Adverse Impact of Systemic Lupus Erythematosus on Pregnancy Outcomes: A Saudi Arabia Retrospective Multi-Center Study.

Objective: The current study aimed to determine the pregnancy outcomes complications in patients with SLE and its association with clinical, laboratory variables, disease activity, and medication use in the Saudi population, as well as pregnancy effect on disease activity. Methods: A multicenter study included pregnant female patients with Systemic Lupus Erythematosus (SLE) from three tertiary centers in Saudi Arabia. The demographics, clinical, and laboratory variables, SLE disease activity index (SLEDAI), medication before, during, and after pregnancy, planned pregnancy, pregnancy-related outcomes, and complications in comparison to age-matched healthy female controls were noted. Results: A total of 66 pregnant patients with SLE and 93 healthy age-matched pregnant controls were included in the study. A total of 77.3% had SLEDAI-2K ≤ 4 before conception, and 84.85% of pregnancies were planned. Age of conception, cesarean section, miscarriage, and low birth weight were statistically significant (p <0.05) higher in SLE patients than in healthy controls. Among all clinical and laboratory variables, SLEDAI-2K > 4 and active lupus nephritis during pregnancy were statistically associated with adverse outcomes (p <0.05), history of lupus nephritis was not associated with statistically adverse pregnancy outcomes. Higher SLEDAI-2K > 4 was an independent risk at least 4.87 times higher association with adverse pregnancy outcomes. (p <0.05). Conclusion: SLE is intricately connected with unfavorable pregnancy outcomes. The preconception of high disease activity stands as a pivotal risk factor for adverse outcomes. Despite the disease remission and meticulous planning, SLE patients frequently grapple with disease exacerbations during pregnancy, culminating in unexpected and unfavorable pregnancy-related outcomes. This underscores the intricate and multifaceted nature of managing SLE during gestation.

Spontaneous Pneumomediastinum and Subcutaneous Emphysema in Dermatomyositis: A Case Series and Literature Review.

Background: Spontaneous pneumomediastinum and subcutaneous emphysema are rare and serious complications of dermatomyositis (DM). Case Presentation: Our article presents two clinically heterogeneous cases of DM who developed pneumomediastinum and subcutaneous emphysema. The first was a 24-year-old lady with a recently diagnosed DM. She developed rapidly progressive pneumonia, interstitial lung disease (ILD), pneumomediastinum, subcutaneous emphysema, and acute respiratory distress syndrome (ARDS). She died despite treatment with steroids and immunosuppressants (methotrexate and mycophenolate mofetil (MMF)). The second was a 30-year-old man diagnosed with amyopathic DM. He developed pneumomediastinum prior to ILD, which worsened over time, and subcutaneous emphysema evolved. However, he recovered completely after corticosteroid, MMF, and rituximab. Conclusion: Spontaneous pneumomediastinum and subcutaneous emphysema may complicate DM. Corticosteroids, immunosuppressants, and respiratory support are the mainstay of management for these conditions. Though they were reported to carry a poor prognostic value, the course and outcome are highly variable among the cases.

Dermatomyositis Flare After a COVID-19 Infection Successfully Treated with Rituximab: A Case Report and Literature Review.

Introduction: Since the beginning of the coronavirus disease 2019 (COVID-19) pandemic, there has been a sudden surge in the incidence of several immune-mediated diseases, including dermatomyositis. The reported cases of COVID-19-related dermatomyositis are heterogeneous in their clinical presentation and implemented therapies. Case Study: We report a 23-year-old female patient diagnosed with a 3-year history of dermatomyositis. She has been well-controlled on maintenance therapy. However, 6 weeks after a mild COVID-19 infection, she developed a dermatomyositis flare. She improved only after aggressive treatment with pulse steroids, intravenous immunoglobulin, and rituximab. Conclusion: Exacerbation of dermatomyositis can be encountered following a COVID-19 infection, even if the infection is mild. Aggressive therapy should be considered in such cases. The prognosis, however, is generally favorable.

COVID-19 breakthrough infections in rheumatic diseases patients after vaccination.

BACKGROUND: Rheumatic diseases patients receiving Rituximab had severe COVID-19 disease. Although they had impaired humoral immune responses following COVID-19 vaccine, they had preserved cellular immune responses. Waning of COVID-19 antibody responses was observed within six months post vaccination among immunocompromised patients. Recent reports showed fatal outcome of breakthrough SARS-CoV-2 infections among vaccinated high-risk rheumatic diseases patients receiving Rituximab. SAR-CoV-2 serological tests were not performed. OBJECTIVE: Evaluation of COVID-19 vaccine humoral responses and breakthrough infections among low risk fully vaccinated rheumatic patients during the Delta Variant Era. METHODS: A case series of 19 fully vaccinated patients with rheumatic diseases were followed to determine post vaccine SARS-CoV-2 neutralizing antibody titers and to monitor the development of breakthrough infections up to eight months post vaccine at our tertiary care center in Jeddah, Saudi Arabia from 1st April until 30th November 2021. RESULTS: The mean age of patients was 49 years old. 10% of patients were receiving Rituximab. 73% of patients had positive SARS-CoV-2 serological testing post second vaccine. Two mild breakthrough COVID-19 infections were diagnosed six months post second dose of vaccine. Patients were less than 65 years, did not receive Rituximab, did not have interstitial lung diseases and had positive post vaccine serological testing. CONCLUSIONS: We demonstrated high SARS-CoV-2 neutralizing antibodies seroprevalence and self-limiting breakthrough infections in low risk rheumatic diseases patients during the Delta Era. Future studies are needed to study the outcome of rheumatic diseases patients in the Era of Omicron in view of viral immune escape responses.

Severe Primary Raynaud's Disease Treated with Rituximab.

Raynaud's phenomenon refers to reversible spasms of the peripheral arterioles that can be primary Raynaud's phenomenon (PRP) or secondary Raynaud's phenomenon (SRP) to underlying connective tissue disease, both of which are characterized by a triphasic color response triggered by cold exposure or stress. PRP is typically a benign disease, whereas SRP may progress into digital ulcers and/or gangrene. Here, we report a case of a 55-year-old female diagnosed with PRP 7 years ago. Treatment with first-line agents, including calcium channel blocker, aspirin, and phosphodiesterase inhibitor, did not control her symptoms, which progressed to digital ulceration and gangrene. There were no symptoms of underlying autoimmune disease or malignancy, and autoimmune, serology, and immunology test results were normal; a biopsy of her left little finger was negative for vasculitis. Development to critical digital ischemia necessitated treatment with intravenous iloprost and heparin infusion followed by angioplasty, which led to a partial improvement. Due to persistent symptoms, rituximab therapy was initiated and two cycles induced a complete resolution of symptoms.

A Case of Rituximab Use as an Induction and Maintenance of Remission in ANCA-Associated Vasculitis.

Antineutrophil cytoplasmic antibody- (ANCA-) associated vasculitis (AAV) is a multisystem autoimmune disease affecting mainly microscopic blood vessels due to circulating autoantibodies against neutrophil cytoplasmic antigens. We report a case of a 57-year-old female patient presenting with hemoptysis, sinusitis, and conjunctivitis. Based on lung biopsy, the diagnosis of antineutrophil cytoplasmic antibody- (ANCA-) associated vasculitis (AAV) was established. She was put on rituximab as induction and maintenance therapy. She responded initially to rituximab as induction therapy but failed to respond in the maintenance course of the drug. Rituximab was stopped and mycophenolate mofetil was administered. She responded as laboratory c-ANCA titers turned negative and symptoms subsided. There are no randomized clinical trials addressing rituximab effect in induction and remission at the same time. This case report doubts the efficacy of the use of rituximab therapy for both induction and maintenance of remission at the same time, waiting for the results of the ongoing trials.