warfarin and Polychondritis--Relapsing

Relapsing polychondritis is a rare immune-mediated condition, characterized by episodic inflammation of the cartilaginous tissue, in particular the ears, nose, and eyes, and involvement of joints and respiratory tract. Nearly one third of patients showed other associated diseases, such as systemic vasculitides, connective tissue diseases, or myelodysplastic syndromes. Antiphospholipid antibodies can be found in relapsing polychondritis in patients with no clinical thrombotic disease. However, when antiphospholipid syndrome is present, its clinical manifestations can be severe and life threatening. We describe the case of a patient with relapsing polychondritis associated to Budd-Chiari syndrome due to antiphospholipid syndrome. The present clinical observations together with the updated review of the literature suggest a search for antiphospholipid antibodies in all patients with relapsing polychondritis.

Topics: Antibodies, Antiphospholipid; Antiphospholipid Syndrome; Azathioprine; Budd-Chiari Syndrome; Comorbidity; Humans; Immune System; Immunologic Factors; Immunosuppressive Agents; Male; Middle Aged; Polychondritis, Relapsing; Prednisone; Treatment Outcome; Warfarin

Relapsing polychondritis is rare and its cause is unknown. The tissues affected are those with a high glycosaminoglycan content, such as cartilage, the aorta, the sclera and cornea, and parts of the ear. Symptoms can usually be controlled with oral steroids, but when there is coexistent progressive crescentic glomerulonephritis quadruple chemotherapy may be used. Three cases of the clinical syndrome of relapsing polychondritis were studied in which rapidly progressive cresentic glomerulonephritis developed. In two the patients appeared to respond to aggressive treatment with immunosuppressive agents and anticoagulants. The multisystemic nature of the disease, the renal lesions, and the response to treatment all suggested that the condition might be related to periarteritis nodosa.

Topics: Adult; Aged; Azathioprine; Dipyridamole; Drug Therapy, Combination; Female; Glomerulonephritis; Humans; Male; Middle Aged; Polychondritis, Relapsing; Prednisolone; Warfarin