Does selective IgA deficiency have a good prognostic role on juvenile dermatomyositis? a case report.
Abstract
Juvenile dermatomyositis is a multisystemic autoimmune disease with uncertain etiology. Both innate immunity and adaptive immunity play a role on the pathogenesis of the disease. Selective immunoglobulin A deficiency is the most common primary immunodeficiency. Association between immunoglobulin A deficiency and autoimmune diseases including few juvenile dermatomyositis patients have been reported. A previously healthy 15-year old girl was diagnosed with juvenile dermatomyositis according to Bohan and Peter criteria and selective immunoglobulin A deficiency due to the low level of immunoglobulin A (<6 mg/dl). After 3 months of immunosuppressive treatment, her physical examination revealed no muscle weakness, no rashes, and normal muscle enzyme levels. While she has been treated with low dose methylprednisolone and subcutaneous methotrexate, muscle strength and muscle enzymes remained in normal levels at 12 months follow-up. Our knowledge about the co-occurrence of immunodeficiency and autoimmunity emerge that patients diagnosed with autoimmunity should have investigations for immunodeficiency or vice versa.
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- 1. Rider LG, Lindsley CB, Miller FW. Juvenile Dermatomyositis. In Textbook of Pediatric Rheumatology (Eds Petty RE, Laxer RM, Lindsley CB, Wedderburn L=7th ed. 351-83. Philadelphia, 2016
- 2. Mendez EP, Lipton R, Ramsey-Goldman R et al. US incidence of juvenile dermatomyositis, 1995-1998: results from the National Institute of Arthritis and Musculoskeletal and Skin Diseases registry. Arthritis Rheum 2003; 49: 300-305.
- 3. McCann LJ, Juggins AD, Maillard SM, Wedderburn LR, Davidson JE, Murray KJ et al. The Juvenile Dermatomyositis National Registry and Repository (UK and Ireland)-clinical characteristics of children recruited within the first 5 yr. Rheumatology (Oxford) 2006; 45: 1255-1260.
- 4. Yel L. Selective IgA deficiency. J Clin Immunol 2010; 30: 10-16. DOI: 10.1007/s10875-009-9357-x
- 5. Jacob CM, Pastorino AC, Fahl K, Carneiro-Sampaio M, Monteiro RC. Autoimmunity in IgA deficiency: revisiting the role of IgA as a silent housekeeper. J Clin Immunol 2008; 28 (Suppl 1): S56-61.
- 6. Meini A, Pillan NM, Villanacci V, Monafo V, Ugazio AG, Plebani A. Prevalence and diagnosis of celiac disease in IgA-deficient children. Ann Allergy Asthma Immunol 1996; 77: 333-336.
- 7. Bohan A, Peter JB. Polymyositis and dermatomyositis (second of two parts). N Engl J Med 1975; 292: 403–407.
- 8. Bastürk B, Sari S, Aral A, Dalgic B. Prevalence of selective immunoglobulin A deficiency in healthy Turkish school children. Turk J Pediatr 2011; 53: 364-368.
- 9. Aloj G, Giardino G, Valentino L, Maio F, Gallo V, Esposito T et al. Severe combined immunodeficiencies: new and old scenarios. Int Rev Immunol 2012; 31: 43-65.
- 10. Singh K, Chang C, Gershwin ME. IgA deficiency and autoimmunity. Autoimmun Rev 2014; 13: 163-177.
