Case Report
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World J Clin Cases. May 16, 2014; 2(5): 160-166
Published online May 16, 2014. doi: 10.12998/wjcc.v2.i5.160
Vasculitis with renal involvement in essential mixed cryoglobulinemia: Case report and mini-review
Sabiha Anis, Khawar Abbas, Mohammad Mubarak, Ejaz Ahmed, Sajid Bhatti, Rana Muzaffar
Sabiha Anis, Khawar Abbas, Rana Muzaffar, Department of Immunology and Molecular Biology, Dewan Farooq Medical Complex, Sindh Institute of Urology and Transplantation, Karachi 74200, Pakistan
Mohammad Mubarak, Department of Histopathology, Sindh Institute of Urology and Transplantation, Karachi 74200, Pakistan
Ejaz Ahmed, Sajid Bhatti, Department of Nephrology, Sindh Institute of Urology and Transplantation, Karachi 74200, Pakistan
Author contributions: Anis S and Abbas K performed the patient follow-ups for the immunological investigations; Anis S planned and analyzed the laboratory investigations and reports; Mubarak M performed the histopathological diagnosis; Ahmed E and Bhatti S were the attending physicians and finalized the diagnosis; Anis S wrote the initial manuscript; Abbas K edited the manuscript; Anis S, Mubarak M and Muzaffar R performed critical editing of the manuscript and prepared the final draft.
Correspondence to: Sabiha Anis, MCPS, FCPS, Assistant Professor, Department of Immunology and Molecular Biology, Dewan Farooq Medical Complex, Sindh Institute of Urology and Transplantation, M-9 N-5 Link Rd, Karachi 74200, Pakistan. sabiha_anis@hotmail.com
Telephone: +92-21-32725543 Fax: +92-21-99215469
Received: November 22, 2013
Revised: February 13, 2014
Accepted: April 11, 2014
Published online: May 16, 2014
Processing time: 174 Days and 21.9 Hours
Abstract

The discovery of a strong association between hepatitis C virus (HCV) infection and mixed cryoglobulinemia (MC) has led to an increasingly rare diagnosis of idiopathic essential MC (EMC). The incidence of EMC is high in regions where there is a comparatively low HCV infection burden and low in areas of high infection prevalence, including HCV. The diagnosis of EMC requires an extensive laboratory investigation to exclude all possible causes of cryoglobulin formation. In addition, although cryoglobulin testing is simple, improper testing conditions will result in false negative results. Here, we present a 46-year-old female patient with a case of EMC with dermatological and renal manifestations, highlighting the importance of extensive investigation to reach a proper diagnosis. We review the need for appropriate laboratory testing, which is often neglected in clinical practice and which can result in false negative results. This review also emphasizes the significance of an extended testing repertoire necessary for better patient management. Despite a strong association of MC with HCV infection and other causes that lead to cryoglobulin formation, EMC remains a separate entity. Correct diagnosis requires proper temperature regulation during sample handling, as well as characterization and quantification of the cryoprecipitate. Inclusion of rheumatoid factor activity and complement levels in the cryoglobulin test-panel promotes better patient management and monitoring. Consensus guidelines should be developed and implemented for cryoglobulin detection and the diagnosis of cryoglobulinemic syndrome, which will reduce variability in inter-laboratory reporting.

Keywords: Cryoglobulin characterization; Cryoglobulin detection; Essential mixed cryoglobulinemia; Cryoglobulinemic glomerulonephritis; Hepatitis C virus; Renal manifestations

Core tip: The diagnosis of essential mixed cryoglobulinemia (EMC) requires thorough laboratory investigation to exclude all possible causes of cryoglobulin formation. Although cryoglobulin testing is simple, it requires careful temperature regulation to avoid false negative results. The testing panel should also include cryoglobulin quantification and characterization, rheumatoid factor activity and complement levels, to better facilitate patient management. Furthermore, there is a need to develop and implement consensus guidelines for laboratory and clinical diagnoses of EMC.