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. Following an extensive evaluation, the patient exhibited skin thickening, kidney failure, thrombocytopenia, microangiopathic anemia, and an antinuclear antibody with a nuclear fine speckled pattern at a titer of 1:320. A diagnosis of SSc complicated by scleroderma renal crisis (SRC) was established. The patient's condition improved after undergoing hemodialysis, receiving an angiotensin-converting enzyme inhibitor, and undergoing cyclophosphamide treatment. Subsequently, she demonstrated sustained improvement during a follow-up period of 20 months.

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). Patients with controlled disease suspended mycophenolate mofetil (MMF) for 7 days or methotrexate (MTX) for 2 weekly doses after vaccination. RESULTS: ARD (n=597) and CG (n=199) had comparable age (p=0.943). Anti-S1/S2 IgG seropositivity rates significantly increased from D210 (60%) to D240 (93%) (p<0.0001) in patients with ARD. NAb positivity also increased: 38% (D210) vs 81.4% (D240) (p<0.0001). The same pattern was observed for CG, with significantly higher frequencies for both parameters at D240 (p<0.05). Multivariate logistic regression analyses in the ARD group revealed that older age (OR=0.98, 95% CI 0.96 to 1.0, p=0.024), vasculitis diagnosis (OR=0.24, 95% CI 0.11 to 0.53, p<0.001), prednisone ≥5 mg/day (OR=0.46, 95% CI 0.27 to 0.77, p=0.003), MMF (OR=0.30, 95% CI 0.15 to 0.61, p<0.001) and biologics (OR=0.27, 95% CI 0.16 to 0.46, p<0.001) were associated with reduced anti-S1/S2 IgG positivity. Similar analyses demonstrated that prednisone ≥5 mg/day (OR=0.63, 95% CI 0.44 to 0.90, p=0.011), abatacept (OR=0.39, 95% CI 0.20 to 0.74, p=0.004), belimumab (OR=0.29, 95% CI 0.13 to 0.67, p=0.004) and rituximab (OR=0.11, 95% CI 0.04 to 0.30, p<0.001) were negatively associated with NAb positivity. Further evaluation of COVID-19 seronegative ARD at D210 demonstrated prominent increases in positivity rates at D240 for anti-S1/S2 IgG (80.5%) and NAb (59.1%) (p<0.0001). CONCLUSIONS: We provide novel data on a robust response to the third dose of CoronaVac in patients with ARD, even in those with prevaccination COVID-19 seronegative status. Drugs implicated in reducing immunogenicity after the regular two-dose regimen were associated with non-responsiveness after the third dose, except for MTX. Trial registration number NCT04754698.

Update on the Treatment of Pulmonary Arterial Hypertension. / Atualização no Tratamento da Hipertensão Arterial Pulmonar.

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. The specific therapy for PAH is based on medications that act on three pathophysiological pathways - prostacyclin, endothelin, and nitric oxide pathways. These drugs have multiple presentations (oral, intravenous, subcutaneous, and inhaled) and have changed the history of PAH. This review presents an overview of drug therapy strategies and different forms and peculiarities of PAH.

Muitos avanços ocorreram nas últimas décadas na terapêutica da hipertensão arterial pulmonar (HAP), uma doença grave, progressiva, incurável e potencialmente fatal. Para seu tratamento adequado, são fundamentais o diagnóstico hemodinâmico e a classificação de sua etiologia, em que várias delas (colagenoses, hipertensão portal, cardiopatia congênitas, esquistossomose) requerem medidas específicas, além do tratamento farmacológico característico para HAP. O tratamento com fármacos-alvo para HAP baseia-se em produtos farmacêuticos que interferem em três vias fisiopatológicas moleculares: da prostaciclina, da endotelina e do óxido nítrico. Tais fármacos apresentam múltiplas apresentações (oral, endovenosa, subcutânea e inalatória) e mudaram a história da HAP. Essas medicações e suas estratégias de uso, assim como particularidades das diferentes formas de HAP, são o foco desta revisão.

Atualização no Tratamento da Hipertensão Arterial Pulmonar / Update on the Treatment of Pulmonary Arterial Hypertension

Resumo Muitos avanços ocorreram nas últimas décadas na terapêutica da hipertensão arterial pulmonar (HAP), uma doença grave, progressiva, incurável e potencialmente fatal. Para seu tratamento adequado, são fundamentais o diagnóstico hemodinâmico e a classificação de sua etiologia, em que várias delas (colagenoses, hipertensão portal, cardiopatia congênitas, esquistossomose) requerem medidas específicas, além do tratamento farmacológico característico para HAP. O tratamento com fármacos-alvo para HAP baseia-se em produtos farmacêuticos que interferem em três vias fisiopatológicas moleculares: da prostaciclina, da endotelina e do óxido nítrico. Tais fármacos apresentam múltiplas apresentações (oral, endovenosa, subcutânea e inalatória) e mudaram a história da HAP. Essas medicações e suas estratégias de uso, assim como particularidades das diferentes formas de HAP, são o foco desta revisão.

Abstract 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. The specific therapy for PAH is based on medications that act on three pathophysiological pathways - prostacyclin, endothelin, and nitric oxide pathways. These drugs have multiple presentations (oral, intravenous, subcutaneous, and inhaled) and have changed the history of PAH. This review presents an overview of drug therapy strategies and different forms and peculiarities of PAH.

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. The disease data were obtained from an ongoing electronic database protocol. A standardized questionnaire, emphasizing gestational history, was applied to both groups. The prevalence of fetomaternal complications and disease variables were evaluated between the groups and a statistical analysis was performed. RESULTS: A total of 89 patients with TA (156 pregnancies) and 89 healthy controls (181 pregnancies) were evaluated. There were 75.6% pregnancies that occurred before the TA diagnosis (pre-TA group) and 24.3% after (post-TA group). In the pre-TA group, higher rates of hypertension (HTN; 27.1% vs 3.9%, p < 0.001), low birth weight (16.8% vs 6.5%, p = 0.012), and perinatal mortality (7.9% vs 0.7%, p = 0.003) were observed compared with healthy controls. The frequency of abortions and the average number of children were similar in both groups (p > 0.05). Further comparison of the pre- and post-TA groups revealed similar rates of HTN, abortion, and low birth weight, and higher rates of Cesarean delivery (p = 0.002), prematurity (p < 0.001), and infection (p = 0.045) in the latter group. CONCLUSION: Our study identified that patients with TA, even before the disease diagnosis, have a worse fetal outcome that is most likely associated with high rates of HTN. TA was identified as an additional differential diagnosis for HTN in pregnancy.

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. At admission, the patient had lumbar deformity, antalgic posture and bilaterally positive unipodalic lumbar hyperextension maneuver. After a research which showed spondyloptosis, the patient underwent surgery. In this article, we discuss, based on this case report, the diagnostic approach to low back pain in young athletes, since the complaint of chronic back pain can be a marker of a structural lesion that may be permanent and bring irreversible functional loss.

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. These are the first cases of mucinosis described in mixed connective tissue disease. Both cases had skin lesions with no other clinical or laboratorial manifestations, with clinical response to azathioprine in one, and to an association of chloroquine and prednisone in the other.

Espondiloptose em atleta / Spondyloptosis in athlete

Os atletas adolescentes estão sob maior risco de lombalgia e lesões estruturais da coluna. A espondilólise é responsável pela maioria das lombalgias em jovens esportistas e raramente ocorre em adultos. Relatamos o caso de uma paciente de 13 anos, atleta de judô, que chegou a nosso serviço com quadro de cinco meses de lombalgia progressiva durante os treinos, sendo inicialmente atribuída a causas mecânicas, sem que houvesse uma investigação mais detalhada por métodos de imagem. Na admissão já apresentava deformidade lombar, postura antálgica e manobra de hiperextensão lombar em unipodálico positiva bilateralmente. Realizou-se investigação, que evidenciou espondiloptose, sendo, então, submetida a tratamento cirúrgico. Com base neste relato de caso, discutimos a abordagem diagnóstica de lombalgia em atletas jovens, uma vez que a queixa de lombalgia crônica pode ser marcador de uma lesão estrutural, a qual pode ser definitiva e trazer perda funcional irreversível.

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. At admission, the patient had lumbar deformity, antalgic posture and bilaterally positive unipodalic lumbar hyperextension maneuver. After a research which showed spondyloptosis, the patient underwent surgery. In this article, we discuss, based on this case report, the diagnostic approach to low back pain in young athletes, since the complaint of chronic back pain can be a marker of a structural lesion that may be permanent and bring irreversible functional loss.

Cutaneous mucinosis in mixed connective tissue disease* / Mucinose cutanea na doenca mista do tecido conjuntivo

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. These are the first cases of mucinosis described in mixed connective tissue disease. Both cases had skin lesions with no other clinical or laboratorial manifestations, with clinical response to azathioprine in one, and to an association of chloroquine and prednisone in the other.

A mucinose cutânea é um grupo de condições em que há um acúmulo de mucina ou glicosaminoglicanos na pele e seus anexos. É descrita em algumas doenças do tecido conjuntivo, porem nunca em associação com doença mista do tecido conjuntivo. Relatamos dois casos de mucinose cutânea em pacientes com doença mista do tecido conjuntivo em remissão, um apresentava-se sob a forma papular e outro sob a forma reticular eritematosa de mucinose. Estes são os primeiros casos de mucinose descritos na doença mista do tecido conjuntivo. Ambos os casos apresentaram o quadro cutâneo de modo isolado, sem nenhuma outra manifestação clínico-laboratorial, havendo resposta à azatioprina em um e à cloroquina associada a prednisona no outro.