Shrinking lung syndrome in primary Sjögren's syndrome: a case-based review.

Primary Sjögren's syndrome (pSS) is a systemic autoimmune disease that affects exocrine glands, mainly the salivary and lacrimal glands, leading to the development of sicca symptoms. Patients with pSS may also present with extraglandular manifestations, including lung involvement, estimated to occur in 9-24% of cases. Shrinking lung syndrome (SLS) is an uncommon respiratory complication primarily associated with systemic lupus erythematosus, with a prevalence of approximately 1% in these patients. It typically manifests as dyspnea, pleuritic chest pain, lung volume reduction, and a restrictive pattern on respiratory function tests. Cases reporting SLS with other connective tissue diseases, including pSS, are even rarer. Herein, we describe a case of a 57-year-old woman with a 10-year history of pSS who presented with dyspnea and pleuritic chest pain. After evaluation, the patient was diagnosed with SLS based on clinical, radiologic, laboratorial, and electrophysiologic characteristics. In addition, we identified and analyzed previously published cases of SLS in pSS. Treatment includes corticosteroids, immunosuppressants, and respiratory muscle training. This study highlights the importance of considering SLS in the differential diagnosis of patients with pSS and respiratory symptoms.

Scleromalacia perforans as an early manifestation of late-onset rheumatoid arthritis: a case-based review.

Rheumatoid arthritis is a chronic autoimmune disease that can affect different organs beyond the joints. Ocular involvement includes keratoconjunctivitis sicca, peripheral ulcerative keratitis (PUK), episcleritis, scleritis, anterior uveitis, and corneal impairment. The most severe form of scleritis, scleromalacia perforans, is an aggressive ophthalmic manifestation that can potentially lead to blindness, usually occurring in late stages of disease. We report a case of an elderly woman in which this severe ocular manifestation occurred early on disease onset, differing from most of the previously reported cases of scleromalacia perforans. Ocular symptoms started concomitantly with the polyarthritis and other extra-articular manifestations, including rheumatoid nodules and vasculitic skin lesions. Ocular disease progressed due to patient's loss to follow-up, requiring pulse therapy with methylprednisolone. However, despite treatment, right eye enucleation was required due to melting of the corneal patch with uveal exposition. The patient was then treated with rituximab with improvement of systemic disease. The present case reinforces that, although rare, this complication is severe and must be promptly diagnosed and aggressively treated to improve prognosis of ocular and systemic RA.

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.

Recommendations for evaluation and diagnosis of extra-glandular manifestations of primary sjogren syndrome: results of an epidemiologic systematic review/meta-analysis and a consensus guideline from the Brazilian Society of Rheumatology (articular, pulmonary and renal).

Sjogren's Syndrome (SS) is an autoimmune disease characterized by lymphocytic infiltration of the exocrine glands and other organs, associated with sicca syndrome but also with systemic involvement with varying degrees of severity. Despite their importance, these systemic manifestations are not routinely evaluated and there is no homogenous approach to their diagnosis or evaluation. To close this gap, a panel of experts from the Brazilian Society of Rheumatology conducted a systematic review and meta-analysis on the identification of epidemiologic and clinical features of these manifestations and made recommendations based on the findings. Agreement between the experts was achieved using the Delphi method. The first part of this guideline summarizes the most important topics, and 11 recommendations are provided for the articular, pulmonary, and renal care of SS patients.

Inactivated SARS-CoV-2 vaccine in primary Sjögren's syndrome: humoral response, safety, and effects on disease activity.

INTRODUCTION: There is no study specifically focused on SARS-CoV-2 vaccine in primary Sjögren's syndrome (pSS). OBJECTIVES: To assess the immunogenicity, safety, possible effects on disease activity, and autoantibody profile of the Sinovac-CoronaVac vaccine in pSS. METHODS: Fifty-one pSS patients and 102 sex- and age-balanced controls without autoimmune diseases were included in a prospective phase 4 trial of the Sinovac-CoronaVac vaccine (two doses 28 days apart, D0/D28). Participants were assessed in three face-to-face visits (D0/D28 and six weeks after the 2nd dose (D69)) regarding adverse effects; clinical EULAR Sjögren's Syndrome Disease Activity Index (clinESSDAI); anti-SARS-CoV-2 S1/S2 IgG (seroconversion (SC) and geometric mean titers (GMT)); neutralizing antibodies (NAb); and pSS autoantibody profile. RESULTS: Patients and controls had comparable female sex frequency (98.0% vs. 98.0%, p = 1.000) and mean age (53.5 ± 11.7 vs. 53.4 ± 11.4 years, p = 0.924), respectively. On D69, pSS patients presented moderate SC (67.5% vs. 93.0%, p < 0.001) and GMT (22.5 (95% CI 14.6-34.5) vs. 59.6 (95% CI 51.1-69.4) AU/mL, p < 0.001) of anti-SARS-CoV-2 S1/S2 IgG but lower than controls, and also, moderate NAb frequency (52.5% vs. 73.3%, p = 0.021) but lower than controls. Median neutralizing activity on D69 was comparable in pSS (58.6% (IQR 43.7-63.6)) and controls (64% (IQR 46.4-81.1)) (p = 0.219). Adverse events were mild. clinESSDAI and anti-Ro(SS-A)/anti-La(SS-B) levels were stable throughout the study (p > 0.05). CONCLUSION: Sinovac-CoronaVac vaccine is safe in pSS, without a deleterious impact on disease activity, and has a moderate short-term humoral response, though lower than controls. Thus, a booster dose needs to be studied in these patients. TRIAL REGISTRATION: ClinicalTrials.gov Identifier: NCT04754698. Key Points • Sinovac-CoronaVac vaccine is safe in pSS, without a detrimental effect on systemic disease activity, and has a moderate short-term humoral response • A booster dose should be considered in these patients.

Recommendations for evaluation and diagnosis of extra-glandular manifestations of primary Sjögren syndrome: results of an epidemiologic systematic review/meta-analysis and a consensus guideline from the Brazilian society of rheumatology (hepatic, gastrointestinal and pancreatic).

Sjogren's syndrome (SS) is an autoimmune disease characterized by lymphocytic infiltration of the exocrine glands and other organs, associated with sicca syndrome but also with systemic involvement with varying degrees of severity. Despite their importance, some systemic manifestations, mainly liver, gastrointestinal, and pancreatic are not routinely evaluated. To address these manifestations, the Sjögren's Syndrome Committee of the Brazilian Society of Rheumatology conducted a broad systematic review of the literature on studies investigating prevalence and diagnosis of these symptoms in Sjogren´s patients and made recommendations based on the findings. Agreement between the experts was achieved using the Delphi method. This is the second part of this guideline, providing 6 recommendations for liver, gastrointestinal, and pancreatic care of SS patients.

2021 recommendations of the Brazilian Society of Rheumatology for the gynecological and obstetric care of patients with Sjogren's syndrome.

Sjogren's syndrome (SS) is an autoimmune disease characterized by lymphocytic infiltration of the exocrine glands and other organs. Women with SS often experience gynecological symptoms due to the disease and need extra care regarding their sexual activity, reproductive health and during pregnancy, conditions that are not properly conducted in the clinical practice. To cover this gap, a panel of experts from the Brazilian Society of Rheumatology conducted a systematic review and meta-analysis on the identification of symptoms, diagnosis, monitoring, prognosis, and treatment of these manifestations. A Focus Group meeting was held and included experts in the field and methodologists, based on a previously developed script, with themes related to the objective of the study. The most important topics were summarized and 11 recommendations were provided.

Recommendations for evaluation and diagnosis of extra-glandular manifestations of primary Sjögren syndrome: results of an epidemiologic systematic review/ meta-analysis and a consensus guideline from the Brazilian society of rheumatology (hepatic, gastrointestinal and pancreatic)

Abstract Sjogren's syndrome (SS) is an autoimmune disease characterized by lymphocytic infiltration of the exocrine glands and other organs, associated with sicca syndrome but also with systemic involvement with varying degrees of severity. Despite their importance, some systemic manifestations, mainly liver, gastrointestinal, and pancreatic are not routinely evaluated. To address these manifestations, the Sjögren's Syndrome Committee of the Brazilian Society of Rheumatology conducted a broad systematic review of the literature on studies investigating prevalence and diagnosis of these symptoms in Sjogren´s patients and made recommendations based on the findings. Agreement between the experts was achieved using the Delphi method. This is the second part of this guideline, providing 6 recommendations for liver, gastrointestinal, and pancreatic care of SS patients.

Recommendations for evaluation and diagnosis of extra-glandular manifestations of primary sjogren syndrome: results of an epidemiologic systematic review/ meta-analysis and a consensus guideline from the Brazilian Society of Rheumatology (articular, pulmonary and renal)

Abstract Sjogren's Syndrome (SS) is an autoimmune disease characterized by lymphocytic infiltration of the exocrine glands and other organs, associated with sicca syndrome but also with systemic involvement with varying degrees of severity. Despite their importance, these systemic manifestations are not routinely evaluated and there is no homogenous approach to their diagnosis or evaluation. To close this gap, a panel of experts from the Brazilian Society of Rheumatology conducted a systematic review and meta-analysis on the identification of epidemiologic and clinical features of these manifestations and made recommendations based on the findings. Agreement between the experts was achieved using the Delphi method. The first part of this guideline summarizes the most important topics, and 11 recommendations are provided for the articular, pulmonary, and renal care of SS patients.

2021 recommendations of the Brazilian Society of Rheumatology for the gynecological and obstetric care of patients with Sjogren's syndrome

Abstract Sjogren's syndrome (SS) is an autoimmune disease characterized by lymphocytic infiltration of the exocrine glands and other organs. Women with SS often experience gynecological symptoms due to the disease and need extra care regarding their sexual activity, reproductive health and during pregnancy, conditions that are not properly conducted in the clinical practice. To cover this gap, a panel of experts from the Brazilian Society of Rheumatology conducted a systematic review and meta-analysis on the identification of symptoms, diagnosis, monitoring, prognosis, and treatment of these manifestations. A Focus Group meeting was held and included experts in the field and methodologists, based on a previously developed script, with themes related to the objective of the study. The most important topics were summarized and 11 recommendations were provided.

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.

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.

Vasculite mesentérica como única manifestação de atividade lúpica: a importância de um diagnóstico precoce / Mesenteric vasculitis as the only manifestation of lupic activity: the importance of a premature diagnosis

A vasculite mesentérica é uma grave complicação do lúpus eritematoso sistêmico (LES), pois é considerada uma importante causa de morbidade e mortalidade nesta doença. Na verdade, ela é também uma preocupação diagnóstica e terapêutica, e seu reconhecimento e intervenção precoce afetam diretamente o prognóstico. Este relato descreve uma paciente de 28 anos com diagnóstico de lúpus eritematoso sistêmico, que subitamente apresentou forte dor abdominal na ausência de outras manifestações de atividade de doença. Os procedimentos diagnósticos são discutidos e revisados. Os autores também enfatizam a necessidade de estabalecer um diagnóstico precoce dessa entidade para evitar complicações e uma pior evolução.