Pulmonary involvement is commonly observed in anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), presenting with manifestations such as diffuse alveolar hemorrhage, inflammatory infiltrates, pulmonary nodules, and tracheobronchial disease. We aimed to identify distinct subgroups of tracheobronchial disease patterns in patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) using latent class analysis (LCA), and to evaluate their clinical characteristics and outcomes. We conducted a retrospective cohort study using electronic medical records of patients aged >18 years diagnosed with AAV and tracheobronchial disease between 1 January 2002 and 6 September 2022.
View Article and Find Full Text PDFThe term Raynaud's phenomenon (RP) is used to describe complex symptoms related to vascular compromise, which are typically exacerbated by cold-induced vasoconstriction, emotional stress, or other sympathomimetic factors. In almost all patients with limited cutaneous systemic sclerosis (SSc), the first symptom is RP, often two to five years before any other symptom of scleroderma. The clinical course and severity of this disease are variable and highly fatal in some individuals, which has led to the development of strategies for timely diagnosis; hence, criteria for the very early diagnosis of systemic sclerosis have been established.
View Article and Find Full Text PDFTo investigate the effect of mycophenolate mofetil (MMF) and rituximab (RTX) on pulmonary function test (PFT) results in a mixed cohort of patients with connective tissue disease-associated interstitial lung disease (CTD-ILD), longitudinally followed up for 1 year in a single academic center. Patients with CTD-ILD were identified in electronic medical records from 1 January 2009 to 30 April 2019. Prescribed MMF and RTX doses, dosage changes, and therapy plans were analyzed individually with improvement in PFT outcomes determined using multivariable linear regression models during 12-month follow-up.
View Article and Find Full Text PDFObjective: Avacopan, an activated complement factor 5 receptor antagonist, has been approved as adjunct therapy for severe active antineutrophil cytoplasmic antibody-associated vasculitis (AAV). Current evidence supports the management of AAV presenting with diffuse alveolar hemorrhage (DAH) by administering glucocorticoids combined with either rituximab or cyclophosphamide in addition to supportive care. The role of avacopan in patients with DAH as a primary severe disease manifestation of AAV has not been well established.
View Article and Find Full Text PDFTakayasu arteritis is a chronic inflammatory vasculitis with granulomatous panarteritis particularly impacting large vessels including the aorta and its branches, especially the subclavian arteries, with clinical manifestation dependent on the involved artery. Sequelae of the active disease vary, including stenosis, occlusions, or aneurysmal dilatations of the large vessels. The prevalence of Takayasu arteritis is higher in the Asian population and in Japan, but quite low in the United States, varying from 0.
View Article and Find Full Text PDFObjective: To assess relationship between Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis and inflammatory bowel disease (IBD).
Methods: This is a retrospective study design. The patients were identified using a preset criteria of patients who have the diagnosis of ANCA associated vasculitis including a diagnosis of granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) or eosinophilic granulomatosis with polyangiitis (EGPA) with overlapping inflammatory bowel disease (Crohn's disease or ulcerative colitis) in the time period from 01/01/2020 to 08/03/2023.
Neurosarcoidosis is one of the most relevant involvements in systemic sarcoidosis and can be the initial presentation. Its diagnosis is often considered difficult because of unusual clinical manifestations or diagnostic mimics. The peripheral nervous system is less frequently involved than the central nervous system, although it may also lead to irreversible neurologic impairment.
View Article and Find Full Text PDFBackground: This retrospective study was designed to analyze the prevalence and impact of associated comorbidities on fibromyalgia (FM) outcomes (functionality, pain, depression levels) for patients who participated in an intensive multicomponent clinical program in a tertiary care center.
Methods: Participants included a sample of 411 patients diagnosed with FM at a large tertiary medical center using the 2016 ACR criteria. Patients completed an intensive 2-day cognitive behavioral treatment (CBT) program, filled out the Fibromyalgia Impact Questionnaire Revised (FIQR), the Center for Epidemiologic Studies Depression Scale (CES-D), the Pain Catastrophizing Scale (PCS), and were followed for 6 months after treatment completion.
Objective: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of illnesses that cause inflammation and alterations to small vessels in the body. Some of the most common and detrimental manifestations, including alveolar hemorrhage and glomerulonephritis, are caused by this capillary inflammation. We sought to clarify whether patients with AAV would have abnormal nailfold capillaries when evaluated with nailfold videocapillaroscopy.
View Article and Find Full Text PDFBackground/objective: Few studies have investigated associations between rheumatologic serology patterns and different interstitial lung disease (ILD) patterns.
Methods: We present novel findings of a historic cohort study (n = 454) with data collected from 2011 to 2021 within our hospital system. In this institutional review board-approved study, data regarding rheumatologic serologies and ILD patterns were noted based on chart review in patients with scleroderma.
Arthritis Care Res (Hoboken)
January 2024
Objective: New-onset and relapsed dermatomyositis (DM) has been reported following SARS-CoV-2 infection or COVID-19 vaccination. This study aims to show the characteristics of a DM cohort after COVID-19 infection and vaccination.
Methods: A retrospective review was performed on patients treated for DM between March 1, 2020, and October 31, 2022.
Radiologists fulfill a vital role in the multidisciplinary care provided to patients with interstitial lung diseases and other diffuse parenchymal lung disorders. The diagnosis of interstitial lung diseases hinges on the consensus of clinical, radiology, and pathology medical subspecialists, but additional expertise from rheumatology, immunology, or hematology can be invaluable. The thin-section computed tomography (CT) features of lung involvement informs the diagnostic approach.
View Article and Find Full Text PDFObjective: To evaluate the safety and efficacy of tocilizumab (TCZ) in giant cell arteritis (GCA) in a large North American cohort.
Methods: Patients with GCA treated with TCZ between January 1, 2010, and May 15, 2020, were retrospectively identified. Kaplan-Meier methods were used to estimate time to TCZ discontinuation and time to first relapse after TCZ discontinuation.
Diffuse alveolar hemorrhage (DAH) is a pulmonary condition that can be caused by autoimmune disorders such as lupus, small vessel vasculitis, and antiphospholipid syndrome. Sarcoidosis as a cause of DAH has been reported; however, the literature remains limited. We performed a chart review for patients with a diagnosis of both sarcoidosis and DAH.
View Article and Find Full Text PDFObjectives: To develop treat-to-target (T2T) recommendations in giant cell arteritis (GCA) and polymyalgia rheumatica (PMR).
Methods: A systematic literature review was conducted to retrieve data on treatment targets and outcomes in GCA/PMR as well as to identify the evidence for the effectiveness of a T2T-based management approach in these diseases. Based on evidence and expert opinion, the task force (29 participants from 10 countries consisting of physicians, a healthcare professional and a patient) developed recommendations, with consensus obtained through voting.
Numerous inflammatory, neoplastic, and genetic skin disorders are associated with interstitial lung disease (ILD), the fibrosing inflammation of lung parenchyma that has significant morbidity and mortality. Therefore, the dermatologist plays a major role in the early detection and appropriate referral of patients at risk for ILD. Part 1 of this 2-part CME outlines the pathophysiology of ILD and focuses on clinical screening and therapeutic principles applicable to dermatological patients who are at risk for ILD.
View Article and Find Full Text PDFPart 2 of this 2-part CME introduces dermatologists to noninfectious inflammatory skin diseases associated with pulmonary involvement. In many cases, dermatologists may be the first physicians recognizing respiratory complications associated with these diagnoses. Because pulmonary involvement is often the leading cause of morbidity and mortality, dermatologists should be comfortable screening and monitoring for lung disease in high-risk patients, recognizing cutaneous stigmata of lung disease in these patients and referring to pulmonary specialists, when appropriate, for prompt treatment initiation.
View Article and Find Full Text PDFJ Prim Care Community Health
August 2022
Background: About 4 out of 10 fibromyalgia patients suffer from depression. The European Alliance of Associations for Rheumatology (EULAR) guidelines recommend using antidepressants to treat fibromyalgia.
Objective: To determine predictors of improved outcomes following a multicomponent treatment program.
Introduction: Colchicine, because of its anti-inflammatory and possible anti-viral properties, has been proposed as potential therapeutic option for COVID-19. The role of colchicine to mitigate "cytokine storm" and to decrease the severity and mortality associated with COVID-19 has been evaluated in many studies.
Objective: To evaluate the role of colchicine on morbidity and mortality in COVID-19 patients.
Objective: To provide evidence-based recommendations and expert guidance for the management of Kawasaki disease (KD), focusing on clinical scenarios more commonly addressed by rheumatologists.
Methods: Sixteen clinical questions regarding diagnostic testing, treatment, and management of KD were developed in the Patient/Population, Intervention, Comparison, and Outcomes (PICO) question format. Systematic literature reviews were conducted for each PICO question.
Objective: To provide evidence-based recommendations and expert guidance for the management of Kawasaki disease (KD), focusing on clinical scenarios more commonly addressed by rheumatologists.
Methods: Sixteen clinical questions regarding diagnostic testing, treatment, and management of KD were developed in the Patient/Population, Intervention, Comparison, and Outcomes (PICO) question format. Systematic literature reviews were conducted for each PICO question.
Ther Adv Musculoskelet Dis
February 2022
The major pulmonary complication of rheumatoid arthritis (RA) is interstitial lung disease (ILD), which causes significant morbidity and mortality and influences the natural course of disease. Recent advances in the management of arthritis have improved patient outcomes. However, exceptionally high medical needs still remain for effective therapies for the patients with ILD in RA.
View Article and Find Full Text PDFObjective: Kawasaki disease (KD) is a self-limited vasculitis affecting medium-sized vessels with a predilection for the coronary arteries. Although treatment reduces the likelihood of developing of coronary artery aneurysms, 5% of patients still develop aneurysms despite treatment, making KD the leading cause of acquired heart disease in children in the United States. Consequently, there is a great deal of interest in optimizing treatment regimens, particularly for higher-risk patients, to decrease morbidity.
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