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Symptomatic Secondary Selective IgM Immunodeficiency in Adult Man with Undiagnosed Celiac Disease

DOI: 10.1155/2012/684247

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Abstract:

Selective IgM immunodeficiency (SIgMID) is a heterogeneous disorder with no known genetic background and may occur as a primary or a secondary condition. Celiac disease has been reported in association with several humeral immunodeficiencies, including isolated severe selective IgA deficiency, panhypogammaglobulinemia, and isolated combined IgA and IgM deficiency. There are only few reported cases of pediatric and adult patients with SIgMID and celiac disease. In this paper, we describe an adult patient with a symptomatic secondary SIgMID associated with undiagnosed celiac disease, with a resolution of clinical symptoms of immunodeficiency and serum IgM normalization following a gluten-free diet. 1. Introduction Selective IgM immunodeficiency is a heterogeneous disorder with no known genetic background and may occur as a primary or a secondary condition, with a reported prevalence of 0.03% to 3% [1] Secondary SIgMID is often associated with several neoplasms or autoimmune diseases [2–5]. Primary SIgMID can be asymptomatic or present with a variety of bacterial and viral infections in the pediatric and adult populations [3]. In this paper, we describe an adult patient with a symptomatic secondary SIgMID associated with undiagnosed celiac disease, with a resolution of clinical symptoms of immunodeficiency and serum IgM normalization following a gluten-free diet. 2. Case Report A 42-year-old previously healthy man, emigrant from Russia, with an unremarkable clinical history presented in April 2011 at our Clinical Immunology Unit with predominant symptoms of fatigue for the past 3 years. He does not smoke and consume alcohol on occasion. A review of systems was significant for chronic fatigue, without deterioration in short term memory or concentration, without sleep disturbances, without weight loss or fever. Although he was able to continue working, his severe fatigue necessitated frequent time off from work, and eventually he had reduced his work commitment to part time. In September 2009, the patient was admitted into a hospital in Moscow for upper-right lobe pneumonia, and after that he frequently caught colds during 2008-2009. In 2010, the patient was admitted for pneumonia twice. These repeated episodes occurred in different lung fields. No microbiologic source of recurrent pneumonia was identified. Additionally, during 2009-2010 the patient suffered from several episodes of staphylococcus aureus associated skin infections. At presentation in our clinic (January 2011), physical examination was unremarkable. After a systematic evaluation of the

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