Translation, cultural adaptation and reproducibility of a Portuguese version of the Functional Index for Hand OsteoArthritis (FIHOA).

BACKGROUND: The Functional Index for Hand Osteoarthritis (FIHOA) is a simple, reliable, and reproducible specific instrument to evaluate hand OA that can be applied both in clinical practice and research protocols. In order to be used in Brazil, FIHOA has to be translated into Portuguese, culturally adapted and have the reliability of the translated FIHOA version tested, which is the purpose of this study. METHODS: The FIHOA was translated into Brazilian Portuguese and administered to 68 patients with hand OA recruited between May 2019 and February 2020. The test-retest was applied to 32 patients and the reliability was assessed using Spearman's correlation coefficient and intraclass correlation coefficient (ICC). The internal consistency reliability was evaluated using Cronbach's alpha. External construction validity was assessed using the Spearman's correlation test between FIHOA and pain, assessed with a Visual Analogue Scale (VAS), the Cochin Hand Functional Scale (CHFS) and Health Assessment Questionnaire (HAQ). RESULTS: The 30 participants that initially answered the translated version of the FiHOA did not report difficulties in understanding or interpreting the translated version. The test-retest reliability for the total score was strong (r = 0.86; ICC = 0.89). Mean differences (1.37 ± 0.68) using Bland Altman's analysis did not significantly differ from zero and no systematic bias was observed. Cronbach's alpha was also high (0.89) suggesting a strong internal coherence in the test items. There were also correlations between FIHOA and the CHFS (r = 0.88), HAQ (r = 0.64) and pain in the hands both at rest (r = 0.55) and in motion (r = 0.44). CONCLUSION: The translation of the FIHOA into Brazilian Portuguese proved a valid instrument for measuring the functional capacity of patients with hand OA who understand Brazilian Portuguese.

Renal involvement in antiphospholipid syndrome.

This is a review of scientific publications on renal involvement in antiphospholipid syndrome (APS), with focus on clinical and histopathological findings and treatment. A search for English-language articles on renal involvement in APS covering the period 1980-2017 was conducted in Medline/PubMed and Scopus databases using the MeSH terms "antiphospholipid syndrome", "antiphospholipid antibodies", "glomerulonephritis" and "thrombotic microangiopathy" (TMA). APS nephropathy is primarily the result of thromboses in renal arteries or veins, intraparenchymatous arteries and glomerular capillaries. On histology, APS nephropathy is characterized by TMA, but chronic vaso-occlusive lesions are also commonly observed (fibrous intimal hyperplasia, focal cortical atrophy, fibrous occlusions of arteries). Anticardiolipin and lupus anticoagulant are the most prevalent antibodies in patients with APS nephropathy. The spectrum of renal manifestations includes renal vein thrombosis, renal artery thrombosis/stenosis, TMA, increased allograft vascular thrombosis and malignant hypertension. Anticoagulation is the standard treatment of thrombotic events. In systemic lupus erythematosus (SLE) patients with antiphospholipid antibodies (aPL), kidney failure due to SLE nephritis (immune-complex disease) should be clearly distinguished from kidney failure due to APS-related TMA. In such cases, renal biopsy is mandatory. SLE nephritis requires immunosuppressive therapy, whereas APS nephropathy is usually treated with anticoagulants. Recently, eculizumab and sirolimus have been proposed as a rescue therapy. Based on our review, APS nephropathy appears to be a distinct clinical condition. TMA is a characteristic histopathological finding in APS and is strongly associated with the presence of aPL. This has important therapeutic implications and allows distinguishing APS nephropathy from lupus nephritis.

Granulomatosis with polyangiitis in Northeastern Brazil: study of 25 cases and review of the literature.

BACKGROUND: Little has been published about the epidemiology of Granulomatosis with polyangiitis (GPA) in South America, especially in the intertropical zone, and no epidemiological data from Brazil are available. The purpose of the present study was to draw a clinical and demographic profile of GPA patients living in Northeastern Brazil based on laboratory, histological and imaging findings, and evaluate the frequency of organic involvement. METHODS: Clinical, epidemiological and treatment data of GPA patients were collected retrospectively and compared with the literature. RESULTS: The cohort included 25 GPA patients (84% female) aged 45.8 ± 16.1 years. Renal and ear-nose-throat (ENT) manifestations were the most common (both 64%). One third (32%) of the patients had 24-h proteinuria > 1 g, 50% had creatinine clearance < 50 mL/min at the time of diagnosis, and 33% had recurrent kidney damage during disease progress. The affected organs included lungs (60%), joints (44%), skin (32%), peripheral nervous system (28%), eyes (28%) and heart (16%). ENT involvement (n = 16/64%) was less frequent in our region than in São Paulo (n = 115/85.8%). Renal (n = 16/64%) and pulmonary (n = 15/60%) involvement was less frequent in our region than in the U.K. (renal n = 30/90%; pulmonary n = 28/84.8%). CONCLUSION: Most of our patients were female, presented the generalized form and were diagnosed late. The frequency of the main clinical manifestations (ENT, renal and pulmonary) was lower than that observed at higher latitudes, suggesting the existence of a Northeast Brazilian clinical and epidemiological profile and adding to our knowledge of this rare condition.