Systemic sclerosis sine scleroderma: distinct features in a large Brazilian cohort.

OBJECTIVE: Systemic sclerosis sine scleroderma (ssSSc) is an infrequent SSc variant characterized by visceral and immunological manifestations of SSc in the absence of clinically detectable skin involvement. We sought to delineate the characteristics of ssSSc in a cohort of Brazilian patients and contrast them with those in the literature. METHODS: SSc patients seen at two academic medical centres in Brazil were retrospectively analysed. Patients were classified as ssSSc if they presented with RP, positive ANAs and at least one visceral involvement typical of SSc in the absence of skin thickening. Demographics, clinical and laboratory data were obtained by chart review. Literature review was performed by searching available original studies up until June 2012. RESULTS: Among the 947 consecutive patients with SSc, 79 (8.3%) were classified as ssSSc. Oesophagus was the most frequently affected organ (83.1%), followed by pulmonary involvement (63.2%). Compared with the limited cutaneous form of SSc, telangiectasia was the only variable significantly different after multivariate logistic regression analyses (odds ratio 0.46; 95% CI 0.27, 0.81). Compared with the diffuse cutaneous form of SSc, multivariate analyses revealed that ssSSc patients were less likely to be male (odds ratio 0.15; 95% CI 0.04, 0.57), have digital ulcers (odds ratio 0.26; 95% CI 0.13, 0.51) or anti-Scl70 antibodies (odds ratio 0.19; 95% CI 0.07, 0.55) and less frequently treated with CYC (odds ratio 0.23; 95% CI 0.12, 0.43). These features were comparable to those in the published literature. CONCLUSION: In this series, patients with ssSSc had a relatively mild disease with good prognosis.

The Influence of Time on the Epidemiology and Clinical Manifestations of Behçet's Disease in Brazil.

OBJECTIVE: Modifications in the severity and clinical expression of Behçet's disease (BD) have been described in some areas that are considered endemic for the disease. This study aims to evaluate the chronological changes in epidemiology and clinical characteristics of BD patients in a referral center in Brazil, which is considered a non-endemic area for the disease. METHODS: A descriptive and cross-sectional study involving BD patients divided into two groups: group 1 patients were diagnosed and followed between 1988 and 2010, and group 2 were diagnosed and followed between 2011 and 2022. RESULTS: No significant differences were found regarding gender and age at onset of symptoms between groups. We found a significant decrease in the frequency of bilateral ocular involvement, posterior uveitis, and retinal vasculitis. CONCLUSION: The demographic dates of this group of Brazilian BD patients remained similar over the last decade. Our study supports the notion that BD is becoming lighter in some regions. BD is a severe blinding disorder, and we found a lower frequency of ocular involvement over time. These findings may be attributed to a higher level of education of patients and a growing awareness of the disease. Newer immunomodulating and biologic agents may offer an improved prognosis in patients with BD with severe manifestations.

Systemic lupus erythematosus associated with vasculitic syndrome (Takayasu's arteritis).

A 43-year-old woman reported pain in the right hypochondrium, which had started 3 years before and had been worsening for the past few days. Claudication in the superior and inferior limbs, diffuse myalgia, dyspnea, precordialgia followed by dizziness and visual turbidity were added to the clinical picture. In the physical examination bilateral carotid bruit was observed, abdominal aorta murmur and the decrease of the right radial and left pedis pulses and arterial hypertension with difference in the diastolic pressure between limbs >10 mmHg was also observed. On cardiac catheterisation with aortography, right coronary with proximal parietal irregularities, slight pressure increase in right chambers and pulmonary artery, preserved left ventricle contractility, competent valves, carotid and subclavian partial obstruction, severe narrowing of the abdominal aorta below the diaphragm (80%) and right renal artery significant stenosis were observed. Takayasu's arteritis (TA) diagnosis was established according to the ACR criteria based on the clinical symptomatology, on physical and image test findings. Two years later she presented malar rash, photosensitivity, nephropathy, leukopenia, lymphopenia and hemolytic anemia confirming the systemic lupus erythematosus (SLE) diagnosis. TA coexisting with SLE has rarely been reported.

Clinical and laboratory profile of juvenile-onset systemic sclerosis in a Brazilian cohort.

Objective: To characterize the clinical and laboratory profile of juvenile-onset compared to adult-onset systemic sclerosis in a large Brazilian cohort. Methods: Retrospective analysis of a cohort of 1016 systemic sclerosis patients followed at the Scleroderma Outpatient Clinic from two referral university centers in Brazil. Patients were classified as systemic sclerosis according to the 1980 American College of Rhaumatology (ACR) criteria. Juvenile-onset systemic sclerosis was defined if age at onset was <16 years. Results: Thirty-one (3.1%) patients were classified as juvenile-onset systemic sclerosis. These patients were predominantly females (90.3%), Caucasians (71.0%), and presented diffuse systemic sclerosis (51.6%), with mean age at onset of 12.71 years. Compared to the adult-onset patients, juvenile onset was associated with diffuse systemic sclerosis (p < 0.001), calcinosis (p < 0.001), myositis (p = 0.050), and lower frequency of interstitial lung disease (p = 0.050), pulmonary hypertension (p = 0.035), and esophageal (p = 0.005) involvement. Conclusion: Juvenile-onset systemic sclerosis characterized a distinct clinical pattern in this large series of systemic sclerosis patients, since it was predominantly associated with diffuse systemic sclerosis without significant organ involvement.

Survival, causes of death, and prognostic factors in systemic sclerosis: analysis of 947 Brazilian patients.

OBJECTIVE: To analyze survival, prognostic factors, and causes of death in a large cohort of patients with systemic sclerosis (SSc). METHODS: From 1991 to 2010, 947 patients with SSc were treated at 2 referral university centers in Brazil. Causes of death were considered SSc-related and non-SSc-related. Multiple logistic regression analysis was used to identify prognostic factors. Survival at 5 and 10 years was estimated using the Kaplan-Meier method. RESULTS: One hundred sixty-eight patients died during the followup. Among the 110 deaths considered related to SSc, there was predominance of lung (48.1%) and heart (24.5%) involvement. Most of the 58 deaths not related to SSc were caused by infection, cardiovascular or cerebrovascular disease, and cancer. Male sex, modified Rodnan skin score (mRSS) > 20, osteoarticular involvement, lung involvement, and renal crisis were the main prognostic factors associated to death. Overall survival rate was 90% for 5 years and 84% for 10 years. Patients presented worse prognosis if they had diffuse SSc (85% vs 92% at 5 yrs, respectively, and 77% vs 87% at 10 yrs, compared to limited SSc), male sex (77% vs 90% at 5 yrs and 64% vs 86% at 10 yrs, compared to female sex), and mRSS > 20 (83% vs 90% at 5 yrs and 66% vs 86% at 10 yrs, compared to mRSS < 20). CONCLUSION: Survival was worse in male patients with diffuse SSc, and lung and heart involvement represented the main causes of death in this South American series of patients with SSc.