Reversibility of pulmonary hypertension in systemic lupus erythematosus after induction immunosuppressive therapy: An inflammatory manifestation?

Objective: To evaluate the possible reversibility of PAH to a normopressoric state in SLE after induction immunosuppressive (IS) and predictors of response.

Methods: We retrospectively evaluated all SLE-PAH patients who underwent IS therapy at our center. PAH reversion was defined as the normalization of pulmonary arterial pressure (PAP), either by the presence of systolic PAP <40 mmHg on echocardiogram or mean PAP <20 mmHg on right heart catheterization (RHC).

Respiratory failure in systemic sclerosis.

Systemic sclerosis (SSc) can lead to dyspnea and respiratory failure through multiple mechanisms, making a precise diagnosis particularly challenging, especially amid the current COVID-19 pandemic. In this report, we present a case involving a 26-year-old female who had previously undiagnosed SSc. She experienced acute respiratory failure necessitating orotracheal intubation.

Critical digital ischaemia in systemic sclerosis exacerbated by multiple myeloma: A case report.

Introduction: The overlapping of systemic sclerosis with hematologic malignancy has been described previously in the literature. This case report presents a patient with systemic sclerosis and multiple myeloma who had severe digital ischaemia that culminated in the amputation of several fingers.

Case Report: A 65-year-old White female patient was diagnosed with limited systemic sclerosis in 2002, smouldering multiple myeloma IgG/kappa in 2017 and liver cirrhosis in 2018 due to autoimmune hepatitis.

Symptomatic fractures in systemic sclerosis: A case-control study.

This case-control study analyzed risk factors for symptomatic fractures in a group of 52 patients with systemic sclerosis compared with a group of 104 patients without fractures, matched for sex and age, who were attended at a single systemic sclerosis outpatient clinic from 2010 to 2020. Fractures affected predominantly vertebral (65.4%), rib (13.

Increment of immunogenicity after third dose of a homologous inactivated SARS-CoV-2 vaccine in a large population of patients with autoimmune rheumatic diseases.

Objective: To determine the immunogenicity of the third dose of CoronaVac vaccine in a large population of patients with autoimmune rheumatic diseases (ARD) and the factors associated with impaired response.

Methods: Adult patients with ARD and age-balanced/sex-balanced controls (control group, CG) previously vaccinated with two doses of CoronaVac received the third dose at D210 (6 months after the second dose). The presence of anti-SARS-CoV-2 S1/S2 IgG and neutralising antibodies (NAb) was evaluated previously to vaccination (D210) and 30 days later (D240).

Predictors of progression to systemic sclerosis: analysis of very early diagnosis of systemic sclerosis in a large single-centre cohort.

Objective: This study analysed the very early disease of SSc (VEDOSS) characteristics in a group of 217 patients with RP and at least one manifestation of SSc in search of predictors for the progression to SSc.

Methods: This was a cross-sectional single-centre analysis of patients presenting with RP with a specific SSc clinical manifestation or SSc autoantibody or SD pattern at nailfold capillaroscopy (SD-NFC), without skin involvement, who attended a scleroderma outpatient clinic between 2010 and 2019. The performance of VEDOSS and the importance of the combination of VEDOSS characteristics to predict the progression to SSc were evaluated.

SARS-CoV-2 vaccine in patients with systemic sclerosis: impact of disease subtype and therapy.

Objective: To analyse the safety, immunogenicity and factors affecting antibody response to Severe Acute Respiratory Syndrome-Coronavirus-2 (SARS-CoV-2) vaccination in patients with SSc.

Methods: This is a phase 4 prospective study within a larger trial of two doses of inactivated SARS-CoV-2 vaccine (CoronaVac) in 51 SSc patients compared with 153 controls. Anti-SARS-CoV-2-IgG and neutralizing antibodies (NAb) were assessed at each vaccine shot (D0/D28) and 6 weeks after the second dose(D69), only in individuals with negative baseline IgG/NAb and those who did not have coronavirus-19(COVID19) during follow-up.

Update on the Treatment of Pulmonary Arterial Hypertension.

In the last decades, important advances have been made in the treatment of pulmonary arterial hypertension (PAH), a severe, progressive, incurable, and potentially fatal disease. For an adequate therapy, correct hemodynamic diagnosis and etiology classification are fundamental. Many etiologies - rheumatic disease, portal hypertension, congenital heart diseases, schistosomiasis - require specific measures, in addition to drug therapy for PAH.

Pulmonary Embolism and Gas Exchange.

Acute pulmonary embolism (PE) impairs hemodynamics, gas exchange, and lung mechanical capacity. Considering PE pathophysiology, most attention has been paid to hemodynamic impairment. However, the most prevalent symptoms in PE patients come from gas exchange alterations, which have not been in the spotlight for many years.

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.

Eosinophilic fasciitis during pregnancy: case report and review of literature.

The authors describe the case of a 23-year-old woman who was referred to the rheumatologist due to symmetrical and progressive stiffness, induration, and swelling of arms and thighs at the 12th week of her first gestation. The characteristic clinical aspect of 'peau d'orange', associated to the histopathologic results of the deep biopsy of the skin confirmed the diagnosis of eosinophilic fasciitis. Treatment with oral prednisone, at an initial dose of 1 mg/kg/day, was effective and rapidly tapered to 10 mg/day till the birth of a healthy newborn.

Systemic sclerosis and silica exposure: a rare association in a large Brazilian cohort.

The objective of this study is to describe the characteristics of patients with Erasmus syndrome (ES) in a large SSc Brazilian cohort. Nine hundred and forty-seven SSc patients attended at the Scleroderma Outpatient Clinic at two academic medical centers in Brazil and classified as SSc according to the ACR/EULAR criteria were retrospectively studied. Information on demographics, clinical, and laboratory features was obtained by chart review.

Maternal and Neonatal Outcomes in 89 Patients with Takayasu Arteritis (TA): Comparison Before and After the TA Diagnosis.

Objective: To evaluate maternal and neonatal outcomes in patients before and after a diagnosis of Takayasu arteritis (TA).

Methods: Patients diagnosed with TA according to the American College of Rheumatology criteria were selected from the Vasculitis Outpatient Clinic of the Rheumatology Division. Healthy female staff members of this hospital of similar age and educational level were selected as the controls.

Spondyloptosis in athlete.

The adolescent athletes are at greater risk of low back pain and structural spine injuries. Spondylolysis is responsible for the majority of back pain cases in young athletes, rarely occurring in adults. We report a case of a 13-year-old judo female athlete, who came to our service with 5 months of progressive low back pain during training which was initially attributed to mechanical causes, without any further investigation by imaging methods.

Cutaneous mucinosis in mixed connective tissue disease.

Cutaneous mucinosis is a group of conditions involving an accumulation of mucin or glycosaminoglycan in the skin and its annexes. It is described in some connective tissue diseases but never in association with mixed connective tissue disease. This report concerns two cases of cutaneous mucinosis in patients with mixed connective tissue disease in remission; one patient presented the papular form, and the other reticular erythematous mucinosis.