy. As well, consensual international management should be defined in the early stages of the disease and when patients develop recurrences. The purpose of this study was to report 3 cases of IgG4-Related disease on the basis of clinical and radiological criteria as well as therapeutic response.
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Affiliation(s)
- Hakima Abid
- Service d´Hépato-Gastro-Entérologie, Centre Hospitalier Universitaire Hassan II, Fès, Maroc
| | | | - Moulay Youssef Alaoui Lamrani
- Service de Radiologie, Centre Hospitalier Universitaire Hassan II, Fès, Maroc.,Faculté de Médecine et de Pharmacie, Université Sidi Mohammed Ben Abdellah, Fès, Maroc
| | - Mouna Figuigui
- Service d´Hépato-Gastro-Entérologie, Centre Hospitalier Universitaire Hassan II, Fès, Maroc
| | - Beiba Cheikh Ahmed
- Service de Radiologie, Centre Hospitalier Universitaire Hassan II, Fès, Maroc.,Faculté de Médecine et de Pharmacie, Université Sidi Mohammed Ben Abdellah, Fès, Maroc
| | - Nada Lahmidani
- Service d´Hépato-Gastro-Entérologie, Centre Hospitalier Universitaire Hassan II, Fès, Maroc
| | - Mounia El Yousfi
- Service d´Hépato-Gastro-Entérologie, Centre Hospitalier Universitaire Hassan II, Fès, Maroc
| | - Dafr-Allah Benajah
- Service d´Hépato-Gastro-Entérologie, Centre Hospitalier Universitaire Hassan II, Fès, Maroc
| | - Mustapha Maaroufi
- Service de Radiologie, Centre Hospitalier Universitaire Hassan II, Fès, Maroc.,Faculté de Médecine et de Pharmacie, Université Sidi Mohammed Ben Abdellah, Fès, Maroc
| | - Mohammed El Abkari
- Service d´Hépato-Gastro-Entérologie, Centre Hospitalier Universitaire Hassan II, Fès, Maroc
| | - Sidi Adil Ibrahimi
- Service d´Hépato-Gastro-Entérologie, Centre Hospitalier Universitaire Hassan II, Fès, Maroc
| | - Nourdin Aqodad
- Service d´Hépato-Gastro-Entérologie, Centre Hospitalier Universitaire Ibn Zohr, Agadir, Maroc
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13
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Park BJ, Starks R, Kirby P, Menezes AH, Dlouhy BJ. IgG4-Related Disease of the Craniovertebral Junction. World Neurosurg 2020; 134:264-271. [DOI: 10.1016/j.wneu.2019.10.195] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/20/2019] [Revised: 10/30/2019] [Accepted: 10/31/2019] [Indexed: 12/28/2022]
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IgG4-related disease: rare presentation as a soft-tissue mass in the thigh of an adolescent. Skeletal Radiol 2020; 49:155-160. [PMID: 31165193 DOI: 10.1007/s00256-019-03250-9] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/04/2019] [Revised: 05/05/2019] [Accepted: 05/16/2019] [Indexed: 02/02/2023]
Abstract
We report a case of a 16-year-old boy who presented a soft-tissue mass in the anterior compartment of the right thigh discovered by positron emission tomography/computed tomography within the work-up of unexplained prolonged inflammatory syndrome. The mass has no calcification. Subsequently, magnetic resonance imaging of the femoral triangle was carried out. Axial short tau inversion recovery images showed a 3.5-cm ill-defined mass in the femoral triangle with focal areas of hypointensity, which suggests that there might be fibrosis or hemosiderin within the tumor. Axial T1-weighted images showed a slight hyperintense mass involving the iliopsoas muscle. Contrast-enhanced fat-suppressed T1-weighted imaging showed a heterogeneous solid enhancement. Adjacent thick fascia enhancement of the vastus intermedius and the vastus lateralis muscles extending from the mass as a tail-like margin suggested the infiltrative spread of the tumor along the fascial plane. The mass and the lymphadenopathy were excised. Immunohistochemically, tumor cells were staining for muscle actin and desmin. Many plasma cells were IgG4+ (175per high-power field) with a ratio IgG4+/IgG+ of 50%. The diagnosis of IgG4-related disease (IgG4-RD) was made. Although a diffuse array of musculoskeletal symptoms has been observed in IgG4-related disease, reports of biopsy-proven musculoskeletal involvement of the limb are rare. We showed the radiological features of IgG4-RD presenting as a soft-tissue mass of the thigh. Musculoskeletal involvement, clinical significance, and treatment of IgG4-RD are also discussed.
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15
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Ando W, Yukioka F, Yamamoto K, Koyama T, Hashimoto Y, Yasui Y, Ogawa T, Tsuda T, Ohzono K. Immunoglobulin G4-Related Disease of the Hip. Orthopedics 2018; 41:e876-e879. [PMID: 30125037 DOI: 10.3928/01477447-20180815-06] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/11/2017] [Accepted: 02/09/2018] [Indexed: 02/03/2023]
Abstract
The authors report a case of immunoglobulin G4-related disease (IgG4-RD) of the hip. A 60-year-old man diagnosed with osteoarthritis of the right hip was referred to the authors' outpatient clinic for surgical intervention. Laboratory test results revealed elevated C-reactive protein and serum IgG levels. A subsequent laboratory test revealed an IgG4 level of 318 mg/dL. Magnetic resonance imaging revealed an abnormal mass in the right hip joint. The authors suspected IgG4-RD of the hip. The mass was resected during total hip arthroplasty. Immunohistochemical analysis for IgG revealed positive staining of many plasma cells. Most of the IgG-positive plasma cells were positive for IgG4, and the ratio of IgG4/IgG-positive cells was 51%. This case met all criteria for IgG4-RD; thus, the authors made a definitive diagnosis of IgG4-RD of the hip. The C-reactive protein level decreased to a negative value, and the IgG level decreased to a normal range at 3 weeks postoperatively. The IgG4 level gradually decreased to 152 mg/dL at 5 months postoperatively. This is the first reported case of IgG4-RD of the hip joint. [Orthopedics. 2018; 41(6):e876-e879.].
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16
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Sulieman I, Mahfouz A, AlKuwari E, Szabados L, Elmoghazy W, Elaffandi A, Khalaf H. IgG4-related disease mimicking pancreatic cancer: Case report and review of the literature. Int J Surg Case Rep 2018; 50:100-105. [PMID: 30096533 PMCID: PMC6082998 DOI: 10.1016/j.ijscr.2018.07.030] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/12/2018] [Accepted: 07/26/2018] [Indexed: 12/12/2022] Open
Abstract
Pancreatic masses pose a diagnostic challenge, and cancer has to be always considered. IgG4-related disease is a rare cause of pancreatic masses. Biopsy from the pancreas is not always required if histopathology from a more accessible peripheral site lesion confirms the diagnosis. Multiorgan involvement and aortitis should raise suspicion of IgG4-related disease. Introduction Most patients with pancreatic masses pose a diagnostic challenge when a benign lesion is suspected, and often, resection is needed before a benign diagnosis is confirmed. Presentation of case A 57 years old male patient presented with a pancreatic head mass, obstructive jaundice and submandibular lymph node enlargement. He also had a history of recurrent eye pain and redness, skin lesions, and benign prostatic hypertrophy. MRI showed a pancreatic head mass with double duct sign, aortic thickening, bilateral renal lesions, diffuse lymph node enlargement, and prostatic enlargement. FDG-PET/CT demonstrated abnormal uptake corresponding to the MRI lesions, and there were elevated IgG4 levels on blood investigations. Biopsy of an inguinal lymph node revealed infiltrates with IgG4 plasma cells, consistent with the diagnosis of IgG4 disease. The patient was treated with IV steroids and showed significant improvement. Discussion IgG4 related disease is a rare entity that is characterized by lesions that show heavy infiltration with IgG4 positive plasma cells, storiform fibrosis, and obliterative phlebitis. The pancreas is the most commonly involved organ, but several other organ systems are involved, and this helps in clinical suspicion of the diagnosis. A biopsy from any easily accessible site that shows the characteristic histological features is sufficient for diagnosis. Patients respond quickly to steroids, but recurrence is frequent. Conclusion IgG4 related disease is a rare cause of pancreatic tumorous lesions that need a high index of suspicion for diagnosis and should be differentiated from pancreatic neoplastic lesions.
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Affiliation(s)
- Ibnouf Sulieman
- Department of Surgery, Division of Organ Transplant, Hamad General Hospital, Doha, PO Box 3050, Qatar.
| | - Ahmed Mahfouz
- Department of Radiology, Hamad General Hospital, Doha, PO Box 3050, Qatar.
| | - Einas AlKuwari
- Department of Pathology, Hamad General Hospital, Doha, PO Box 3050, Qatar.
| | - Lajos Szabados
- Department of Nuclear Medicine, Hamad General Hospital, Doha, PO Box 3050, Qatar.
| | - Walid Elmoghazy
- Department of Surgery, Division of Organ Transplant, Hamad General Hospital, Doha, PO Box 3050, Qatar.
| | - Ahmed Elaffandi
- Department of Surgery, Division of Organ Transplant, Hamad General Hospital, Doha, PO Box 3050, Qatar.
| | - Hatem Khalaf
- Department of Surgery, Division of Organ Transplant, Hamad General Hospital, Doha, PO Box 3050, Qatar.
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17
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Cousin E, Cousin I, Aziz K, Chailloux P, Breton E. Autoimmune Pancreatitis and Ulcerative Rectocolitis in an Adolescent. Pediatrics 2018; 141:S456-S461. [PMID: 29610171 DOI: 10.1542/peds.2016-0765] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 05/31/2017] [Indexed: 11/24/2022] Open
Abstract
Autoimmune pancreatitis (AIP) is rare in teenagers and difficult to diagnose. There are no clear and established diagnostic criteria in the pediatric population to distinguish subtype 1 and subtype 2. Here, we report the case of a 16-year-old white French teenager admitted to the pediatric emergency service with more than 1 year's history of pain originating from the epigastric and the right hypochondriac regions, with bloody diarrhea. After exclusion of pancreatic cancer and other common causes of acute pancreatitis, the diagnosis of AIP was suspected. Biological analyses revealed acute pancreatitis with severe cholestasis and an elevated level of serum immunoglobulin G4. Magnetic resonance cholangiography revealed a voluminous pancreas presenting a typical "sausage-like" aspect. Anatomopathological analyses of the liver biopsy specimen revealed a biliary obstruction due to pancreatic involvement without the typical aspect of chronic destructive cholangitis. Corticotherapy and immunosuppressive treatment proved effective after 1 week of treatment. Without a pancreatic biopsy specimen, the distinction between AIP type 1 and 2 could not be made clearly in this case. The succession of clinical observations could allow clinicians to recognize, treat, and manage AIP in children.
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Affiliation(s)
| | - Ianis Cousin
- Department of Pediatric Surgery, Brest University Hospital, Brest, France
| | - Karim Aziz
- Gastroenterology, Saint-Brieuc Hospital, Saint-Brieuc, France; and
| | - Pascal Chailloux
- Gastroenterology, Saint-Brieuc Hospital, Saint-Brieuc, France; and
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18
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Bridges KJ, DeDeaux CH, Than KD. IgG4-related disease presenting as intradural extramedullary lesion: a case report and review of the literature. Br J Neurosurg 2017; 33:570-576. [PMID: 28958166 DOI: 10.1080/02688697.2017.1384793] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/24/2022]
Abstract
Object: IgG4-related disease (IgG4-RD) is a fibro-inflammatory disorder affecting various anatomical sites, and only recently was identified to affect the dura of the spine. The authors present the second reported case of an intradural extramedullary lesion consistent with IgG4-related spinal disease. Methods: A literature review was performed that identified 15 other cases of spinal disease, and common features of all known reported spinal IgG4-RD are discussed. Results: Spinal IgG4-RD typically affects males of approximately 50 years of age, and often presents as a T1 and T2 hypo- or isointense lesion that homogenously enhances. Surgical intervention typically involves subtotal resection or biopsy, and histopathologic findings include increased IgG4-positive cells or an IgG4:IgG ratio >40%. The disease responds well to steroids early on, and treatment can include adjuvant therapy such as azathioprine. Conclusions: Systemic involvement is possible, and, early treatment can quickly minimize disease burden. Thus, increased suspicion would result in early diagnosis and improved prognosis.
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Affiliation(s)
- Kelly J Bridges
- Department of Neurological Surgery, Oregon Health & Science University , Portland , OR , USA
| | - Caitlin H DeDeaux
- Department of Neurological Surgery, Oregon Health & Science University , Portland , OR , USA
| | - Khoi D Than
- Department of Neurological Surgery, Oregon Health & Science University , Portland , OR , USA
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19
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Chang CC, Chang YS, Wang SH, Lin SY, Chen YH, Chen JH. Primary Sjogren's syndrome and the risk of acute pancreatitis: a nationwide cohort study. BMJ Open 2017; 7:e014807. [PMID: 28801391 PMCID: PMC5724124 DOI: 10.1136/bmjopen-2016-014807] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/25/2022] Open
Abstract
OBJECTIVE Studies on the risk of acute pancreatitis in patients with primary Sjogren's syndrome (pSS) are limited. We evaluated the effects of pSS on the risk of acute pancreatitis in a nationwide, population-based cohort in Taiwan. STUDY DESIGN Population-based retrospective cohort study. SETTING We studied the claims data of the >97% Taiwan population from 2002 to 2012. PARTICIPANTS We identified 9468 patients with pSS by using the catastrophic illness registry of the National Health Insurance Database in Taiwan. We also selected 37 872 controls that were randomly frequency matched by age (in 5 year bands), sex and index year from the general population. PRIMARY OUTCOME MEASURE We analysed the risk of acute pancreatitis by using Cox proportional hazards regression models including sex, age and comorbidities. RESULTS From 23.74 million people in the cohort, 9468 patients with pSS (87% women, mean age=55.6 years) and 37 872 controls were followed-up for 4.64 and 4.74 years, respectively. A total of 44 cases of acute pancreatitis were identified in the pSS cohort versus 105 cases in the non-pSS cohort. Multivariate Cox regression analysis indicated that the incidence rate of acute pancreatitis was significantly higher in the pSS cohort than in the non-pSS cohort (adjusted HR (aHR) 1.48, 95% CI 1.03 to 2.12). Cyclophosphamide use increased the risk of acute pancreatitis (aHR 5.27, 95% CI 1.16 to 23.86). By contrast, hydroxychloroquine reduced the risk of acute pancreatitis (aHR 0.23, 95% CI 0.09 to 0.55). CONCLUSION This nationwide, retrospective cohort study demonstrated that the risk of acute pancreatitis was significantly higher in patients with pSS than in the general population.
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Affiliation(s)
- Chi-Ching Chang
- Division of Rheumatology, Immunology and Allergy, Department of Internal Medicine, Taipei Medical University Hospital, Taipei, Taiwan
- Division of Allergy, Immunology and Rheumatology, Department of Internal Medicine, School of Medicine, College of Medicine, Taipei Medical University, Taipei, Taiwan
| | - Yu-Sheng Chang
- Division of Allergy, Immunology and Rheumatology, Department of Internal Medicine, School of Medicine, College of Medicine, Taipei Medical University, Taipei, Taiwan
- Division of Allergy, Immunology and Rheumatology, Department of Internal Medicine, Shuang Ho Hospital, Taipei Medical University, New Taipei City, Taiwan
| | - Shu-Hung Wang
- Division of Allergy, Immunology and Rheumatology, Department of Internal Medicine, Shuang Ho Hospital, Taipei Medical University, New Taipei City, Taiwan
| | - Shyr-Yi Lin
- Department of Primary Care Medicine, Taipei Medical University Hospital, Taipei, Taiwan
- Department of General Medicine, School of Medicine, College of Medicine, Taipei Medical University, Taipei, Taiwan
| | - Yi-Hsuan Chen
- Biostatistics Centre College of Management, Taipei Medical University, Taipei, Taiwan
| | - Jin Hua Chen
- Biostatistics Centre College of Management, Taipei Medical University, Taipei, Taiwan
- Graduate Institute of Data Science, College of Management, Taipei Medical University, Taipei, Taiwan
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20
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Huart J, Grosch S, Bovy C, Moutschen M, Krzesinski JM. IgG4-related membranous glomerulonephritis and generalized lymphadenopathy without pancreatitis: a case report. BMC Nephrol 2017; 18:139. [PMID: 28446143 PMCID: PMC5405476 DOI: 10.1186/s12882-017-0561-2] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/16/2016] [Accepted: 04/20/2017] [Indexed: 12/24/2022] Open
Abstract
Background IgG4-related disease is a recently described pathologic entity. This is the case of a patient with nephrotic syndrome and lymphadenopathy due to IgG4-related disease. Such a kidney involvement is quite peculiar and has only been described a few times recently. Renal biopsy showed a glomerular involvement with membranous glomerulonephritis in association with a tubulo-interstitial nephropathy. Moreover, the patient was not suffering from pancreatitis. Case presentation The patient is a middle-aged man of Moroccan origin. He has developed recurrent episodes of diffuse lymphadenopathies, renal failure and nephrotic syndrome. Renal biopsies showed membranous glomerulonephritis. Discussion and conclusion The diagnostic approach of this atypical presentation is discussed in this case report as well as diagnostic criteria, therapeutic strategies, biomarkers and pathophysiology of IgG4-related disease. IgG4-related membranous glomerulonephritis is a well-established cause of membranous glomerulonephritis. It must be sought after in every patient with a previous diagnosis of IgG4-related disease and in every patient with this histological finding on renal biopsy. Corticoids are still the first-line treatment of IgG4-related disease. New therapeutic strategies are needed to avoid glucocorticoids long term side-effects. Interestingly, the patient was prescribed cyclophosphamide in addition to glucocorticoids for an immune thrombocytopenia. This treatment had a very good impact on his IgG4-related disease.
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Affiliation(s)
- Justine Huart
- Division of Nephrology, University of Liège Hospital (ULg CHU), Liège, Belgium.
| | - Stéphanie Grosch
- Division of Nephrology, University of Liège Hospital (ULg CHU), Liège, Belgium
| | - Christophe Bovy
- Division of Nephrology, University of Liège Hospital (ULg CHU), Liège, Belgium
| | - Michel Moutschen
- Division of Infectious diseases and General Internal Medecine, University of Liège Hospital (ULg CHU), Liège, Belgium
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Lee LIT, Gillibrand R, Mukerjee D, Kaneria SS. Isolated pulmonary IgG4-related disease mimicking lung malignancy. BJR Case Rep 2017; 3:20160134. [PMID: 30363259 PMCID: PMC6159196 DOI: 10.1259/bjrcr.20160134] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/25/2016] [Revised: 01/29/2017] [Accepted: 03/02/2017] [Indexed: 12/24/2022] Open
Abstract
Immunoglobulin G4 (IgG4)-related disease is a relatively rare and only recently recognized immune-mediated fibro-inflammatory condition that is commonly associated with autoimmune pancreatitis. Reports have further been characterized in almost all other organ systems, with several lung-related IgG4 disease reports emerging over the past decade. IgG4-related disease affects more than one organ in 60–90% of patients. To this date, there have been few published cases of pathologically proven isolated IgG4-related lung disease (IgG4-RLD), where no other organ is affected. We report an isolated pulmonary case of IgG4-RLD in a 65-year-old female with clinical and radiological manifestations suggestive of primary lung malignancy. CT revealed multiple sub-solid ground glass opacities, several of which were part-solid, others were pure ground glass. Histological analysis revealed IgG4 disease with no evidence of neoplasia. Serum IgG4 levels were elevated (206 mg dl–1). Malignancy was ruled out and the patient was treated with corticosteroids, though there was no change in CT appearance over 16 months. The CT imaging pattern in our case is atypical from previous literature characterisation.
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Affiliation(s)
- Louise I T Lee
- Department of Radiology, North Middlesex University Hospital NHS Trust, England, UK
| | - Rebecca Gillibrand
- Department of Radiology, North Middlesex University Hospital NHS Trust, England, UK
| | - Dev Mukerjee
- Department of Radiology, North Middlesex University Hospital NHS Trust, England, UK
| | - Sashin S Kaneria
- Department of Radiology, North Middlesex University Hospital NHS Trust, England, UK
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22
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Liu C, Zhang H, Yao G, Hu Y, Qi J, Wang Y, Chen W, Tang X, Li W, Lu L, Gu L, Sun L. Characteristics of primary Sjögren’s syndrome patients with IgG4 positive plasma cells infiltration in the labial salivary glands. Clin Rheumatol 2016; 36:83-88. [DOI: 10.1007/s10067-016-3472-x] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/29/2016] [Revised: 09/14/2016] [Accepted: 10/27/2016] [Indexed: 12/24/2022]
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Stamatopoulos A, Patrini D, Koletsis E, Borg E, Khiroya R, Hayward M, Lawrence D, Panagiotopoulos N. IgG4 related lung disease extending to the thoracic vertebrae. Respir Med Case Rep 2016; 19:162-165. [PMID: 27766198 PMCID: PMC5066297 DOI: 10.1016/j.rmcr.2016.10.008] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/28/2016] [Revised: 09/25/2016] [Accepted: 10/06/2016] [Indexed: 12/11/2022] Open
Abstract
IgG4-related disease (IgG4-RD) is a fibroinflammatory condition that can affect practically every organ. Although it was first identified in pancreas and salivary glands, major organs like liver, biliary tree, kidney, thyroid glands and lungs are commonly involved, sometimes resulting in organ failure. We describe a case of an 41-year-old man presented with back pain after a rotator cuff injury. A Computed Tomography (CT) revealed incidentally a right lower lobe paravertebral lesion extending across the T5 and T6 vertebral levels and invading into the adjacent pleural surface. The laboratory findings and the CT guided biopsy were inconclusive. Morphological and immunohistochemical findings after a lung biopsy by video-assisted thoracic surgery (VATS) were suggestive to IgG4-related lung disease (IgG4-RLD), which was confirmed with high serum levels of IgG4. This represents the first case of a IgG4-RLD lesion located in the mediastinum and extending to the adjacent pleural surface and vertebrae and should be included in the differential diagnosis of posterior mediastinal masses.
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Affiliation(s)
| | - Davide Patrini
- Department of Thoracic Surgery, University College London Hospitals (UCLH), London, UK
| | - Efstratios Koletsis
- Department of Thoracic Surgery, University College London Hospitals (UCLH), London, UK
| | - Elaine Borg
- Department of Pathology, University College London Hospitals (UCLH), London, UK
| | - Reena Khiroya
- Department of Pathology, University College London Hospitals (UCLH), London, UK
| | - Martin Hayward
- Department of Thoracic Surgery, University College London Hospitals (UCLH), London, UK
| | - David Lawrence
- Department of Thoracic Surgery, University College London Hospitals (UCLH), London, UK
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24
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IgG4-related disease presenting as hypertrophic pachymeningitis and compressive optic neuropathy. Joint Bone Spine 2016; 83:601-2. [DOI: 10.1016/j.jbspin.2015.07.016] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/02/2015] [Accepted: 07/24/2015] [Indexed: 01/15/2023]
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25
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Cornec D, Saraux A, Jousse-Joulin S, Pers JO, Boisramé-Gastrin S, Renaudineau Y, Gauvin Y, Roguedas-Contios AM, Genestet S, Chastaing M, Cochener B, Devauchelle-Pensec V. The Differential Diagnosis of Dry Eyes, Dry Mouth, and Parotidomegaly: A Comprehensive Review. Clin Rev Allergy Immunol 2016; 49:278-87. [PMID: 24952023 DOI: 10.1007/s12016-014-8431-1] [Citation(s) in RCA: 39] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/25/2022]
Abstract
Primary Sjögren's syndrome (pSS) is a frequent autoimmune systemic disease, clinically characterized by eyes and mouth dryness in all patients, salivary gland swelling or extraglandular systemic manifestations in half of the patients, and development of lymphoma in 5 to 10 % of the patients. However, patients presenting with sicca symptoms or salivary gland swelling may have a variety of conditions that may require very different investigations, treatments, or follow-up. Eye and/or mouth dryness is a frequent complaint in clinical setting, and its frequency increases with age. When evaluating a patient with suspected pSS, the first step is to rule out its differential diagnoses, before looking for positive arguments for the disease. Knowledge of normal and abnormal lachrymal and salivary gland physiology allows the clinician to prescribe the most adapted procedures for evaluating their function and structure. New tests have been developed in recent years for evaluating these patients, notably new ocular surface staining scores or salivary gland ultrasonography. We describe the different diagnoses performed in our monocentric cohort of 240 patients with suspected pSS. The most frequent diagnoses are pSS, other systemic autoimmune diseases, idiopathic sicca syndrome and drug-induced sicca syndrome. However, other diseases are important to rule out due to their specific management, such as sarcoidosis, granulomatosis with polyangeitis, IgG4-related disease, chronic hepatitis C virus or human immunodeficiency virus infections, graft-versus-host disease, and head and neck radiation therapy. At the light of these data, we propose a core of minimal investigations to be performed when evaluating a patient with suspected pSS.
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Affiliation(s)
- Divi Cornec
- Department of Rheumatology, Brest Teaching Hospital, Brest, France.,EA2216, INSERM ESPRI, ERI29, Université de Brest, Brest, France.,LabEx IGO, Brest, France
| | - Alain Saraux
- Department of Rheumatology, Brest Teaching Hospital, Brest, France.,EA2216, INSERM ESPRI, ERI29, Université de Brest, Brest, France.,LabEx IGO, Brest, France
| | - Sandrine Jousse-Joulin
- Department of Rheumatology, Brest Teaching Hospital, Brest, France.,EA2216, INSERM ESPRI, ERI29, Université de Brest, Brest, France.,LabEx IGO, Brest, France
| | - Jacques-Olivier Pers
- EA2216, INSERM ESPRI, ERI29, Université de Brest, Brest, France.,LabEx IGO, Brest, France.,Department of Odontology, Brest Teaching Hospital, Brest, France
| | | | - Yves Renaudineau
- EA2216, INSERM ESPRI, ERI29, Université de Brest, Brest, France.,LabEx IGO, Brest, France.,Department of Odontology, Brest Teaching Hospital, Brest, France.,Laboratory of Immunology, Brest Teaching Hospital, Brest, France
| | - Yves Gauvin
- Department of Ear, Nose, Throat, Brest Teaching Hospital, Brest, France
| | | | - Steeve Genestet
- Department of Neurological Functional Explorations, Brest Teaching Hospital, Brest, France
| | - Myriam Chastaing
- Department of Psychiatry, Brest Teaching Hospital, Brest, France
| | | | - Valérie Devauchelle-Pensec
- Department of Rheumatology, Brest Teaching Hospital, Brest, France. .,EA2216, INSERM ESPRI, ERI29, Université de Brest, Brest, France. .,LabEx IGO, Brest, France. .,Service de Rhumatologie, Hôpital de la Cavale Blanche, BP 824, 29609, Brest Cedex, France.
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Gajewska ME, Rychwicka-Kielek BA, Sørensen K, Kubik M, Hilberg O, Bendstrup E. Immunoglobulin G4-related pleuritis - A case report. Respir Med Case Rep 2016; 19:18-20. [PMID: 27408782 PMCID: PMC4925619 DOI: 10.1016/j.rmcr.2016.05.006] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/20/2016] [Accepted: 05/23/2016] [Indexed: 12/11/2022] Open
Abstract
Immunoglobulin G4-related disease (IgG4-RD) is a chronic disease that presents with inflammation and fibrosis of involved tissue. It encompasses several disorders previously described using different terms, but all disorders are characterised by IgG4-positive plasma cells and lymphocytes infiltration of tissues. We report a rare case of a 58-year-old man with IgG4-related pleural disease without other systemic manifestations. The diagnosis was based on characteristic changes on PET-CT and typical histopathology in a pleural specimen. The patient’s condition improved following immunosuppressive therapy.
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Affiliation(s)
- Marta E Gajewska
- Dept. of Respiratory Diseases and Allergy, Aarhus University Hospital, Nørrebrogade 44, 8000 Aarhus C, Denmark
| | | | - Kathina Sørensen
- Dept. Pathology, Aalborg University Hospital, Ladegårdsgade 3, 9000 Aalborg, Denmark
| | - Magdalena Kubik
- Dept. of Nuclear Medicine, Aalborg University Hospital, Hobrovej 18-22, 9000 Aalborg, Denmark
| | - Ole Hilberg
- Dept. of Respiratory Diseases and Allergy, Aarhus University Hospital, Nørrebrogade 44, 8000 Aarhus C, Denmark
| | - Elisabeth Bendstrup
- Dept. of Respiratory Diseases and Allergy, Aarhus University Hospital, Nørrebrogade 44, 8000 Aarhus C, Denmark
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Li B, Hou J, Zhang Y, Hou X, Shi H. Retroperitoneal IgG4-related sclerosing disease mimics renal pelvic cancer on 18 F-FDG PET/CT. Rev Esp Med Nucl Imagen Mol 2016. [DOI: 10.1016/j.remnie.2015.12.006] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/22/2022]
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28
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Sarkar A, Pitchumoni CS. The protean manifestations of IgG4-RD in gastrointestinal disorders. Dis Mon 2015; 61:493-515. [DOI: 10.1016/j.disamonth.2015.09.008] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/15/2022]
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29
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Retroperitoneal IgG4-related sclerosing disease mimics renal pelvic cancer on (18)F-FDG PET/CT. Rev Esp Med Nucl Imagen Mol 2015; 35:67-9. [PMID: 26324752 DOI: 10.1016/j.remn.2015.06.001] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/11/2015] [Accepted: 06/29/2015] [Indexed: 02/07/2023]
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30
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Liang L, Zhou J, Chen L. Perifollicular granulomas with IgG4 plasmacytosis: A case report and review of literature. World J Clin Cases 2015; 3:650-654. [PMID: 26244157 PMCID: PMC4517340 DOI: 10.12998/wjcc.v3.i7.650] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/26/2015] [Revised: 04/13/2015] [Accepted: 05/18/2015] [Indexed: 02/05/2023] Open
Abstract
Perifollicular granuloma is a unique histologic feature and whether it is associated with immunoglobulin G4 (IgG4)-related disease is controversial. We report a case of a 38-year-old man who presented with worsening left eye pain, proptosis, tearing, gritty sensation, blurred vision and multiple lymphadenopathy. An axillary lymph node resection showed reactive follicular and interfollicular lymph node hyperplasia, and increased eosinophils and plasma cells (at least 80% of IgG+ plasma cells were positive for IgG4). A distinct feature was the presence of multifocal, perifollicular histiocytic granulomas, which formed a wreath around the entire follicles. The human herpes virus 8 was not detected by immunohistochemistry. In addition, an extensive panel of special stains, immunohistochemistry, and flow cytometry was negative for lymphoma, fungal, or mycobacterial infection. The findings were suggestive of IgG4-related sclerosing disease-associated lymphadenopathy. Further laboratory testing showed a significant increase of serum immunoglobulin E (> 23000 IU/mL) and slight increase of total IgG, but normal serum IgG4. Even though perifollicular granuloma is a nonspecific histopathologic feature and can be seen in other diseases, such as nodular lymphocyte predominant Hodgkin lymphoma, IgG4-related lymphadenopathy should be listed in the differential diagnoses of benign reactive lymph nodes, especially when perifollicular granuloma and plasmacytosis coexist.
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Abstract
IgG4-related disease (IgG4-RD) is a recently recognized systemic disease characterized by tumefactive lesions in various organ systems. The list of organs that can be involved continues to expand, and recently computed tomography (CT) descriptions of the pulmonary lesions found in the disease have been described. The clinical symptoms are nonspecific and may include cough, dyspnea, chest pain, and fever. The appropriate clinical presentation along with elevated serum IgG4 concentrations and pathologic evidence of lymphoplasmacytic infiltrates with abundant IgG4-positive plasma cells and storiform fibrosis is consistent with the disease. Steroids are used to treat this disease in addition to immunosupressives such as cyclosporine or rituxumab for steroid refractory disease. The pulmonary manifestations and imaging features can often mimic malignancy, and as such knowledge of the diagnostic, clinicopathologic, and radiographic features of the disease is required in order to provide appropriate diagnostic workup and treatment.
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32
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Immunoglobulin G4-related disease mimicking lymphoma in a Chinese patient. Rheumatol Int 2015; 35:1749-52. [PMID: 25800963 DOI: 10.1007/s00296-015-3259-4] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/19/2015] [Accepted: 03/16/2015] [Indexed: 12/13/2022]
Abstract
Immunoglobulin G4-related disease (IgG4-RD) is a systemic disorder characterized by multiorgan fibrosis with IgG4-producing plasma cells, increased IgG4 serum concentration, and responsiveness to steroid therapy. IgG4-RD tends to form tumefactive lesions. As a result, patients are often suspected of having a malignancy such as lymphoma. In this article, a patient with IgG4-RD and the deep vein thrombosis who was initially suspected of lymphoma is reported. The 63-year-old man presented with painless salivary swelling and multi-lymphadenopathy, progressively swelling and pain in the left leg. Salivary biopsy showed IgG4+ plasma cells >50 per high-power field and IgG4+/IgG+ plasma cell ratio >40 %. The serum IgG4 level was 4.28 g/L (range 0.03-2.01 mg/dL). Ultrasonography showed that the inferior vena cava was partially occluded, and thrombosis in the left iliac vein. Computed tomography scan revealed plaque-like tissue surrounding the inferior vena cava and abdominal aortic, which is typical for the diagnosis of retroperitoneal fibrosis. The patient was effectively treated with corticosteroids, interventional therapy, and anticoagulant therapy which resulted in a reduction in the swelling of the lymph nodes and left leg. Patient with IgG4-RD and deep vein thrombosis is rare and could be misdiagnosed easily as malignant disease. Accurate diagnosis is critical for disease management.
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Tomiyama F, Watanabe R, Fujii H, Kamogawa Y, Fujita Y, Shirota Y, Nakamichi T, Sato H, Ishii T, Harigae H. Synovitis in a Patient with IgG4-related Disease. Intern Med 2015; 54:1427-32. [PMID: 26028001 DOI: 10.2169/internalmedicine.54.4320] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/06/2022] Open
Abstract
A 71-year-old man was admitted to our department due to arthralgia and renal dysfunction. A physical examination disclosed swelling of the right shoulder and left wrist joints. Laboratory tests showed elevated serum IgG4 and creatinine levels, and magnetic resonance imaging of the wrist revealed bone erosion and synovitis. In addition, fluorodeoxyglucose positron emission tomography showed uptake in the submandibular glands, pancreas, kidneys, and affected joints and a renal biopsy revealed tubulointerstitial nephritis with the infiltration of IgG4+ plasma cells. The patient was subsequently diagnosed with IgG4-related disease (IgG4-RD) and successfully treated with corticosteroid therapy. This case suggests that erosive arthritis may occur in patients with IgG4-RD.
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Affiliation(s)
- Fumiko Tomiyama
- Department of Hematology and Rheumatology, Tohoku University Graduate School of Medicine, Japan
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Couderc M, Mathieu S, Tournadre A, Dubost JJ, Soubrier M. Acute ocular myositis occurring under etanercept for rheumatoid arthritis. Joint Bone Spine 2014; 81:445-6. [DOI: 10.1016/j.jbspin.2014.03.003] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/07/2014] [Accepted: 03/11/2014] [Indexed: 12/30/2022]
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35
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Mulay K, Honavar SG. Orbital Immunoglobulin G4-Related Disease: A Systematic Review. Asia Pac J Ophthalmol (Phila) 2014; 3:322-5. [PMID: 26107919 DOI: 10.1097/apo.0000000000000077] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/24/2022] Open
Abstract
Immunoglobulin G4-related disease (IgG4-RD) is a clinically distinct systemic condition that can involve the orbital tissue. Characterized by a triad of a mass-forming lesion, infiltration by IgG4-positive plasma cells, and elevated serum IgG4 titers in many cases, IgG4-RD has clinicopathologic features that overlap with ocular adnexal lymphomas and orbital inflammatory conditions. Although most cases of orbital IgG4-RD respond well to steroids, it may become necessary to include supplemental immunosuppressant therapy in the management.
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Affiliation(s)
- Kaustubh Mulay
- From the *National Reporting Centre for Ophthalmic Pathology (NRCOP) and †Ophthalmic and Facial Plastic Surgery and Ocular Oncology, Centre for Sight, Hyderabad, India
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