New This Week: Extraarticular RA, CryoVas, Ormond and Schulman disease

March 5, 2013

A cohort study shows ethnic differences in the occurrence of extraarticular RA. a pleasant surprise about cryoglobulinemia vasculitis, and revelations about two eponymic disorders in rheumatology, Ormond disease and Schulman disease.

Last week's articles on rheumatology in the major nonspecialty journals.Extraarticular Manifestations of Rheumatoid Arthritis in a Multiethnic Cohort of Predominantly Hispanic and Asian PatientsMedicine, published online Feb. 20, 2013.  Full text $49


Hispanic rheumatoid arthritis patients were significantly more likely to develop extraarticular manifestations (ExRA) than Asian patients in a cohort study from the University of California San Francisco. The prevalence of ExRA was 21.5%, and the most common manifestations were subcutaneous nodules (17.2%) and interstitial lung disease (3.6%). The development of ExRA was also associated with disease duration, male sex, and seropositivity for serum rheumatoid factor.

 

The Spectrum of Type I Cryoglobulinemia Vasculitis: New Insights Based on 64 CasesMedicine, published online Feb. 22, 2013. Full text $49

The prognosis of type I cryoglobulinemia vasculitis (CryoVas), and the frequency of glomerulonephritis, was better than expected. A French nationwide survey included 64 patients with type I CryoVas, 28 patients with monoclonal gammopathy of unknown significance (MGUS) and 36 with hematologic malignancy. Ten-year survival rates were 87%. Treatments based on alkylating agents, rituximab, thalidomide or lenalinomide, and bortezomib showed similar efficacy against vasculitis manifestations, with clinical response rates from 80% to 86%.


Rethinking Ormond's Disease: "Idiopathic" Retroperitoneal Fibrosis in the Era of IgG4-Related DiseaseMedicine, published online Feb. 20, 2013. Full text $49

In 23 cases of idiopathic retroperitoneal fibrosis (RPF), over half (13) were IgG4-related. These were distinguished by histopathologic and extra-organ manifestations. Biopsies were more likely to show lymphoplasmacytic infiltrate, storiform fibrosis, or tissue eosinophilia.


Severe Aplastic Anemia Associated With Eosinophilic Fasciitis: Report of 4 Cases and Review of the LiteratureMedicine, published online Feb. 22, 2013.  Full text $49

In the first such reported case, rituximab has been used for severe aplastic anemia associated with eosinophilic fasciitis (Schulman disease). Rituximab had “significant efficacy” for both conditions. All four patients in this report had eosinophilic fasciitis and associated severe aplastic anemia, which can be refractory and life-threatening. For three of those patients, the aplastic anemia did not respond to conventional treatment with antithymocyte globulin and cyclosporine, and one of those patients received rituximab. A literature review identified 19 additional cases of eosinophilic fasciitis and aplastic anemia. Corticosteroids often improved skin condition but not the aplastic anemia, which was profound in over half the cases, and was the cause of death in eight cases (35%). Only 22% achieved long-term remission, with corticosteroids, cyclosporine, or stem cell transplantation.