Does selective IgA deficiency have a good prognostic role on juvenile dermatomyositis? a case report.

Year 2019, Volume: 44 Issue: 4, 1511 - 1514, 29.12.2019

Sibel Balcı , Rabia Miray Kışla Ekinci , Dilek Doğruel Mahir Serbes Derya Ufuk Altıntaş , Mustafa Yılmaz

https://doi.org/10.17826/cumj.523304

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.

Keywords

Autoimmunity, IgA deficiency, juvenile dermatomyositis

IgA eksikliğinin juvenil dermatomiyozit prognozuna olumlu etkisi var mıdır? olgu sunumu.

Abstract

Juvenil dermatomiyozit nedeni tam olarak bilinmeyen, birçok sistemi etkileyebilen otoimmün bir hastalıktır. Doğuştan ve kazanılmış immünitenin hastalık patogenezinde rolü bulunmaktadır. Selektif immünglobulin A eksikliği en sık görülen primer immün yetmezliktir. İmmünglobulin A eksikliği ile otoimmün hastalıkların birlikteliği daha önce birkaç juvenil dermatomiyozit tanılı olguda bildirilmiştir.  Öncesinde sağlıklı olan 15 yaşında kız hasta Bohan ve Peter kriterlerine göre juvenil dermatomiyozit ve serum immunglobulin A değeri <6 mg/dl saptandığı için selektif immünglobulin A tanısı aldı. Üç ay immünsupresif tedavi aldıktan sonra fizik muayenede raş saptanmadı, kas gücü normaldi, ayrıca kas enzimleri normal düzeylerdeydi. Düşük doz metilprednizolon ve subkutan metotreksat tedavileri alan hastanın kas gücü muayenesi ve kas enzimleri 12 aylık izlem sonrasında halen normal sınırlarda seyretmiştir. İmmün yetmezlik ve otoimmün hastalık birlikteliği hakkındaki bilgilerimiz, otoimmün hastalık veya immün yetmezlik tanılı hastaların yine bu hastalıklar açısından incelenmesini gerekli kılmaktadır. 

Keywords

Otoimmünite, IgA eksikliği, Juvenil Dermatomiyozit

References

• 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.
• 11. Woof JM, Kerr MA. The function of immunoglobulin A in immunity. J Pathol. 2006; 208: 270-282.
• 12. Lisak RP, Zweiman B. Serum immunoglobulin levels in myasthenia gravis, polymyositis, and dermatomyositis. J Neurol Neurosurg Psychiatry. 1976; 39: 34-37
• 13. Abolhassani H, Gharib B, Shahinpour S, Masoom SN, Havaei A, Mirminachi B et al. Autoimmunity in patients with selective IgA deficiency. J Investig Allergol Clin Immunol. 2015; 25: 112-119.