Clinical disease activity index applicability in lupus arthropathy: Unraveling underdiagnosed joint activity.

INTRODUCTION: Lupus arthropathy (LA) ranges from arthralgia and non-deforming arthritis to severe forms such as Jaccoud-type deformities and mutilating arthritis. Considering the evolving concept of LA, measuring arthritis activity in lupus patients may require a more practical and sensitive tool other than the classical composite scores. METHODS: In this cross-sectional study, we evaluated the articular pattern of a sample of SLE patients which were divided into those that scored in articular domain on Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) and those with activity arthritis using the Clinical Disease Activity Index (CDAI). After all, we analyzed the association between CDAI and arthritis by SLEDAI-2K as well as its association with the presence or not of Jaccoud-type arthropathy (JA). RESULTS: A total of 127 patients with SLE were evaluated. According to SLEDAI-2K, 17 (13.4%) patients have scored in its joint criteria and 32 patients (25.19%) were considered to have some articular activity by CDAI. A total of 16 patients (50%) who scored some activity on CDAI did not score in articular domain of SLEDAI-2K. Also, the presence of Jaccoud-type arthropathy was significantly associated with arthritis activity according to the CDAI score (p = .014) but not with SLEDAI-2K joint criteria (p = .524). CONCLUSION: The CDAI was not directly associated with the presence of arthritis by the joint criteria of SLEDAI-2K and the presence of JA was significantly associated with the CDAI but not with arthritis at SLEDAI-2K.

Cryofibrinogenemia: What Rheumatologists Should Know.

Cryofibrinogenemia refers to the presence of cryofibrinogen in plasma. This protein has the property of precipitating at lower temperatures. Cryofibrinogenemia is a rare disorder, clinically characterized by skin lesions, such as ulcers, necrosis, livedo reticularis, arthralgia, thrombosis, and limb ischemia. These features are most often observed in rheumatological practice and consist of differential diagnoses of antiphospholipid syndrome, primary vasculitis, thrombotic thrombocytopenic purpura, and cryoglobulinemia. Classical histopathological findings include the presence of thrombi within the lumen of blood vessels of the skin without vasculitis. To date, there are no validated classification criteria. Management includes corticosteroids, immunosuppressive therapy, anticoagulants, and fibrinolytic agents. This narrative review aims to make physicians, particularly rheumatologists, aware of the existence of this underdiagnosed condition. There are no epidemiological studies evaluating the prevalence of cryofibrinogenemia in different rheumatological disorders. Studies are also required to investigate if certain features of rheumatological diseases are related to the presence of cryofibrinogenemia.

Jaccoud-type lupus arthropathy.

BACKGROUND: Jaccoud's arthropathy (JA) is a condition characterized by joint deformities that have a "reducible" pattern, that is, they return to the normal appearance with a passive manoeuvre. JA was described in patients with rheumatic fever (RF) more than a century ago, and presently, the majority of the patients have systemic lupus erythematosus (SLE). PURPOSE: The aim of this review is to draw one attention to the epidemiology, pathogenesis, histopathology, clinical features, imaging, and management of JA in patients with SLE (Jaccoud-type lupus arthropathy). DATA COLLECTION: The search strategy included articles retrieved from PubMed utilizing the terms "lupus arthropathy", "lupus deforming arthropathy", "lupus hand", "lupus foot", "chronic postrheumatic arthropathy", "Jaccoud's" and "Jaccoud" from 1950 until March 2021, with no language restriction. RESULTS: The prevalence of Jaccoud-type arthropathy in SLE is approximately 5%. The aetiopathogenic mechanisms of JA are not yet known. The most common joint deformities of JA, are ulnar deviation, swan neck, and the "z" of the thumb. Unfortunately, none of the proposed classification criteria for JA have been validated so far. Characteristically, there is no bone erosion on plain radiographs of the joints, but more sensitive imaging methods, magnetic resonance imaging or high-performance ultrasound may reveal small bone erosions. There is no preventive measure against JA development, specific clinical treatment or convincing surgical approach for correcting the deformities. CONCLUSION: As daily activities and quality of life are compromised in patients with JA, other studies are urgently needed in this area.

Acute Peripheral and/or Cutaneous Ischemic Syndrome: What Rheumatologists Should Know.

ABSTRACT: In daily practice, the frequent appearance of limb and/or skin necrosis, which we term "acute peripheral and/or cutaneous ischemic syndrome" (APCIS), can be a manifestation of numerous underlying diseases, or it can sometimes be a clinical phenomenon whose etiology is undefined even after a wide investigation. The mechanisms for the development of APCIS include vessel wall abnormalities (atherosclerosis, vasculitis, and calciphylaxis), embolic processes (infectious endocarditis, atrial myxoma, and cholesterol emboli), local thrombotic injuries (genetic or acquired thrombophilias and heparin- and warfarin-induced ischemia), dysproteinemias (cryoglobulinemia and cryofibrinogenemia), or venous limb gangrene. Here, we report 5 illustrative cases of APCIS with different pathogenetic mechanisms, thereby highlighting some clinical conditions that cause APCIS that may be of special interest for rheumatologists, such as antiphospholipid syndrome, primary and secondary vasculitis, and cryoproteinemias. Furthermore, we describe a large spectrum of other causes of APCIS beyond the scope of rheumatology. Because there are no validated guidelines for APCIS, we tentatively propose an initial diagnostic workup and a therapeutic approach based on full-dose anticoagulation and immunosuppressive therapy.

Two-Year, Randomized, Controlled Trial of Belimumab in Lupus Nephritis.

BACKGROUND: In adults with active lupus nephritis, the efficacy and safety of intravenous belimumab as compared with placebo, when added to standard therapy (mycophenolate mofetil or cyclophosphamide-azathioprine), are unknown. METHODS: In a phase 3, multinational, multicenter, randomized, double-blind, placebo-controlled, 104-week trial conducted at 107 sites in 21 countries, we assigned adults with biopsy-proven, active lupus nephritis in a 1:1 ratio to receive intravenous belimumab (at a dose of 10 mg per kilogram of body weight) or matching placebo, in addition to standard therapy. The primary end point at week 104 was a primary efficacy renal response (a ratio of urinary protein to creatinine of ≤0.7, an estimated glomerular filtration rate [eGFR] that was no worse than 20% below the value before the renal flare (pre-flare value) or ≥60 ml per minute per 1.73 m2 of body-surface area, and no use of rescue therapy), and the major secondary end point was a complete renal response (a ratio of urinary protein to creatinine of <0.5, an eGFR that was no worse than 10% below the pre-flare value or ≥90 ml per minute per 1.73 m2, and no use of rescue therapy). The time to a renal-related event or death was assessed. RESULTS: A total of 448 patients underwent randomization (224 to the belimumab group and 224 to the placebo group). At week 104, significantly more patients in the belimumab group than in the placebo group had a primary efficacy renal response (43% vs. 32%; odds ratio, 1.6; 95% confidence interval [CI], 1.0 to 2.3; P = 0.03) and a complete renal response (30% vs. 20%; odds ratio, 1.7; 95% CI, 1.1 to 2.7; P = 0.02). The risk of a renal-related event or death was lower among patients who received belimumab than among those who received placebo (hazard ratio, 0.51; 95% CI, 0.34 to 0.77; P = 0.001). The safety profile of belimumab was consistent with that in previous trials. CONCLUSIONS: In this trial involving patients with active lupus nephritis, more patients who received belimumab plus standard therapy had a primary efficacy renal response than those who received standard therapy alone. (Funded by GlaxoSmithKline; BLISS-LN ClinicalTrials.gov number, NCT01639339.).

Scleroderma with Acro-Osteolysis and Papular Mucinosis Resembling Multicentric Reticulohistiocytosis.

OBJECTIVES: We describe a case of systemic sclerosis (SS) with acro-osteolysis associated with cutaneous mucinosis, usually characterized by mucin deposition in the skin. The main differential diagnosis was multicentric reticulohistiocytosis due to the presentation of papulonodular skin lesions. MATERIALS AND METHODS: A physical examination, imaging studies and laboratory tests were performed. RESULTS: Distal bone resorption was evident on plain radiographs, and skin biopsy confirmed mucinosis. The SS diagnosis was based on the clinical features, high levels of antinucleolar antibodies and typical nailfold capillaroscopy findings. CONCLUSION: To the best of our knowledge, this is the first description of cutaneous mucinosis accompanying SS with acro-osteolysis. LEARNING POINTS: Cutaneous mucinosis is rarely associated with systemic sclerosis.Systemic sclerosis with cutaneous mucinosis may resemble multicentric reticulohistiocytosis.

The Influence of Hand Dominance on the Degree of Deformities in Patients With Systemic Lupus Erythematosus and Jaccoud Arthropathy.

BACKGROUND/OBJECTIVE: Joint deformities in Jaccoud arthropathy (JA), associated with systemic lupus erythematosus (SLE), can lead to a reduction in articular function and an impaired quality of life. There is controversy in the literature as to whether hand dominance contributes to deformities observed in rheumatoid arthritis and other forms of rheumatism. The purpose of this study was to assess whether hand dominance has any influence on the degree of deformity or joint range of motion in patients with JA associated with SLE. METHODS: This was a pilot cross-sectional study of 22 female patients (mean age, 46.18 ± 12.61 years) with both SLE and JA. Each patient's hand grip and hand and wrist joint range of motion were assessed by the same examiner. The Wilcoxon test was used to compare the median values of the angles and muscular strength found between the sides. RESULTS: All the study participants were right-side dominant. The median muscle strength of the right and left hands was 13.25 kilogram-force (KgF) (range, 7.00-18.00 KgF) and 10.50 KgF (range, 7.50-17.25 KgF), respectively. Both the fingers of the right and left hands had a median ulnar drift of 10.00°; however, their ranges differed (right, 4.50° to 20.00°; left: 0.00° to 15.50°). No statistically significant differences were found in the extension angulation of the proximal interphalangeal joints or the flexion of the distal interphalangeal joints of the second to fifth fingers, between the dominant and nondominant hands. CONCLUSIONS: Hand dominance does not seem to influence the degree of JA deformities in patients with SLE.

Association between Takayasu arteritis and latent or active Mycobacterium tuberculosis infection: a systematic review.

Takayasu arteritis (TA) is a granulomatous vasculitis of large vessels with unknown aetiopathogenesis. An association between TA and tuberculosis (TB) has been suggested by several authors. The objective of this study was to perform a systematic review of the literature on the association of Mycobacterium tuberculosis (MT) infection in patients with TA. The research was conducted using the PUBMED/Medline and LILACS databases including studies published until June 2019 and using the descriptors "takayasu arteritis", "tuberculosis", "mycobacterium tuberculosis", "purified protein derivate" (PPD), "mantoux test", "quantiferon tb gold", and "interferon gamma release assay" (IGRA). A total of 113 publications were found, but only 38 publications were included after the pre-established criteria were applied. The results were divided into (1) active TB in adolescents with TA: 13 cases; (2) active TB in adults with TA: 116 cases, with a prevalence ranging from 6.3 to 20%, including a South Korean study of 267 cases of TA that found a prevalence of active TB of 17.7%; (3) latent TB in TA patients: the most widely used method was PPD, and only one study compared PPD with IGRA; the prevalence ranged from 20 to 82%; (4) findings that indicate TB in arterial biopsy or autopsies: 5 studies, with different results ranging from an absence of MT DNA in the aortic tissue to 70% positivity; and (5) immunological studies that evaluated the presence of antimycobacterial antibodies and heat shock proteins in TA patients. Although most of the studies show a high prevalence of TB, it is not possible to establish a causal relationship. We suggest that greater care be taken with latent TB screening in patients who are TA candidates for immunosuppressive therapy.

Clinical and laboratory aspects of dyslipidemia in Brazilian women with systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is associated with dyslipidemia, atherosclerosis, and cardiovascular disease. In this study, we investigated the presence of dyslipidemia in Brazilian SLE patients by evaluating their lipid profile and immune status, including the production of autoantibodies and cytokines involved in atherogenesis. Ninety-four female SLE patients participated in this study and, based on their lipid profile, were classified as dyslipidemic or not. All were tested for antinuclear antibodies (ANAs), antiphospholipid antibodies, and autoantibodies to extractable nuclear antigens and double-stranded DNA. Serum levels of apolipoproteins A and B, C3, C4, and C-reactive protein were measured, as well as serum levels of interleukin (IL)-6, tumor necrosis factor (TNF)-α, and IL-10. Lupus activity was scored according to the Systemic Lupus Erythematosus Disease Activity Index 2000. Sixty-nine patients (73.4%) had dyslipidemia, and the remaining 25 patients (26.6%) were non-dyslipidemic. Lupus activity was correlated with non-high-density lipoprotein cholesterol and triglyceride (TG) levels (non-HDL-C, r = 0.34 and p = 0.0043 and r = 0.46 and p < 0.0001, respectively). Atherogenic indexes apolipoprotein B/apolipoprotein A and TG:HDL-C ratios were higher in dyslipidemic women, and TG:HDL was correlated with disease activity (r = 0.40, p = 0.0007). IL-6, TNF-α, and IL-10 levels were similar between groups; however, a positive correlation between IL-6 and CRP levels was only observed in the group with dyslipidemia (r = 0.55, p < 0.0001). Female Brazilian SLE patients present a high prevalence of dyslipidemia and exhibit a higher risk of cardiovascular diseases as compared with female SLE patients without dyslipidemia and healthy individuals.

Quality of life in spondyloarthritis: analysis of a large Brazilian cohort.

OBJECTIVE: To analyze quality of life and demographic and clinical variables associated to its impairment in a large Brazilian cohort of patients with spondyloarthritis (SpA). METHODS: A common protocol of investigation was applied to 1465 Brazilian patients classified as SpA according to the European Spondyloarthropaties Study Group (ESSG) criteria, attended at 29 reference centers for Rheumatology in Brazil. Clinical and demographic variables were recorded. Quality of life was analyzed through the Ankylosing Spondylitis Quality of Life (ASQoL) questionnaire. RESULTS: The mean ASQoL score was 7.74 (+5.39). When analyzing the specific diseases in the SpA group, the ASQoL scores did not present statistical significance. Demographic data showed worse scores of ASQoL associated with female gender (p=0.014) and African-Brazilian ethnicity (p<0.001). The analysis of the clinical symptoms showed that buttock pain (p=0.032), cervical pain (p<0.001) and hip pain (p=0.001) were statistically associated with worse scores of ASQoL. Continuous use of nonsteroidal anti-inflammatory drugs (p<0.001) and biologic agents (p=0.044) were associated with higher scores of ASQoL, while the other medications did not interfere with the ASQoL scores. CONCLUSION: In this large series of patients with SpA, female gender and African-Brazilian ethnicity, as well as predominant axial symptoms, were associated with impaired quality of life.

Qualidade de vida nas espondiloartrites: análise de uma grande coorte brasileira / Quality of life in spondyloarthritis: analysis of a large Brazilian cohort

Resumo Objetivo: Analisar as variáveis demográficas e clínicas associadas à diminuição da qualidade de vida em uma grande coorte brasileira de pacientes com espondiloartrite (EpA). Métodos: Foi aplicado um protocolo de pesquisa único a 1.465 pacientes brasileiros classificados como tendo EpA de acordo com os critérios do European Spondyloarthropaties Study Group (ESSG), atendidos em 29 centros de referência em reumatologia do Brasil. Foram registradas as variáveis clínicas e demográficas. A qualidade de vida foi analisada por meio do questionário Ankylosing Spondylitis Quality of Life (ASQoL). Resultados: A pontuação média do ASQoL foi de 7,74 (+ 5,39). Ao analisar doenças específicas no grupo de EpA, as pontuações do ASQoL não apresentaram diferença estatisticamente significativa. Os dados demográficos mostraram piores escores de ASQoL associados ao gênero feminino (p = 0,014) e etnia negra (p < 0,001). Quanto aos sintomas clínicos, a dor na região glútea (p = 0,032), a dor cervical (p < 0,001) e a dor no quadril (p = 0,001), estiveram estatisticamente associadas a piores escores no ASQoL. O uso contínuo de fármacos anti-inflamatórios não esteroides (p < 0,001) e agentes biológicos (p = 0,044) esteve associado a escores mais elevados de ASQoL, enquanto outros medicamentos não interferiram nos escores do ASQoL. Conclusão: Nesta grande série de pacientes com EpA, o sexo feminino e a etnia negra, bem como sintomas predominantemente axiais, estiveram associados a uma qualidade de vida reduzida.

Abstract Objective: To analyze quality of life and demographic and clinical variables associated to its impairment in a large Brazilian cohort of patients with spondyloarthritis (SpA). Methods: A common protocol of investigation was applied to 1465 Brazilian patients classified as SpA according to the European Spondyloarthropaties Study Group (ESSG) criteria, attended at 29 reference centers for Rheumatology in Brazil. Clinical and demographic variables were recorded. Quality of life was analyzed through the Ankylosing Spondylitis Quality of Life (ASQoL) questionnaire. Results: The mean ASQoL score was 7.74 (± 5.39). When analyzing the specific diseases in the SpA group, the ASQoL scores did not present statistical significance. Demographic data showed worse scores of ASQoL associated with female gender (p = 0.014) and African-Brazilian ethnicity (p < 0.001). Regarding clinical symptoms, buttock pain (p = 0.032), cervical pain (p < 0.001) and hip pain (p = 0.001), were statistically associated with worse scores of ASQoL. Continuous use of nonsteroidal anti-inflammatory drugs (p < 0.001) and biologic agents (p = 0.044) were associated with higher scores of ASQoL, while the other medications did not interfere with the ASQoL scores. Conclusion: In this large series of patients with SpA, female gender and African-Brazilian ethnicity, as well as predominant axial symptoms, were associated with impaired quality of life.

High Avidity dsDNA Autoantibodies in Brazilian Women with Systemic Lupus Erythematosus: Correlation with Active Disease and Renal Dysfunction.

We investigated in Brazilian women with SLE the prevalence and levels of high avidity (HA) dsDNA antibodies and tested their correlation with lupus activity and biomarkers of renal disease. We also compared these correlations to those observed with total dsDNA antibodies and antibodies against nucleosome (ANuA). Autoantibodies were detected by ELISA, while C3 and C4 levels were determined by nephelometry. Urine protein/creatinine ratio was determined, and lupus activity was measured by SLEDAI-2K. The prevalence of total and HA dsDNA antibodies was similar to but lower than that verified for ANuA. The levels of the three types of antibodies were correlated, but the correlation was more significant between HA dsDNA antibodies and ANuA. High avidity dsDNA antibodies correlated positively with ESR and SLEDAI and inversely with C3 and C4. Similar correlations were observed for ANuA levels, whereas total dsDNA antibodies only correlated with SLEDAI and C3. The levels of HA dsDNA antibodies were higher in patients with proteinuria, but their levels of total dsDNA antibodies and ANuA were unaltered. High avidity dsDNA antibodies can be found in high prevalence in Brazilian women with SLE and are important biomarkers of active disease and kidney dysfunction.

Severe Jaccoud's arthropathy in systemic lupus erythematosus.

Jaccoud's arthropathy (JA) is a clinical situation nowadays present mostly in systemic lupus erythematosus (SLE). It is characterized by the presence of joint deformities such as "swan neck," ulnar deviation and "Z-thumb" resembling rheumatoid arthritis (RA) but that are passively correctable and without bone erosion on plain radiographs. From our cohort of SLE patients with JA, we selected a subgroup with a more severe form of this arthropathy and looked at their clinical and laboratory profile as well as studied the magnetic resonance imaging (MRI) findings or ultrasound (US) obtained from the hand with most evident deformities. Seven SLE patients with a severe form of JA were identified. All seven patients have "swan neck," ulnar deviation and "Z-thumb" deformities. Two out of seven had "mutilans-type JA" and four had fixed deformities in the metacarpophalangeal (MCP) joints. The MRI of the hand with more evident deformity clinically performed in six cases and US performed in one case showed mild synovitis in five and moderate synovitis in two patients, mild flexor tenosynovitis in six and severe tenosynovitis in one. Only two small bone erosions were observed in the second and third MCP joints of one patient with moderate synovitis. Severe JA compromises the functional capacity of the joints and imposes the risk of misdiagnosis of RA. With the improvement of the survival rate of SLE and the lack of specific prophylactic or therapeutical measures for JA, it is reasonable to assume that more and more cases of severe JA are going to be identified.

Ultrasound evaluation of joints in systemic lupus erythematosus: a systematic review.

OBJECTIVES: The aim of this systematic review is to describe the scientific evidence regarding sonographic findings of joints in SLE patients. METHODS: Seven databases were searched (PubMed, ScienceDirect, Scopus, Cochrane, EMBASE, LILACS, and SciELO) for articles from 1950 to January 2015. The keywords used for selecting articles include "lupus", "ultrasound imaging", "ultrasonography", "synovitis", "tenosynovitis", and "arthritis". RESULTS: A total of 12 articles were included in the final analysis. In total, 610 SLE patients and 1,091 joints were studied. Most patients underwent bilateral joint examination by US. A total of 888 hands and wrists, 154 ankles/feet, and 56 knees were examined. Effusion was identified in 602 joints, synovitis in 213, tenosynovitis in 210, synovial hypertrophy in 150, and bone erosions in 73 cases. The majority of the studies demonstrated higher frequency of musculoskeletal abnormalities on US than those observed on physical examination. CONCLUSION: US seems to be a valuable tool to identify subclinical joint manifestations in SLE. Prospective studies are necessary to determine if those patients with subclinical joint abnormalities have a higher risk for the development of chronic deformities as those seen in Jaccoud's Arthropathy. KEY POINTS: • Musculoskeletal involvement occurs in more than 90% of SLE cases. • Arthralgia or tender/swollen joints found on physical examination showed more US findings. • Patients without joint symptoms or physical examinations changes showed musculoskeletal sonographic findings. • US became a useful tool for rheumatologists. • A substantial number of asymptomatic patients show abnormalities at musculoskeletal US.

Avaliação do desempenho do BASDAI (Bath Ankylosing Spondylitis Disease Activity Index) numa coorte brasileira de 1492 pacientes com espondiloartrites: dados do Registro Brasileiro de Espondiloartrites (RBE) / Evaluation of performance of BASDAI (Bath Ankylosing Spondylitis Disease Activity Index) in a Brazilian cohort of 1492 patients with spondyloarthritis: data from the Brazilian Registry of Spondyloarthritides (RBE)

Objetivo Avaliar os resultados da aplicação do Índice de Atividade de Doença da Espondilite Anquilosante de Bath (BASDAI) numa série de pacientes brasileiros com EpA e estabelecer suas correlações com as variáveis específicas do grupo. Métodos Um protocolo comum de investigação foi prospectivamente aplicado em 1.492 pacientes brasileiros classificados como EpA pelos critérios do Grupo Europeu de Estudo das Espondiloartropatias (ESSG), acompanhados em 29 centros de referência em reumatologia no Brasil. Variáveis clínicas, demográficas e índices de doença foram colhidos. Os valores totais do BASDAI foram comparados com a presença das diferentes variáveis. Resultados O valor médio do BASDAI foi de 4,20 ± 2,38. Os escores médios do BASDAI foram mais elevados nos pacientes com forma clínica combinada, comparado às formas axiais e periféricas isoladas, nos pacientes do sexo feminino e nos sedentários. Com relação ao componente axial, valores mais altos do BASDAI estiveram significativamente associados à lombalgia inflamatória, à dor alternante em nádegas, à dor cervical e ao acometimento de coxofemorais. Houve associação estatística entre os valores do BASDAI e o comprometimento periférico, relacionado ao número de articulações inflamadas, tanto dos membros inferiores quanto dos membros superiores, e às entesites. A positividade do HLA-B27 e a presença de manifestações extra-articulares não estiveram correlacionadas com os valores médios do BASDAI. Valores mais baixos do BASDAI estiveram associados ao uso de agentes biológicos (p < 0,001). Conclusão Nesta série heterogênea de pacientes brasileiros com EpA, o BASDAI conseguiu demonstrar “atividade de doença” tanto nos pacientes com acometimento axial quanto naqueles com envolvimento periférico. .

Objective To analyze the results of the application of the Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) in a large series of Brazilian patients with the diagnosis of SpA and establish its correlations with specific variables into the group. Methods A common protocol of investigation was prospectively applied to 1492 Brazilian patients classified as SpA according to the European Spondyoarthropathies Study Group (ESSG), attended at 29 referral centers of Rheumatology in Brazil. Clinical and demographic variables, and disease indices (BASDAI, Basfi, Basri, Mases, ASQol) were applied. The total values of BASDAI were compared to the presence of the different variables. Results The mean score of BASDAI was 4.20 ± 2.38. The mean scores of BASDAI were higher in patients with the combined (axial + peripheral + entheseal) (4.54 ± 2.38) clinical presentation, compared to the pure axial (3.78 ± 2.27) or pure peripheral (4.00 ± 2.38) clinical presentations (P < 0.001). BASDAI also presented higher scores associated with the female gender (P < 0.001) and patients who did not practice exercises (P < 0.001). Regarding the axial component, higher values of BASDAI were significantly associated with inflammatory low back pain (P < 0.049), alternating buttock pain (P < 0.001), cervical pain (P < 0.001) and hip involvement (P < 0.001). There was also statistical association between BASDAI scores and the peripheral involvement, related to the lower (P = 0.004) and upper limbs (P = 0.025). The presence of enthesitis was also associated to higher scores of BASDAI (P = 0.040). Positive HLA-B27 and the presence of cutaneous psoriasis, inflammatory bowel disease, uveitis and urethritis were not correlated with the mean scores of BASDAI. Lower scores of BASDAI were associated with the use of biologic agents (P < 0.001). Conclusion In this heterogeneous Brazilian series of SpA patients, BASDAI was able to demonstrate “disease ...

[Evaluation of performance of BASDAI (Bath Ankylosing Spondylitis Disease Activity Index) in a Brazilian cohort of 1,492 patients with spondyloarthritis: data from the Brazilian Registry of Spondyloarthritides (RBE)]. / Avaliação do desempenho do BASDAI (Bath Ankylosing Spondylitis Disease Activity Index) numa coorte brasileira de 1.492 pacientes com espondiloartrites: dados do Registro Brasileiro de Espondiloartrites (RBE).

OBJECTIVE: To analyze the results of the application of the Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) in a large series of Brazilian patients with the diagnosis of SpA and establish its correlations with specific variables into the group. METHODS: A common protocol of investigation was prospectively applied to 1492 Brazilian patients classified as SpA according to the European Spondyoarthropathies Study Group (ESSG), attended at 29 referral centers of Rheumatology in Brazil. Clinical and demographic variables, and disease indices (BASDAI, Basfi, Basri, Mases, ASQol) were applicated. The total values of BASDAI were compared to the presence of the different variables. RESULTS: The mean score of BASDAI was 4.20 ± 2.38. The mean scores of BASDAI were higher in patients with the combined (axial + peripheral + entheseal) (4.54 ± 2.38) clinical presentation, compared to the pure axial (3.78 ± 2.27) or pure peripheral (4.00 ± 2.38) clinical presentations (p<0.001). BASDAI also presented higher scores associated with the female gender (p<0.001) and patients who did not practice exercises (p < 0.001). Regarding the axial component, higher values of BASDAI were significantly associated with inflammatory low back pain (p<0.049), alternating buttock pain (p<0.001), cervical pain (p<0.001) and hip involvement (p<0.001). There was also statistical association between BASDAI scores and the peripheral involvement, related to the lower (p=0.004) and upper limbs (p=0.025). The presence of enthesitis was also associated to higher scores of BASDAI (p=0.040). Positive HLA-B27 and the presence of cutaneous psoriasis, inflammatory bowel disease, uveitis and urethritis were not correlated with the mean scores of BASDAI. Lower scores of BASDAI were associated with the use of biologic agents (p<0.001). CONCLUSION: In this heterogeneous Brazilian series of SpA patients, BASDAI was able to demonstrate "disease activity" in patients with axial as well as peripheral disease.