Comparisons of SLE-DAS and SLEDAI-2K and classification of disease activity based on the SLE-DAS with reference to patient-reported outcomes.

OBJECTIVES: Although the SLE Disease Activity Score (SLE-DAS) and its definitions to classify disease activity have been recently developed to overcome the drawbacks of the SLE Disease Activity Index 2000 (SLEDAI-2K), the performance of the SLE-DAS for patient-reported outcomes (PROs) has not been fully examined. We aimed to compare SLE-DAS with SLEDAI-2K and validate the classifications of disease activity based on SLE-DAS in terms of PROs. METHODS: We assessed generic quality of life (QoL) using the Medical Outcome Survey 36-Item Short-Form Health Survey (SF-36), disease-specific QoL using the lupus patient-reported outcome tool (LupusPRO), burden of symptoms using the SLE Symptom Checklist (SSC), patient global assessment (PtGA) and physician global assessment (PhGA). RESULTS: Of the 335 patients with SLE, the magnitudes of the mean absolute error, root mean square error, Akaike information criterion, and Bayesian information criterion were comparable for most PROs between the SLE-DAS and SLEDAI-2K. In contrast, SLEDAI-2K had a higher predictive value for health-related QoL of LupusPRO and PtGA than SLE-DAS. Low disease activity, Boolean and index-based remission and categories of disease activity (remission, mild and moderate/severe activity) were significantly associated with health-related QoL in LupusPRO, SSC and PhGA, but not SF-36 or PtGA. CONCLUSION: No clear differences were identified in the use of the SLE-DAS over the SLEDAI-2K in assessing PROs in patients with SLE. The classification of disease activity based on the SLE-DAS was validated against several PROs. SLE-DAS and its categories of disease activity effectively explain some of the PROs.

Atypical Presenting Symptoms of Acute Onset Acquired Haemophilia with Eosinophilic Fasciitis.

Acquired haemophilia is a bleeding disorder caused by antibodies against coagulation factors. Some cases are associated with autoimmune diseases. However, no cases of acquired haemophilia with eosinophilic fasciitis have been previously reported. Herein we describe the case of a patient with eosinophilic fasciitis associated with acquired haemophilia. LEARNING POINTS: Eosinophilic fasciitis is one of the underlying diseases of acquired haemophilia.The underlying disease of acquired haemophilia should be investigated thoroughly and systematically to optimize therapeutic strategies.This is the first report of acquired haemophilia in association with eosinophilic fasciitis.

Acute Anorectal Thrombophlebitis Caused by a Protein C Deficiency.

A 46-year-old man visited the emergency department of our hospital with a 3-day history of anal pain, hemorrhaging, and a slight fever. He had previously been diagnosed with protein C deficiency and was prescribed dabigatran, a direct oral anticoagulant. Contrast-enhanced computed tomography showed severe rectal wall thickening with partial defect of enhancement. In addition, sigmoidoscopy revealed a dusky purplish swollen anorectal mucosa just above the dentate line. He was diagnosed with acute anorectal thrombophlebitis, and anticoagulant therapy with heparin was initiated. To our knowledge, this is the first case report of acute anorectal thrombophlebitis caused by protein C deficiency.