Protein S deficiency associated to anti-protein S antibodies in a patient with mixed connective-tissue disease and its reversal by danazol

Guillermo J. Ruíz‐Argüelles, G J Ruiz-Argüelles, A Ruiz-Argüelles, Alejandro Ruı́z-Argüelles, B Pérez-Romano, Beatriz Pérez‐Romano, D Alarcón-Segovia
Acta haematologica · 2009 · vol. 89(4) , pp. 206–208 · doi:10.1159/000204525 · PMID:8213003 · W2046747990
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This report details a patient with mixed connective tissue disease and protein S deficiency due to anti-protein S antibodies, which normalized upon danazol treatment.

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Abstract

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

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MeSH descriptors

Autoantibodies Connective Tissue Diseases Danazol Protein S Protein S Deficiency Adult Antiphospholipid Syndrome Antiphospholipid Syndrome Autoantibodies Connective Tissue Diseases Connective Tissue Diseases Danazol Female Humans Hypertension, Pulmonary Hypertension, Pulmonary Prednisone Prednisone Protein S

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