PROTEIN-S DEFICIENCY ASSOCIATED TO ANTI-PROTEIN-S ANTIBODIES IN A PATIENT WITH MIXED CONNECTIVE-TISSUE DISEASE AND ITS REVERSAL BY DANAZOL

We describe here a female patient with mixed connective tissue disease , secondary antiphospholipid syndrome, pulmonary hypertension and seve re acquired, probably autoimmune, antibody-mediated type I coagulation protein S deficiency (total, free and C4bp-bound). No previous instan ce of anti-PS anti-body-associated PS deficiency had been reported. Th e patient was treated initially with prednisone, but the protein S lev els did not rise until danazol was added, and dropped again after its withdrawal.