Acute-onset Type Research Articles

Systemic lupus erythematosus in three ethnic groups: IX. Differences in damage accrual

To determine the factors predictive of damage in a multiethnic (Hispanic, African American, and Caucasian) LUMINA (lupus in minority populations, nature versus nurture) cohort of patients with systemic lupus erythematosus (SLE) with disease duration of < or =5 years at enrollment (T0). Variables (socioeconomic/demographic, clinical, immunologic, immunogenetic, behavioral, and psychological) were measured at T0 and annually thereafter. Disease damage was measured with the Systemic Lupus International Collaborating Clinics Damage Index (SDI), and disease activity was measured with the Systemic Lupus Activity Measure. The relationship between the different variables and the SDI at the last visit (TL) was examined (mean followup from diagnosis to TL 61 months; adjusted for disease duration). Poisson regression was used to identify the independent association between the different variables and SDI scores at TL. Seventy-two Hispanics, 104 African Americans, and 82 Caucasians were included. One-half of patients had not accrued any damage. Caucasians had the lowest SDI scores at T0, and Hispanics had the highest scores at TL. Renal damage occurred more frequently among Hispanics and African Americans, while integument damage was more frequent among African Americans. Neuropsychiatric (20%), renal (16%), and ocular (15%) damage occurred most frequently among all patients. Independent predictors of SDI at TL were age, corticosteroid use (maximum dose at T0), number of American College of Rheumatology (ACR) criteria met, disease activity, and abnormal illness-related behaviors. Other variables were less consistently associated with damage accrual (poverty in African Americans, lack of HLA-DRB1*0301 in Hispanics, presence of HLA-DQB1*0201 and acute onset of SLE in Caucasians). Damage in SLE occurs from the outset in some, but not all, patients; Hispanics accrue damage more rapidly. Disease factors (corticosteroid use, number of ACR criteria met, disease activity, and acute-onset type) are important, but age and abnormal illness-related behaviors also contribute to overall damage in SLE.

T-cell insulitis found in anti-GAD65+ diabetes with residual beta-cell function. A case report.

We recently encountered a 65-year-old anti-GAD+ diabetic woman with residual beta-cell function who was proved to have T-cell insulitis. The proportion of CD4+ and CD8+ cells varied among individual islets, although CD4+ cells tended to be the predominant T-cell type in the islets examined. All of the islets examined still contained insulin, suggesting that beta-cell mass may have been preserved. It is well known that lymphocytic infiltration of pancreatic islets, a condition referred to as "insulitis," is seen in acute-onset type 1 diabetes at autopsy and in biopsy specimens. However, there have been no proven cases of insulitis in type 1 diabetes with residual beta-cell function. We believe that this is the first type 1 diabetic patient with residual beta-cell function who was proven to have T-cell insulitis. This novel evidence will contribute to the proper classification and treatment of diabetes and to a better understanding of the pathophysiology of type 1 diabetes.

A Human Endogenous Retroviral Superantigen as Candidate Autoimmune Gene in Type I Diabetes

Microbial superantigens (SAGs) have been implicated in the pathogenesis of human autoimmune diseases. Preferential expansion of the Vβ7 T cell receptor positive T cell subset in patients suffering from acute-onset type I diabetes has indicated the presence of a surface membrane–bound SAG. Here, we have isolated a novel mouse mammary tumor virus–related human endogenous retrovirus. We further show that the N-terminal moiety of the envelope gene encodes an MHC class II–dependent SAG. We propose that expression of this SAG, induced in extrapancreatic and professional antigen-presenting cells, leads to β-cell destruction via the systemic activation of autoreactive T cells. The SAG encoded by this novel retrovirus thus constitutes a candidate autoimmune gene in type I diabetes.

Prediction of prognosis in patients with hemolytic-uremic syndrome.

AbstractHemolytic uremic syndrome (HUS) is characterized by hemolytic anemia, thrombocytopenia and acute renal insufficiency. Recently we experienced 6 HUS patients who had different clinical modes of onset, different clinical courses and a poor prognosis compared with the patients with the above typical clinical pictures. Thirteen HUS patients were evaluated in two aspects: mode of onset (acute or gradual type) and clinical corn (non‐recurrent or recurrent type).During 4.9 ± 3.1 (mean ± sd) years' of observation, renal insufficiency was more frequently observed in the gradual onset and recurrent course types respectively than in the acute onset and recurrent types (p &lt; 0.05, p &lt; 0.01). Also, the mean age was significantly lower and platelet counts were much higher in the gradual onset type than in the acute onset type (p &lt; 0.05, p &lt; 0.01). However, clinical findings except renal failure revealed no differences between non‐recurrent and recurrent HUS. For the prediction of HUS, we emphasize the importance of classifying HUS by the mode of onset (acute or gradual) and/or clinical course (non‐recurrent or recurrent).