Os autores relatam um caso de osteoartropatia hipertrófica do tipo primária em paciente de 29 anos de idade, com dores articulares e aumento de volume das extremidades há 15 anos. Ao exame físico apresentava baqueteamento dos dedos e unhas em "vidro de relógio". A osteoartropatia hipertrófica foi considerada como primária porque foram excluídas todas as causas da forma secundária. As principais alterações radiológicas foram: espessamento das camadas corticais dos ossos tubulares e reação periosteal contínua do tipo lamelar. Não foram notadas alterações dos espaços articulares.

No abstract

Rheumatoid cachexia can be defined as an involuntary loss of body cell mass, which predominates in skeletal muscle, but is also observed in the viscera and immune system. It occurs with little or no weight loss in the presence of stable or increased fat mass. The etiology is likely multifactorial, and involves excessive inflammatory cytokine production, namely excess tumor necrosis factor-α and interleukin-1β production, reduced peripheral insulin action, and low habitual physical activity. Cachexia occurs in active rheumatoid arthritis and even in the presence of disease control. In this article, we discuss the pathogenesis of rheumatoid cachexia, its clinical implications and potential therapies.

BACKGROUNDPulmonary arterial hypertension (PAH) is a rare and severe complication of SLE, with a worse prognosis and poor survival with estimated prevalence 0.5-17.5%. Divided in two cluster: vasculitic when acute associated to other systemic manifestations or vasculopathic when chronic and in isolated form. Lupus myocarditis is also a rare and severe manifestation. Both require rapid clinical suspicion, diagnosis and aggressive immunosuppressive treatment. CASE REPORTA 29-year-old female was initially evaluated in the emergency department describing oligoarthralgia of small joints, fatigue, loss weight of 26 kg in 5 months. Worsening of the symptoms in the last 4 days with facial swelling, malar rash and erythematous plaques on face and neck, oral ulcers and daily fever. At physical exam: recurrent bradycardia, but normal vital signs. The patient had previous diagnose of fibrous dysplasia in facial bones, and facial computed tomography (CT) scan demonstrated periorbital cellulitis and broad-spectrum antibiotics were initiated. Cardiomegaly in chest X-ray. Transthoracic echocardiogram with pericardiac effusion, moderated mitral insufficiency, mid-apical dyskinesia, right ventricular failure (30%), preserved left ventricular function. Estimated pulmonary arterial systolic of 43 mmHg. Chest CT angiography excluded pulmonary embolism and interstitial lung disease. In the meantime, immune panel was compatible is SLE with ANA 1/640 quasi-homogeneous pattern, anti-DNA antibodies positive (38.4), anti-RNP and IgM anticardiolipin in high titles, low c3 and c4 complement levels-and also elevated natriuretic peptide (1,090 pg/mL). Initiated intravenous methylprednisolone 1 g for 3 consecutive days and cyclophosphamide mensal pulse (0.5 g/m²/month) by 6 months followed prednisone 1 mg/kg/day (tapering 10 mg/month) plus hydroxychloroquine 5 mg/kg/day. CONCLUSIONAlthough the patient didn't present dyspnea and edema, fever, weight loss, bradycardia and cardiomegaly, the X-ray led us to clinical suspicion confirmed by echocardiogram. We describe a rare and severe case of vasculitic cluster of SLE-PAH associated to myocarditis and severe right ventricular failure. Rapid recognition and treatment were important for therapeutic success.

No abstract

No abstract

No abstract

Chronic inflammation presented in rheumatoid arthritis (RA) is associated with systemic manifestations, that include micronutrient deficiency, changes in body composition and cardiac morphofunctional alterations. The aim of this study is to evaluate the association of serum vitramin D and body composition with cardiac geometric pattern in patients with RA. Methods: 30 patients with RA were submitted to clinical, nutritional and biochemical evaluation. Bioimpedance analysis and dual‐energy X‐ray absorptiometry (DEXA) was performed to evaluate body composition. Transthoracic dopller‐ echocardiography was performed to evaluate left ventricle mass and relative wall thickness (RWT) in order to find cardiac geometry patterns. Patients were divided in Normal or abnormal pattern (concentric remodeling or concentric hypertrophy.) Results: The mean age was 53±9 years; 70% were woman, 43% presented with hypertension. All patients have been treated with methotrexate and a biological agent and presented with Disease activity Score (DAS28) 2,7±1,2. Serum vitamin D (ng/mL) was 30± 10,Body composition showed: BMI (kg/m2 ) 26±6, abdominal circumference (cm) 96±12, hand grip (kgf) 19±4,6 ; fat mass evaluated by DEXA (%) 33,8 ± 9,5 and by bioimpedance (%) 34,7±8,3; phase angle 6,6±0,6. Normal Cardiac geometric pattern was observed in 45%, concentric remodeling in 31% and concentric hypertrophy in 24%. Multiple logistic regression showed that hypertension and phase angle explain abnormalities in geometric pattern, even when adjusted by age, gender and hypertension. Vitamin D correlated with RWT, however it is not associated with geometric pattern. Conclusion: phase angle may be a potential marker of cardiac geometric pattern in patients with RA. Grant Funding Source: FAPESP: Fundação de Amparo à Pesquisa do Estado de São Paulo