A RARE CASE REPORT OF MULTIPLE CONNECTIVE TISSUE DISEASE WITH SEVERE PULMONARY HYPERTENSION

Abstract

A 38 year old female presented with complaints of tightness, dryness, hyperpigmentation of skin , tightness of mouth, tingling numbness and pain in ngers while handling cold water since 6 months. Exertional breathelessness since 1 month .She had history of swelling of hands six months back. Finger examination showed sclerodactyly and ulceration over skin on interphalangeal joints of both hands without any deformity .Since clinical ndings were suggestive of Raynaud's phenomenon, sclerodactyly, swelling of hands , scleroderma, she was further investigated for presence of connective tissue disease . On immunological investigations , ANA prole was positive with presence of strong positive U1RNP antibodies (titre>1:1600). Chest x ray and 2d echocardiography revealed presence of Pulmonary Hypertension. She was diagnosed with Multiple Connective Tissue Disease (MCTD) with severe pulmonary hypertension based on clinical , radiological and laboratory ndings satisfying Alarcon Segovia criteria.