SESSION TITLE: ILD Case Report Posters II
SESSION TYPE: Case Report Poster
PRESENTED ON: Sunday, March 23, 2014 at 01:15 PM - 02:15 PM
INTRODUCTION: The Antisynthetase syndrome is a rare entity, its comprises of a triad of inflammatory myopathy, diffuse interstitial lung disease, and small joint involvement. Serum auto antibodies to aminoacyl transfer RNA synthetases (anti-ARS) especially anti Jo-1 antibody present in most of the cases. Interstitial lung disease is an important diagnostic criteria and a major determinant of mortality of this syndrome. We are reporting a case of ASS
CASE PRESENTATION: 48 year old female patient presented in OPD with complained of persistent dry cough and grade III dyspnea on exertion, generalised muscular pain and weakness. On examination she had mild anaemia, Pulse rate 96/mt BP 140/90 no lymphadenopathy, no clubbing. Respiratory system examination revealed crepitation in infrascapular area on both the side.Her hand were rough with creaky skin, crease of palm were dark suggestive of classical mechanics hand (figure 1). The movements of both the thumb were restricted .She was giving the history of joint pain and of Reynaud phenomenon.Investigations revealed,Hb 9.5 % gm, serum ANA was positive, Anti Ds DNA was negative, and C3 C4 and Rheumatoid factor was also negative. Transbronchial lung biopsy revealed nonspecific fibrosis and no evidence of granulomatus disorders. HRCT scan thorax was suggestive of bilateral lower lobe interstitial fibrosis in form of septal thickening, traction bronchieactasis and early honeycombing (figure 3&4). Spirometry revealed restrictive pattern . In the serum anti JO-1 was strongly positive. X-ray of the hand revealed subluxations of metacarpophalengeal joints in both the hand and progressive ankylosis of the same joint (figure2). Clinic radiological findings and serological findings were suggestive of interstitial lung disease due to AAS.
DISCUSSION: The Antisynthetase syndrome is a rare clinical entity, its comprises of a triad of inflammatory myopathy, diffuse interstitial lung disease, and small joint involvement1. Skin lesions characteristic of "mechanics hands", Reynaud’s phenomena, also present. Antisynthetase syndrome is also known as Jo-1 syndrome. It was first described by Marguerie and co-workers in 19902. At onset of disease, respiratory symptoms are present in 40-60 % of patients.The ILD infect is a major determinant of morbidity and mortality in the ASS. The most common of the anti-ARS, is anti-Jo-1, which is found in these case. In recent case series, the anti-Jo-1 antibody accounts for 68-87% of the anti-ARS observed in ASS 3. . As it is generally agreed upon that ILD is the clinical hallmark of ASS, this clinical feature is regarded mandatory for the diagnosis of ASS by most workers. Lung involvement in ASS is common. Interstitial lung disease is found in more than 60% patients of ASS . Join involvement includes a broad spectrum of clinical manifestation . In 21 patients of ASS who were anti Jo-1 positive two distinct groups of articular manifestations have been recognised . A subluxating arthropathy involving the distal joints of the fingers is a rather characteristic feature of ASS .
CONCLUSIONS: The Antisynthetase syndrome is a rare clinical entity, having multisystem involvement .It is comprises of a triad of inflammatory myopathy, diffuse interstitial lung disease, and small joint involvement. If this clinical entity diagnosed on time and treated adequately , then irreversible changes can be prevented.
Reference #1: Love LA, Leff RH, Fraser DD , Targoff IN, Dalakas M, Plotz PH. Miller FW.A new approach to the classification of idiopathic inflammatory myopathy:myositis-specific autoantibody define useful homogeneous patient groups.Medicine(Baltimore) 70:360-74;1991
Reference #2: Marguerie C, Bunn CC, Beynon HL, Bernstein RM, Hughes JM, So AK et al. Polymyositis, pulmonary fibrosis and autoantibodies to aminoacyl-Trna synthetase enzymes. Q J Med 1990; 77(282):1019-38.
Reference #3: Chinoy H, Salway F, Fertig N, Shephard N, Tait BD, Thomson W et al. In adult onset myositis, the presence of interstitial lung disease and myositis specific/associated antibodies are governed by HLA class II haplotype, rather than by myositis subtype. Arthritis Res Ther 2006; 8(1):R13
DISCLOSURE: The following authors have nothing to disclose: Ashok Singh, Raghvendra Jaiswal
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