Letters To The Editor
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World J Gastroenterol. Dec 14, 2014; 20(46): 17686-17689
Published online Dec 14, 2014. doi: 10.3748/wjg.v20.i46.17686
Reversal of IgM deficiency following a gluten-free diet in seronegative celiac disease
Lucia Montenegro, Domenico Piscitelli, Floriana Giorgio, Claudia Covelli, Maria Grazia Fiore, Giuseppe Losurdo, Andrea Iannone, Enzo Ierardi, Alfredo Di Leo, Mariabeatrice Principi
Lucia Montenegro, Domenico Piscitelli, Floriana Giorgio, Claudia Covelli, Maria Grazia Fiore, Giuseppe Losurdo, Andrea Iannone, Enzo Ierardi, Alfredo Di Leo, Mariabeatrice Principi, Department of Emergency and Organ Transplantation, University of Bari, 70124 Bari, Italy
Alfredo Di Leo, Gastroenterology Unit, Policlinico Hospital, 70124 Bari, Italy
Author contributions: Montenegro L and Iannone A followed the patient; Piscitelli D, Covelli C and Fiore MG performed histology and immunohistochemistry; Principi M performed endoscopy, Giorgio F and Losurdo G performed molecular investigation; Ierardi E and Di Leo A collected the data and wrote the paper; all approved the final version.
Correspondence to: Alfredo Di Leo, Professor, Gastroenterology Unit, Policlinico Hospital, Piazza G Cesare 11, 70124 Bari, Italy. alfredo.dileo@uniba.it
Telephone: +39-80-5592577 Fax: + 39-80-5593088
Received: April 23, 2014
Revised: June 17, 2014
Accepted: August 13, 2014
Published online: December 14, 2014
Processing time: 238 Days and 21.3 Hours
Abstract

Selective IgM deficiency (sIGMD) is very rare; it may be associated with celiac disease (CD). We present the case of an 18-year-old man with sIGMD masking seronegative CD. Symptoms included abdominal pain, diarrhea and weight loss. Laboratory tests showed reduced IgM, DQ2-HLA and negative anti-transglutaminase. Villous atrophy and diffuse immature lymphocytes were observed at histology. Tissue transglutaminase mRNA mucosal levels showed a 6-fold increase. The patient was treated with a gluten-free diet (GFD) and six months later the symptoms had disappeared, the villous architecture was restored and mucosal tissue transglutaminase mRNA was comparable to that of healthy subjects. After 1 year of GFD, a complete restoration of normal IgM values was observed and duodenal biopsy showed a reduction of immature lymphocytes and normal appearance of mature immune cells.

Keywords: Selective IgM deficiency; Seronegative celiac disease; Tissue transglutaminase; Gluten-free diet

Core tip: Selective IgM deficiency is a rare condition, associated in 5% of cases to Celiac Disease (CD). The CD diagnosis in patients with immunoglobulin deficiencies is based only on response to a Gluten-Free Diet (GFD). Deposits of tissue transglutaminase are found in duodenal samples of seronegative CD. Our case (IgM deficiency/seronegative CD) disclosed novel insights: (1) it is possible to observe a selective association of IgM deficiency/seronegative CD; (2) the histological diagnosis of CD may be complex due to poor lymphocyte maturation; (3) mRNA coding for tissue transglutaminase in duodenal mucosa could be a useful diagnostic tool; and (4) GFD may reverse IgM deficiency by promoting lymphocyte maturation.