Successful treatment of refractory lupus erythematosus profundus with the interleukin-6 antagonist tocilizumab: a case report.

Lupus erythematosus profundus (LEP) is an unusual form of cutaneous lupus erythematosus (CLE) characterized by multiple subcutaneous induration and associated with considerable physical and psychological morbidity. The heterogeneity of CLE makes it difficult to understand its underlying pathogenesis and represents a therapeutic challenge. Recently, new insight into the pathogenesis of CLE has implicated various cytokines, opening doors to targeted biologic agents. We report a case of a 23-year-old female who presented with refractory LEP ulcers as an initial presentation of systemic lupus erythematosus. The lesions were resistant to multiple conventional therapies and remarkably responded to tocilizumab.

Effective control of polymyalgia rheumatica with tocilizumab.

Despite their disadvantages, glucocorticoids (GCs) remain a mainstay of therapy for polymyalgia rheumatica (PMR). Second-line antirheumatic and immune-modulatory drugs are not infrequently required because of disease relapses during GC tapering and GC adverse effects. Therapy with methotrexate or with an anti-tumor necrosis factor drug showed modest efficacy in this situation. Tocilizumab (TCZ) is an anti-interleukin 6 receptor antibody that is being recently studied in the treatment of PMR patients who are intolerant or refractory to GCs, especially after failure of a second-line agent. We report a case of PMR in which GCs were stopped because of adverse effects despite good response. The condition responded to neither methotrexate nor etanercept. Treatment with TCZ has led to significant improvement of the patient's clinical and biochemical PMR activity parameters, and she was kept in a solid remission for 1 year without any TCZ-related adverse effects. Tocilizumab is a promising drug in the management of PMR. Further studies are required to clearly define the indications and duration of TCZ therapy in the management of PMR.

ANCA negative pulmonary renal syndrome with pathologic findings of thrombotic microangiopathy.

INTRODUCTION: Thrombotic microangiopathy (TMA) is characterized by aggregation of platelets in the renal and/or systemic circulation, thrombocytopenia and intravascular hemolysis. The syndrome classically spares the lung. The term pulmonary-renal syndrome describes a number of diseases in which pulmonary hemorrhages and glomerulopathy coexist. CASE REPORT: We report a 44-year-old man admitted to hospital because of chronic unexplained fever. Six days after admission he developed hemoptysis, respiratory distress and biochemical evidence of acute renal failure. High-resolution computed tomography scan of the chest demonstrated alveolar hemorrhages. The patient developed hypoxia and was shifted to the intensive care unit to be supported by mechanical ventilation. He also received two sessions of continuous veno-venous hemodiafiltration. Kidney biopsy revealed pathological findings of TMA. Serology for anti-neutrophil cytoplasmic antibodies, anti-cardiolipin antibodies and anti-glomerular basement membrane antibodies was negative. The patient was treated with pulse steroids followed by prednisolone with mild improvement. Seven days later, his condition deteriorated with an increase in serum creatinine and pulmonary hemorrhages. His hemoglobin level dropped and he developed features of intravascular hemolysis. A diagnosis of TMA was made and treatment with plasma exchange was initiated. The patient showed dramatic improvement and was discharged in good condition. He remained in remission throughout his subsequent follow up. CONCLUSION: TMA should be considered in the differential diagnosis of pulmonary renal syndromes, and can be successfully managed by corticosteriods combined with plasma exchange.

Granulomatous hepatitis associated with etanercept therapy.

Etanercept has recently been implicated in the induction of granulomatous reactions. We describe a patient with rheumatoid arthritis who developed granulomatous hepatitis after taking etanercept. Infectious and metabolic causes of liver disease had been excluded and the liver biopsy was not typical of sarcoidosis. Liver enzyme abnormalities improved after etanercept was discontinued. We suggest that etanercept was responsible for the development of granulomatous hepatitis. This has not been previously described and adds to the increasing reports of rare granulomatous reactions induced by etanercept therapy.