COVID-19 Hospitalization Outcomes Among Patients With Autoimmune Rheumatic Diseases in the United States.

OBJECTIVES: To investigate the outcomes of COVID-19-related hospitalizations among patients with autoimmune rheumatic diseases (ARDs) in the United States in 2020. The primary outcome was in-hospital mortality, and secondary outcomes included intubation rate, length of hospital stay (LOS), and total hospital charges (THCs). METHODS: Data for the study were obtained from the National Inpatient Sample database and included patients who were hospitalized with a principal diagnosis of COVID-19. Univariable and multivariable logistic regression analyses were conducted to calculate odds ratios for the outcomes, adjusting for age, sex, and comorbidities. RESULTS: Out of the 1,050,720 COVID-19 admissions, 30,775 had an ARD diagnosis. The unadjusted analysis showed higher mortality (12.21%) and intubation (9.2%) rates in the ARD group compared with the non-ARD group (mortality rate: 11.14%, P = 0.013; intubation rate: 8.5%, P = 0.048). However, this difference was not significant after adjusting for confounding factors. The mean LOS and THCs did not differ significantly between the two groups. Among all ARD subgroups, the vasculitis group had significantly higher intubation rate, LOS, and THC. CONCLUSION: The study suggests that ARD is not associated with an increased risk of mortality or worse outcomes among patients hospitalized with COVID-19 after adjusting for confounding factors. However, the vasculitis group had poorer outcomes during COVID-19 hospitalizations. Further studies are needed to evaluate the effect of ARD activity and immunosuppressants on outcomes. Additionally, more research is required to investigate the relationship between COVID-19 and vasculitis.

Paraneoplastic myopathy in pancreatic cancer: a case report and literature review.

Dermatomyositis (DM) and polymyositis (PM) are both immune-mediated inflammatory myopathies known to occur in paraneoplastic syndromes associated with a new diagnosis of malignancy, most commonly breast, ovarian, lung, pancreatic, stomach, colorectal, and Non-Hodgkin's lymphoma1 in DM and breast, lung, bladder cancer, and Non-Hodgkin's lymphoma in PM. 2,3,4 While inflammatory markers such as creatine kinase (CK) may be elevated with either DM or PM, marked elevation is rare. Herein, we report a case of newly diagnosed pancreatic cancer presenting with inflammatory myopathy and marked CK elevation. We review the frequency of PM as a paraneoplastic syndrome, the association with marked CK elevation, and the association with pancreatic cancer.

Polymyalgia rheumatica: An updated review.

Polymyalgia rheumatica should be suspected in older patients with bilateral shoulder and hip stiffness that is worse in the morning and improves with use. An array of nonspecific musculoskeletal complaints, constitutional symptoms, and elevated serum inflammatory markers may be present, so other conditions should also be considered. Prolonged glucocorticoids with patient-tailored dosing and duration are the mainstay of treatment. Corticosteroid-sparing therapy with adjunctive methotrexate may benefit select patients.

Correction to: IgG4 sclerosing disease of the esophagus: a case­based review.

In the original article published, the name of the second author is incorrect.

IgG4 sclerosing disease of the esophagus: a case-based review.

IgG4-related disease (IgG4-RD) is an inflammatory and fibrosing disease which causes tumor-like swelling of organs and commonly mimics symptoms of malignancy. It has been increasing in prevalence in the last decade, but esophageal involvement remains rare. IgG4-RD was first known to involve certain organs, such as the pancreas. It has, since, been described as a systemic disease process. IgG4-RD should be considered in patients presenting with dysphagia. Initiation of appropriate treatment with corticosteroids can avoid unnecessary procedures and improve outcomes. The aim of this review is to discuss 17 cases of IgG4-RD of the esophagus. Literature review was conducted using NCBI database (PMC and PubMed filters) using the keywords "IgG4 disease," "sclerosing," "esophagus" and "gastrointestinal." The search was narrowed to include cases describing IgG4 disease of the esophagus using the same filters. Literature review identified 16 documented cases of IgG4-RD involving the esophagus. Upon literature review, it remains clear that it is extremely rare for IgG4-RD to affect the esophagus. Sixteen cases have been reported. We present a 17th case and discuss the implications of IgG4-RD. It is important to keep a broad differential diagnosis that includes IgG4-RD for patients presenting with dysphagia, especially when symptoms are refractory.

Giant cell arteritis: An updated review of an old disease.

Giant cell arteritis is a common systemic vasculitis that affects the elderly and has a variable clinical presentation. Physicians should be aware of its different clinical phenotypes so that they can recognize it early and promptly initiate glucocorticoids, the mainstay of therapy. Clinicians should also be familiar with the toxicity of glucocorticoids and how to manage adverse effects. Tocilizumab, an interleukin 6 receptor inhibitor, is emerging as a glucocorticoid-sparing treatment, though its long-term safety and efficacy are still under study.

Aortitis caused by antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis: a case-based review.

Antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) is a systemic necrotizing small vessel vasculitis primarily affecting elderly patients. Neutrophil apoptosis and release of pro-inflammatory mediators promote small vessel inflammation and hence multi-organ disease. It rarely affects larger vessels with extremely rare aortic involvement. Diagnosis is made based on clinical presentation, tissue biopsy of affected organ, as well as immunofluorescence and ELISA assays for ANCA. Management includes immunosuppression (e.g., glucocorticoids, cyclophosphamide and rituximab) and supportive therapy. We present a rare case of a younger patient with AAV involving the aorta. The patient's diagnosis was supported by clinical presentation, systemic organ involvement, strongly positive c-ANCA, and skin as well as aortic tissue biopsy results. After failing multiple immunosuppressants, he responded well to rituximab with improved symptoms, inflammatory markers, and imaging findings. Based on our literature review, we were only able to find ten cases of ANCA-related vasculitis involving the aorta. This is the first reported case of successful treatment of AAV-related aortitis using rituximab. Our case report and literature review provide insight into treatment of severe cases of AAV with aortic involvement.

When the plot thickens: a rare complication of rheumatoid arthritis.

Introduction: Rheumatoid arthritis (RA) is a common illness with many extraarticular manifestations. Rheumatoid pachymeningitis is a rare neurologic complication of this common disease. Here in we present a patient with longstanding RA who developed this uncommon complication. Case description: A 75-year-old woman with longstanding RA presented to the clinic with multiple seizure-like spells per day. Upon admission to the hospital, brain MRI showed enhancement of the meninges. After an extensive workup for possible other infectious or inflammatory causes, the patient was diagnosed with rheumatoid pachymeningitis. Conclusion: Rheumatoid pachymeningitis is a rare complication of RA that usually occurs late in the disease course. It may have many neurologic manifestations include mimicking seizure or stroke and must be considered in patients with RA presenting with neurologic symptoms. Pachymeningitis is treated distinctly from articular RA, so early recognition can lead to appropriate treatment.

Closed trauma, Mycoplasma hominis osteomyelitis, and the elusive diagnosis of Good's syndrome.

Mycoplasma hominis septic arthritis has a well-established association with hypogammaglobulinaemia, but is rarely seen in immunocompetent hosts. An association also exists with a closed trauma and a predisposition to M hominis bone infections. In this clinical case report, a patient with M hominis osteomyelitis following a closed trauma was diagnosed using 16S ribosomal studies, and led to the diagnosis of a severe underlying immunodeficiency syndrome known as Good's syndrome.

Update: biomarkers for idiopathic inflammatory myopathies.

PURPOSE OF REVIEW: Establishing diagnoses and distinguishing active disease from chronic injury remain significant clinical challenges in idiopathic inflammatory myopathies (IIM). Recent 'discovery' approaches utilizing novel genomic and proteomic techniques have revealed candidate molecular biomarkers to augment clinical and classical histological data. RECENT FINDINGS: Whole blood and serum Type 1 interferons (IFN-1) and IFN-1 inducible genes are gaining traction as disease biomarkers in IIM. IFNß is emerging as a disease activity marker specifically for dermatomyositis. Recently, molecules associated with innate immune-cell function, including TLR-3, high mobility group box (HMGB)-1, B7 Homolog 1, S100A4, and resistin have been detected in tissues of dermatomyositis patients. Serum Interleukin-17 (IL-17) and IL-23 correlate with active disease in early IIM. Antibodies recognizing the Survival Motor Neuron complex have been newly identified in a subset of patients with polymyositis. Protein aggregates are potential disease activity sensors for inclusion body myositis. Skin and lung harbor potential biomarkers for IIM. SUMMARY: Recent advances in understanding the pathogenesis of IIM have led to discovery of molecules that are candidate biomarkers of disease activity. Type 1 interferon and myeloid-cell signatures are leading candidate markers for use in IIM activity monitoring.