Rituximab with or without a conventional maintenance agent in the treatment of relapsing granulomatosis with polyangiitis (Wegener's): a retrospective single-center study.

OBJECTIVE: To evaluate the efficacy and safety of rituximab (RTX) induction therapy and the duration of remission, when RTX is used with or without a conventional maintenance agent, in a cohort of patients with granulomatosis with polyangiitis (Wegener's) (GPA). METHODS: This was a retrospective, single-center study of patients with relapsing GPA treated with at least 1 course of RTX (4 weekly doses of 375 mg/m(2) intravenously [IV] or 2 fixed doses of 1,000 mg IV 2 weeks apart). Complete remission was defined as the absence of disease activity measured by a Birmingham Vasculitis Activity Score for Wegener's granulomatosis of 0 and not qualified by the prednisone dosage at the time. RESULTS: Eighty-nine patients achieved remission after their first course of RTX and were not re-treated preemptively with RTX to maintain remission of their disease during followup. Among these patients, relapse-free survival was significantly higher in those who received a conventional maintenance agent (azathioprine, methotrexate, or mycophenolate mofetil) in conjunction with RTX and glucocorticoids (n = 47) than in those who received no additional immunosuppressive agent (n = 42) (P = 0.04). The hazard ratio of relapse in those receiving a maintenance agent was 0.53 (95% confidence interval 0.29-0.97). Serious adverse events did not differ between the 2 groups. Within a subset of 15 patients in the cohort who were relapse free 2 years after 1 course of RTX, remissions endured for 2-6 years in 8 patients. CONCLUSION: RTX is an effective remission-inducing agent in GPA. The addition of a conventional maintenance agent to RTX and glucocorticoids decreased the incidence of relapse and did not result in a higher incidence of adverse events.

Paraneoplastic rheumatologic syndromes.

PURPOSE OF REVIEW: To describe rheumatic syndromes that can be a paraneoplastic manifestation of an underlying malignancy. The pathogenesis of paraneoplastic rheumatologic diseases is complex and not fully understood in the majority of instances. In the absence of a defined pathogenic link between the malignancy and the rheumatic syndrome, the association between these diseases has been based on concurrence and a parallel clinical course. RECENT FINDINGS: This is an updated review of paraneoplastic presentations of synovitis, bone disease, myositis, and vasculitis. SUMMARY: Although paraneoplastic rheumatic syndromes are rare, clinicians should be aware that they can be the first sign of an occult malignancy and that early recognition is vital for early cancer diagnosis and treatment.

Perivascular carotid inflammation: an unusual case of carotidynia.

A 59-year-old woman was admitted to the hospital with a fever and rigors for 2 days. She was on chemotherapy (docetaxel, carboplatin, and trastuzumab) for her stage II invasive ductal carcinoma of the breast. Her physical exam was unremarkable except for the fever. The white blood cells were 21,200/mm(3) with 92% of neutrophils. ESR was 106 mm/h. An extensive infectious workup was negative. On day 6, while still febrile, the patient complained of a left-sided neck pain. She exhibited tenderness over the left carotid artery. A CT scan of the neck without intravenous contrast showed perivascular inflammation of the left common carotid artery, without evidence of a collection, arterial thrombosis, aneurysm, or dissection. The etiology of this finding was possibly chemotherapy related. It dramatically responded to oral prednisone. A repeat CT scan of the neck with IV contrast 2 weeks later showed a remarkable improvement. Drug reactions can simulate systemic inflammatory diseases and should always be considered in the diagnosing process.