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Objective The present study was undertaken to conduct a phase IV, open‐label, prospective study to characterize the long‐term safety of rituximab in a 4‐year observational registry of adult patients with granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) within the US. Methods Patients initiating treatment with rituximab were evaluated every 6 months for up to 4 years. Outcomes included the incidence of serious adverse events (SAEs), infusion‐related reactions (IRRs), and SAEs of specific interest, including serious infections, serious cardiac events, serious vascular events, and malignancies. Results Overall, 97 patients (72 with GPA and 25 with MPA) received rituximab through a median of 8 (range 1–28) infusions and were followed up for a median of 3.94 years (range 0.05–4.32 years). The estimated incidence rates (95% confidence interval [95% CI]) of serious infections, serious cardiac events, and serious vascular events were 7.11 (4.55–10.58), 5.03 (2.93–8.06), and 2.37 (1.02–4.67) per 100 patient‐years (PYs), respectively. No IRRs or SAEs occurred within 24 hours of an infusion of rituximab. None of the 9 deaths reported (crude mortality rate 2.67 [95% CI 1.22–5.06] per 100 PYs) were considered to be related to use of rituximab. Conclusion The safety profile of long‐term treatment with rituximab in patients with GPA or MPA was consistent with that of rituximab administered for shorter durations and with rituximab’s known safety profile in other autoimmune diseases for which it has received regulatory approval. These findings provide clinicians with long‐term, practice‐level safety data for rituximab in the treatment of GPA or MPA.

Background Takayasu arteritis is a rare large-vessel vasculitis that predominantly affects females of Asian descent. This retrospective analysis was performed to increase understanding of the epidemiology of the disease in the United States. Methods We performed a retrospective cohort study in 2 tertiary centers. Patients were selected according to the American College of Rheumatology classification criteria for Takayasu arteritis. Data collected included demographic characteristics and details of physical examinations, treatments, and surgical interventions. Data were managed with REDCap (Research Electronic Data Capture) tools. Results The study included 57 patients. The female:male ratio was 4.2:1, the median age at diagnosis was 29 years, 61.4% of the patients were Caucasians, and 86% of the patients had stenosis on imaging. Hata V was the most common angiographic classification (37.5% of patients). Vascular interventions were required in 43.9% of patients. The most frequent complications were hypertension (56.1%), renal artery stenosis (28.1%), and aortic insufficiency (19.3%). Conclusions Takayasu arteritis continues to be a rare large-vessel vasculitis. In the United States, it tends to affect predominantly Caucasian females, with cervicobrachial involvement. This cohort reflects the morbidity, multiple interventions, and complications experienced by patients with Takayasu arteritis.

A 68-year-old man, with a history of non-Hodgkin’s lymphoma in remission, was admitted for homonymous hemianopsia, headaches and subacute progressive cognitive decline. Imaging revealed brain infarcts and angiography suggested vasculitis. A brain biopsy, however, revealed an intravascular large B-cell lymphoma (IVLBL). Central nervous system (CNS) vasculitis and IVLBL of the brain are extremely rare diseases that can have an almost identical clinical presentation. Angiographic findings are very similar but usually are reported as compatible with vasculitis. Brain biopsy or a random skin biopsy are crucial in diagnosing IVLBL as the accuracy of angiographic findings for CNS vasculitis is low.

Background Tumor necrosis factor α inhibitors (TNFi) are commonly used to treat immune-mediated disorders, but they are associated with an increased risk of mycobacterial and fungal infections. We compared the outcomes of TNFi recipients screened for asymptomatic coccidioidomycosis with those of unscreened patients to compare the development of symptomatic coccidioidomycosis and to describe its outcomes for patients with abnormal coccidioidal screenings. Methods We searched electronic health records from 4 September 2010 through 26 September 2016 for all patients receiving a TNFi for dermatologic, rheumatologic, or gastroenterologic diagnoses, then categorized patients by whether or not they had undergone coccidioidal serologic testing for screening or diagnostic purposes. Results A total of 2793 patients had a TNFi prescribed. Of those, 1951 met the inclusion criteria: 1025/1951 (52.5%) never had coccidioidal screening; 925/1951 (47.4%) had serologic screening either before beginning TNFi therapy or annually, or both after beginning a TNFi. Symptomatic coccidioidomycosis developed in 35/1025 (3.4%) unscreened patients. Of those screened, 861/925 (93.1%) had negative serologic tests, of which 11/861 (1.3%) subsequently developed symptomatic coccidioidomycosis; 36/925 (3.9%) had coccidioidomycosis at screening (7, probable infection; 11, possible infection; 18, asymptomatic seropositive result); and 17 had only positive findings for immunoglobulin M antibodies and did not meet the definition for coccidioidomycosis. The unscreened cohort was more likely to have symptomatic coccidioidomycosis than the screened cohort (35/1025 vs 11/861, P Conclusions Screening for asymptomatic coccidioidomycosis within a Coccidioides-endemic area allowed for identifying and managing asymptomatic coccidioidomycosis before patients began TNFi therapy. Less symptomatic infection developed in the screened than the unscreened cohort.

Coccidioidomycosis is a potentially serious fungal infection contracted in endemic areas of the desert southwestern United States. Limited information exists about its incidence and clinical course in patients with rheumatic diseases, who may be at higher risk of symptomatic or disseminated coccidioidomycosis because of either the rheumatic disease itself or its treatment. We analyzed the incidence and risk factors for symptomatic and complicated coccidioidomycosis in our academic rheumatology practice in central Arizona. Between January 1, 2000, and June 30, 2006, coccidioidomycosis was diagnosed in 1.9% of the overall practice and in 3.1-3.6% of patients with rheumatoid arthritis (RA). The annual incidence was 1% in patients recently diagnosed with RA and 2% among patients with recently initiated infliximab treatment. Coccidioidomycosis was identified only in patients with inflammatory rheumatic diseases and extrathoracic dissemination occurred only to joints in two patients. Corticosteroids, immunosuppressive medications, and tumor necrosis factor inhibitors (TNFIs) appeared to be risk factors for symptomatic, but not disseminated coccidioidomycosis.

Objective. Between June 1, 1992 and August 31, 1994 we conducted an open pilot study of antithymocyte globulin (ATGAM; Upjohn, Kalamazoo, MI) in 10 patients with early systemic sclerosis (SSc) to assess whether this agent might prevent the progression of cutaneous and pulmonary involvement in this disease. Methods. Adult patients with early SSc (

Enzyme-linked immunosorbent assay (ELISA) and immunofluorescence assay (IFA) were performed in 25 and 32 cases of morphea, respectively. The more sensitive and specific ELISA was positive in only 1 of 25 cases and the mean value was lower in cases of morphea than in controls. IFA showed minimally reactive titers in 6 (19%) of 32 cases. There were an additional six cases with borderline titers. These data indicate that there is no specific association between Borrelia burgdorferi infection and morphea but that patients with morphea tend to have circulating antibodies that are cross-reactive.