Vascular disease is frequent in patients with systemic lupus erythematosus, which can be related to the disease process, or can develop as an accompanying co-morbidity and represents the most frequent cause of death in established disease. However, at times the presentations can be uncommon and subtle, and warrants a thorough examination both clinically and radiologically. We report a case of a young female with photosensitive malar rash, oral ulcers, intermittent fever with joint pains, history of two abortions, and unilateral absent radial and brachial artery pulses on clinical examination. The evaluation revealed positive antinuclear antibody (4+), anti-Smith antibody (2+), direct Coomb's test (2+), and antiphospholipid antibody panel was negative. Color doppler flow imaging of right upper limb (arterial) revealed irregular wall thickening with a narrow lumen and mildly reduced peak systolic volume. Computed tomography aortogram revealed wall thickening and luminal narrowing involving the entire length of the right brachial and radial artery. We report this case for its rarity and unique presentation of medium vessel vasculopathy.

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Petersdorf and Beeson first defined fever of unknown origin (FUO) in 1961, and subsequently, over the next 60 years, the definition of FUO has changed considerably. In the western world, non-infectious inflammatory diseases are the most common cause of FUO; however, in developing countries, infections remain the leading cause of FUO. Dermatomyositis (DM) is an autoimmune inflammatory disease of unknown aetiology which mainly affects skin and muscles. Anti-melanoma differentiation-associated protein 5 (MDA-5) positive DM generally presents with classical cutaneous manifestations, early interstitial lung disease, and patients generally do not have clinical features of muscle involvement. We present a case of a 39-year-old male who presented with FUO and hepatitis and was diagnosed as clinically amyopathic DM after two weeks of admission. Subsequently, he was found to have a high titre of Anti-MDA-5 antibody. This is the first case of Anti-MDA-5 positive DM presenting as FUO and hepatitis with a favourable outcome to the best of our knowledge.

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