ABSTRACT
Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare form of systemic vasculitis in which cardiac involvement is frequent and severe, and accounts for half of EGPA-related deaths. ANCA-positive EGPA differs from ANCA-negative EGPA in that the former is significantly associated with renal involvement, peripheral neuropathy and biopsy proven vasculitis, whereas the latter is associated with cardiac involvement. Herein, we report a case of EGPA with myocarditis in a woman, who was successfully treated with steroids and cyclophosphamide. This report highlights the importance of diagnosing cardiac involvement in EGPA early, especially in ANCA-negative patients.
Subject(s)
Churg-Strauss Syndrome/complications , Churg-Strauss Syndrome/diagnosis , Cyclophosphamide/therapeutic use , Granulomatosis with Polyangiitis/complications , Granulomatosis with Polyangiitis/diagnosis , Immunosuppressive Agents/therapeutic use , Myocarditis/diagnosis , Myocarditis/etiology , Steroids/therapeutic use , Antibodies, Antineutrophil Cytoplasmic/blood , Churg-Strauss Syndrome/drug therapy , Diagnosis, Differential , Female , Granulomatosis with Polyangiitis/drug therapy , Humans , Middle Aged , Myocarditis/drug therapy , Myocarditis/immunology , Prognosis , Severity of Illness Index , Treatment OutcomeABSTRACT
Systemic lupus erythematosus (SLE) has been described as a cause of thrombotic microangiopathy, especially thrombotic thrombocytopenic purpura (TTP). Haemolytic-uraemic syndrome (HUS) is less frequent in SLE. We report a case of such an association during an episode of severe lupus nephritis in a young woman, who was successfully treated with steroids, cyclophosphamide and especially plasma exchange with plasma replacement. This report highlights the importance of recognising atypical HUS in SLE patients by looking for schistocytes in case of haemolytic anemia with a negative antiglobulin test, in order to begin plasma exchange.
Subject(s)
Hemolytic-Uremic Syndrome/therapy , Lupus Nephritis/complications , Plasma Exchange , Acute Kidney Injury/etiology , Adult , Biopsy , Combined Modality Therapy , Cyclophosphamide/therapeutic use , Drug Therapy, Combination , Erythrocytes, Abnormal , Female , Hemolytic-Uremic Syndrome/blood , Hemolytic-Uremic Syndrome/diagnosis , Hemolytic-Uremic Syndrome/drug therapy , Hemolytic-Uremic Syndrome/etiology , Humans , Hydroxychloroquine/therapeutic use , Immunosuppressive Agents/therapeutic use , Kidney/pathology , Lupus Nephritis/drug therapy , Methylprednisolone/therapeutic use , Models, Immunological , Mycophenolic Acid/analogs & derivatives , Mycophenolic Acid/therapeutic use , Prednisone/therapeutic useABSTRACT
Diffuse and abundant sweating in a middle age patient evolving for several weeks should raise suspicion of malignant lymphoma and infectious or neuroendocrine disorders before considering a drug origin. We report a patient who presented with severe and invalidating excessive sweating related to hydromorphone therapy for vertebral pain. Amongst their many reported side-effects, excessive sweating disappearing with discontinuation of the drug have been reported with some opiates.
