INTERESTING CASE OF MIXED CONNECTIVE TISSUE DISORDER

Mixed connective tissue disease (MCTD) has features common to lupus, scleroderma and myositis with high levels of antibodies to U1 ribonucleoprotein (U1 RNP). Its prevalence among patients with rheumatism has been reported to be low in India by Malaviya et al (0.3) and Nedumaran et al. (0.125 )20,26. Here we are presenting a 35 yr old lady with one month history of joint pain, dysphagia, exertional breathlessness and dry gangrene of upper limb.On evaluation she was found to have features of scleroderma (sclerodactyly, esophageal dysmotility),Rheumatoid arthritis(small joint pain and stiffness), Raynauds phenomenon, Interstitial lung disease with pulmonary hypertension, minimal renal involvement and no neurological involvement,with high titres of serum anti U1RNP levels, arterial Doppler showing intimal atherosclerotic plaque. We are reporting this case due to the unusual presentation of MCTD as Pulmonary hypertension even at the onset of symptoms, which indicates the multivariate effect of anti U1RNP in the disease process