Abstract

Introduction: Antisynthetase syndrome is an original entity and rare autoimmune myositis and systemic disease, characterized clinically by a wide spectrum of clinical manifestations and the presence of autoantibodies directed against aminoacyl RNAt synthetases. We describe this disease in 03 Senegalese patients. Observations: The first patient was a 49-years-old black woman who was referred in our department after 06-months of follow-up for a misdiagnosis of tuberculosis. The clinical examination revealed polyarthritis, muscle weakness, chronic cough with crackling rales at the pulmonary bases, Raynaud phenomenon and dry syndrome. The second patient, a 21-years-old black woman, had polyarthritis and a progressive muscle weakness. The clinical examination showed also cutaneous signs including an erythema on the dorsal part of the fingers and the presence of the heliotrope erythema on the eyes. The last patient was a 52 years-old black woman. His clinical examination showed polyarthritis, muscle weakness and an appearance of mechanics’ hands. The creatinine phosphokinase was at 6.26 × N, 40.3 × N and 33.64 × N respectively in our patients. The chest computer tomography revealed an interstitial lung disease with a pattern of non-specific interstitial pneumonia in all three patients. The autoantibodies anti-Jo1 was also positive in all patients. The diagnosis of antisynthetase syndrome was retained with an overlap of antisynthetase and Sj?gren’s syndrome in the first observation. The evolution was favourable in our 03 observations with a therapeutic combination including Prednisone-Azathioprine and Kinesitherapy. Conclusion: Antisynthetase syndrome has been exceptionally reported in sub-Saharan Africa. It must be particularly mentioned in front of the triad: myositis, arthritis and interstitial lung disease. The identification of an auto-antibody directed against RNA t synthetases, particularly anti-Jo1, is essential for its diagnosis. Prognosis is related to interstitial lung involvement. The evolution has been favourable in our patients receiving Glucorticoid-Azathioprine combination therapy.

Highlights

  • Antisynthetase syndrome is an original entity and rare autoimmune myositis and systemic disease, characterized clinically by a wide spectrum of clinical manifestations and the presence of autoantibodies directed against aminoacyl RNAt synthetases

  • It must be mentioned in front of the triad: myositis, arthritis and interstitial lung disease

  • The identification of an auto-antibody directed against RNA t synthetases, anti-Jo1, is essential for its diagnosis

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Summary

Introduction

The idiopathic inflammatory myopathies (IIMs), known collectively as myositis, constitute a large spectrum of clinical phenotypes. As indicated by the name, the classical clinical manifestations of IIMs, such as muscle weakness, relate to chronic inflammation in skeletal muscle. This inflammation frequently affects other organs, including the skin, joints, lungs, gastrointestinal tract and heart, indicating the systemic nature of this disease. ASAs constitute one of the key laboratory features of ASS, which is defined as an association of inflammatory myopathy, diffuse interstitial lung disease, joint involvement, fever and cutaneous signs such as mechanic’s hands and Raynaud’s syndrome [3] [5] [6]. We discuss 03 Senegalese cases of ASS and we hope that these observations will add to medical literature and aid in early diagnosis of other patients presenting with similar features

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