Widespread vasculitis with pulmonary hypertension in a patient with mixed connective tissue disease －A case considered to have converted to SLE－

Abstract

　We describe a female diagnosed with MCTD at the age of 44 with Raynaud’s phenomenon, finger swelling, positive of anti-RNP antibody (but not anti-Sm or anti-DNA antibody), polyserositis, leukopenia and pulmonary fibrosis. She had a history of cutaneous vasculitis in the legs at the age of 50. She developed pulmonary hypertension at the age of 60 and the severity gradually worsened despite steroid therapy. However, pulmonary hypertension became comparatively stable after initiation of the endothelin receptor antagonist, bosentan. She developed general fatigue without fever, skin ulcer or other remarkable symptoms. On admission, slightly elevated anti-dsDNA antibody level, mild hypocomplementemia and image findings suspected of infection were observed. Diarrhea and cerebral hemorrhage successively occurred and lethal heart failure subsequently developed. Postmortem examination revealed widespread vasculitis involving the lungs, pancreas, digestive tracts, brain, uterus, ovaries, skeletal muscles and gall bladder (but not kidney). Careful follow-up of serum markers for SLE may be useful in selected MCTD cases for early detection of vasculitis because a marked predisposition to SLE might have been a contributory factor for onset of vasculitis in this case. In terms of the pathophysiology, prolonged morbidity of pulmonary hypertension might also have contributed to the onset of vasculitis. In the current situation where long-term survival is increasing in patients with MCTD complicated with pulmonary hypertension, careful observation is required when treating MCTD patients, especially those with a marked predisposition to SLE.