Abstract
Vasculopathy is a frequent complication of systemic sclerosis (SSc) and many overlap syndromes with SSc, including mixed connective tissue disease (MCTD). It manifests itself mainly by pulmonary arterial hypertension (PAH) and by the presence of digital ulcers (DUs).
The treatment of both events is difficult and prolonged. We describe a case of a 31-year-old female with MCTD accompanied by PAH and DUs.
The disease was characterized by Raynaud's phenomenon, pleurisy, pericarditis, Coombs' positive haemolytic anaemia, interstitial lung disease and positive autoantibodies against the nuclear antigen U1-RNP. She met the diagnostic criteria for MCTD by Sharp et al. (1987).
The target organ for MCTD was the lung. Immunosuppressive therapy usually selected for this complication (cyclophosphamide, cyclosporin A and azathioprine) was discontinued due to recurrent septic states.
Three DUs developed in the lower limbs. An examination for dyspnoea led to the diagnosis of PAH (mean pulmonary artery pressure of 40 mm Hg documented by heart catheterisation).
The drug of choice for this PAH patient, bosentan, resulted not only in cardiac improvement (echocardiographic finding and NYHA), but also complete healing of the DUs. Our MCTD patient is in long-term remission.
We report the treatment of PAH and DUs in a patient with MCTD. The healing of DUs was noted during bosentan treatment, in addition to preventing the development of new DUs.