Objective: To determine how to use the Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI) to classify patients according to disease severity (mild, moderate, and severe) and to identify which patients respond to therapy.Design: Cohort.
Background
There is increased expression of type I interferon (IFN)-regulated proteins in the blood and target tissues of patients with cutaneous lupus erythematosus (CLE) and systemic lupus erythematosus (SLE). Patients with SLE have increased IFN-regulated gene expression pointing towards a possible underlying genetic defect.
Objectives
We measured expression levels of five type I IFN-regulated genes that are highly expressed in SLE in the peripheral blood of patients with CLE and correlated expression levels with cutaneous disease activity.
Methods
Peripheral blood was obtained from 10 healthy controls and 30 patients with CLE, including 8 with concomitant SLE. Total RNA was extracted and reverse transcribed into complimentary DNA. Gene expression levels were measured by real time PCR. Gene expression was normalized to GAPDH, standardized to healthy controls and then summed to calculate an IFN score for each patient. Disease activity was assessed with the Cutaneous Lupus Area and Severity Index (CLASI).
Results
Patients with subacute cutaneous lupus erythematosus (SCLE) and discoid lupus erythematosus (DLE) had elevated IFN scores compared to healthy controls regardless of concomitant SLE (p< 0.01 with SLE and p<0.05 without SLE). There was no difference between patients with tumid lupus erythematosus (TLE) and healthy controls. The IFN score correlated with CLASI scores (Spearman’s Rho (r) = 0.55, p = 0.0017).
Conclusions
Patients with SCLE and DLE have an IFN signature, as seen in SLE. The level of gene expression correlates with cutaneous disease activity. These findings support a shared pathogenesis between SLE and some subtypes of CLE.
Background-Dermatomyositis is an autoimmune disease of unknown etiology characterized by inflammation of the skin and muscles. Several medications have been implicated in the development of dermatomyositis 1 , however the disease has rarely been linked to the use of tumor necrosis factor-inhibitors (TNF-inhibitors). Herein we report four cases of dermatomyositis that developed or were exacerbated by exposure to the TNF-inhibitors etanercept and adalimumab.Observation-Four patients with symptoms of inflammatory arthritis were treated with TNFinhibitors, for a duration ranging from two months to two years. All patients developed symptoms consistent with dermatomyositis, including inflammatory rash and muscle weakness. Their symptoms persisted after discontinuation of the TNF-inhibitors but responded to treatment with steroids and immunosuppressive medications.
Conclusion-TNF-inhibitors have been associatedwith the onset of a number of autoimmune disorders, most commonly vasculitis and a lupus-like syndrome. Rarely have they been associated with dermatomyositis. The four cases presented here indicate that TNF-inhibitor use can be associated with either induction or exacerbation of dermatomyositis.
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