Clin Exp Rheumatol
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Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are a group of autoimmune disorders including Wegener's granulomatosis (WG), microscopic polyangiitis (MPA), Churg-Strauss syndrome (CSS) and renal-limited vasculitis (RLV). This paper reviews updated information on the pathogenesis of AAV. Additional clinical evidence for a pathogenic role of ANCA comes from the observation that patients with severe acute renal failure treated with plasma exchange had a lower risk for progression to end-stage renal disease than patients who received intravenous methylprednisolone therapy, both in addition to standard treatment. ⋯ Rats developed both cross-reactive antibodies to LAMP-2 and crescentic glomerulonephritis when immunized with FimH, an adhesin from Gram-negative bacteria which has strong homology with human LAMP-2. Together, clinical, in vitro and in vivo data support a pathogenic role for ANCA in AAV, although this role is more evident for myeloperoxidase-ANCA than for PR3-ANCA. The role of anti- LAMP-2 requires further studies.
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Comparative Study
The patient-rated wrist evaluation (PRWE): cross-cultural adaptation into German and evaluation of its psychometric properties.
To cross-culturally adapt the Patient-Rated Wrist Evaluation form (PRWE) into German (PRWE-G) and to evaluate its reliability and validity. ⋯ The PRWE-G represents a valid and reliable instrument to evaluate self-rated outcome in German-speaking patients with hand and wrist pathology.
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To investigate the association between articular chondrocalcinosis and calcification of the atlantoaxial region on a cervical computed tomography (CT) scan and to explore the relation between such calcifications and neck pain. ⋯ These results suggest that CPPD deposition disease frequently involves the cervical spine. Although such calcification often remains asymptomatic, it may be associated with attacks of acute neck pain with segmentary stiffness, fever, and an increased erythrocyte sedimentation rate, sometimes mimicking PMR and/or GCA or neurological symptoms.
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Systemic sclerosis is a rare and potentially devastating connective tissue disease. It is highly heterogeneous in terms of clinical presentation, extent and severity of organ involvement, immunologic abnormalities, and clinical course. ⋯ Results of these studies are presented and discussed, with emphasis on methodological limitations. Suggestions are made for the design, conduct, and reporting of further research on these themes.
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To adapt and validate the Hungarian version of the DASH and the shorter QuickDASH Outcome Measures and to establish their validity in patients with systemic sclerosis (SSc). ⋯ The Hungarian version of the DASH and QuickDASH demonstrated equivalent reproducibility, internal consistency and validity to the originals. The strong correlations of the DASH and QuickDASH with the HAQ-DI, and with the physical dimensions of the SF-36 show that the disability of the patient with SSc is predominantly caused by the functional impairment of the upper limb. Because both questionnaires were valuable tools for measuring upper extremity function and joint damage in SSc patients, we recommend the shorter and simpler QuickDASH for everyday clinical use.