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Rheumatology (Oxford, England) Jul 2022To study the prevalence, risk and clinical associations of hypothyroidism among several forms of vasculitis.
OBJECTIVE
To study the prevalence, risk and clinical associations of hypothyroidism among several forms of vasculitis.
METHODS
Patients with GCA, Takayasu's arteritis (TAK), PAN and the three forms of ANCA-associated vasculitis [AAV; granulomatosis with polyangiitis (GPA), microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis (EGPA)] enrolled in a prospective, multicentre, longitudinal study were included.
RESULTS
The study included data on 2085 patients [63% female, 90% White] with a mean age of 54.6 years (s.d. 17.2). Diagnoses were GCA (20%), TAK (11%), PAN (5%), GPA (42%), microscopic polyangiitis (8%) and EGPA (14%). Hypothyroidism was present in 217 patients (10%) (83% female), with a mean age 59.8 years (s.d. 14.5). Age- and sex-adjusted risk of hypothyroidism was GCA, odds ratio (OR) 0.61 (95% CI 0.41, 0.90); TAK, OR 0.57 (95% CI 0.31, 1.03); PAN, OR 0.59 (95% CI 0.25, 1.38); GPA, OR 1.51 (95% CI 1.12, 2.05); microscopic polyangiitis, OR 1.81 (95% CI 1.18, 2.80) and EGPA, OR 0.82 (95% CI 0.52, 1.30). Among patients with AAV, age- and sex-adjusted risk of hypothyroidism was higher with positive MPO-ANCA [OR 1.89 (95% CI 1.39, 2.76)]. The clinical manifestations of vasculitis were similar in patients with and without hypothyroidism, except transient ischaemic attacks, which were more frequently observed in patients with GCA and hypothyroidism (12% vs 2%; P = 0.001).
CONCLUSIONS
Differences in the risk of hypothyroidism among vasculitides may be due to genetic susceptibilities or immune responses. This study confirms an association of hypothyroidism with MPO-ANCA.
Topics: Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis; Antibodies, Antineutrophil Cytoplasmic; Churg-Strauss Syndrome; Female; Granulomatosis with Polyangiitis; Humans; Hypothyroidism; Longitudinal Studies; Male; Microscopic Polyangiitis; Middle Aged; Prospective Studies
PubMed: 34730828
DOI: 10.1093/rheumatology/keab817 -
Current Dermatology Reports 2022Rituximab has transformed the treatment of B-cell malignancies and rheumatoid arthritis in the past 2 decades. More recently, this anti-CD20 monoclonal antibody has seen... (Review)
Review
PURPOSE OF REVIEW
Rituximab has transformed the treatment of B-cell malignancies and rheumatoid arthritis in the past 2 decades. More recently, this anti-CD20 monoclonal antibody has seen increasing usage in the field of dermatology. This review highlights the evidence supporting its use in several important dermatologic conditions.
RECENT FINDINGS
Key recent findings include the 2018 FDA approval of rituximab for the treatment of moderate-to-severe pemphigus.
SUMMARY
Data from randomized controlled trials have demonstrated the efficacy of rituximab in pemphigus, ANCA-associated vasculitis, and cryoglobulinemic vasculitis. More limited data suggests its use in recalcitrant cases of diseases such as pemphigoid, epidermolysis bullosa acquisita, and dermatomyositis. There is scarce evidence and mixed results for rituximab when studied in cutaneous polyarteritis nodosa and cutaneous lupus erythematosus.
PubMed: 36217351
DOI: 10.1007/s13671-022-00375-4 -
Diagnostics (Basel, Switzerland) Apr 2024Systemic vasculitides are a rare and complex group of diseases that can affect multiple organ systems. Clinically, presentation may be vague and non-specific and as... (Review)
Review
Systemic vasculitides are a rare and complex group of diseases that can affect multiple organ systems. Clinically, presentation may be vague and non-specific and as such, diagnosis and subsequent management are challenging. These entities are typically classified by the size of vessel involved, including large-vessel vasculitis (giant cell arteritis, Takayasu's arteritis, and clinically isolated aortitis), medium-vessel vasculitis (including polyarteritis nodosa and Kawasaki disease), and small-vessel vasculitis (granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis). There are also other systemic vasculitides that do not fit in to these categories, such as Behcet's disease, Cogan syndrome, and IgG4-related disease. Advances in medical imaging modalities have revolutionized the approach to diagnosis of these diseases. Specifically, color Doppler ultrasound, computed tomography and angiography, magnetic resonance imaging, positron emission tomography, or invasive catheterization as indicated have become fundamental in the work up of any patient with suspected systemic or localized vasculitis. This review presents the key diagnostic imaging modalities and their clinical utility in the evaluation of systemic vasculitis.
PubMed: 38667483
DOI: 10.3390/diagnostics14080838 -
Zeitschrift Fur Rheumatologie Sep 2023IgA vasculitis (IgAV) is an immune complex-mediated vasculitis characterized by IgA1-dominant immune deposits in small vessels. It is the most common systemic vasculitis...
IgA vasculitis (IgAV) is an immune complex-mediated vasculitis characterized by IgA1-dominant immune deposits in small vessels. It is the most common systemic vasculitis in childhood with a mostly uncomplicated and self-limiting course. Adults are less affected but the course is frequently more complicated and more frequently accompanied by renal involvement. IgAV characteristically manifests itself on the skin with palpable purpura and in joints, the kidneys and the gastrointestinal tract. In cases of incomplete or atypical symptoms a differential diagnostic work-up is required. A number of triggers have been suggested, especially infections and drugs. Disease management is tailored to organ manifestations and the severity of the symptoms. For children, optimized supportive care and targeted symptom relief are usually sufficient. Management of renal and gastrointestinal manifestations follows recommendations for ANCA-associated vasculitis and IgA nephropathy. Treatment options include glucocorticoids and immunosuppressive agents with varying and mostly insufficient evidence.
Topics: Adult; Child; Humans; IgA Vasculitis; Glomerulonephritis, IGA; Immunoglobulin A; Skin; Immunosuppressive Agents; Polyarteritis Nodosa; Giant Cell Arteritis; Granulomatosis with Polyangiitis
PubMed: 37266676
DOI: 10.1007/s00393-023-01355-0 -
Journal of Clinical and Experimental... 2022Skin manifestations of systemic disorders give a clue to the organ involved and help identify the possible disease-causing injury. Skin changes of liver cirrhosis are... (Review)
Review
Skin manifestations of systemic disorders give a clue to the organ involved and help identify the possible disease-causing injury. Skin changes of liver cirrhosis are not specific, as they may be seen in disorders not involving the liver. Thus, a constellation of skin changes along with systemic features may help us to identify the disease-causing liver cirrhosis. Pruritus is one of the most common and distressful symptoms of liver cirrhosis, severely affecting the quality of life, which further necessitates understanding cutaneous manifestations of cirrhosis. Other nonspecific cutaneous manifestations include spider telangiectasia, palmar erythema, paper money skin, xanthomas, pigmentation changes, nutritional deficiencies, hair changes, and nail changes. This review discusses the nonspecific skin manifestations associated with liver cirrhosis followed by specific cutaneous findings seen in common diseases causing liver cirrhosis, such as viral infections, biliary tract disorders, chronic alcoholism, and metabolic disorders. Early recognition of cutaneous features can help prevent or delay the development of complications and end-stage disease, decreasing morbidity and mortality.
PubMed: 35814509
DOI: 10.1016/j.jceh.2021.12.013 -
Frontiers in Immunology 2022
Topics: Humans; Adenosine Deaminase; Intercellular Signaling Peptides and Proteins; Vasculitis; Polyarteritis Nodosa
PubMed: 36569902
DOI: 10.3389/fimmu.2022.1108853 -
Frontiers in Immunology 2022Blood vessels are indispensable for host survival and are protected from inappropriate inflammation by immune privilege. This protection is lost in patients with... (Review)
Review
Blood vessels are indispensable for host survival and are protected from inappropriate inflammation by immune privilege. This protection is lost in patients with autoimmune vasculitides, a heterogeneous group of diseases causing damage to arteries, arterioles, and capillaries. Vasculitis leads to vascular wall destruction and/or luminal occlusion, resulting in hemorrhage and tissue ischemia. Failure in the quantity and quality of immunosuppressive regulatory T cells (Treg) has been implicated in the breakdown of the vascular immune privilege. Emerging data suggest that Treg deficiencies are disease-specific, affecting distinct pathways in distinct vasculitides. Mechanistic studies have identified faulty CD8 Tregs in Giant Cell Arteritis (GCA), a vasculitis of the aorta and the large aortic branch vessels. Specifically, aberrant signaling through the NOTCH4 receptor expressed on CD8 Treg cells leads to rerouting of intracellular vesicle trafficking and failure in the release of immunosuppressive exosomes, ultimately boosting inflammatory attack to medium and large arteries. In Kawasaki's disease, a medium vessel vasculitis targeting the coronary arteries, aberrant expression of miR-155 and dysregulated STAT5 signaling have been implicated in undermining CD4 Treg function. Explorations of mechanisms leading to insufficient immunosuppression and uncontrolled vascular inflammation hold the promise to discover novel therapeutic interventions that could potentially restore the immune privilege of blood vessels and pave the way for urgently needed innovations in vasculitis management.
Topics: Arteries; Giant Cell Arteritis; Granulomatosis with Polyangiitis; Humans; Inflammation; Mucocutaneous Lymph Node Syndrome; Polyarteritis Nodosa; T-Lymphocytes, Regulatory
PubMed: 35296082
DOI: 10.3389/fimmu.2022.844300 -
Clinical Rheumatology Oct 2023Cardiovascular involvement in juvenile rheumatic diseases is the primary manifestation in paediatric vasculitis and a major organ manifestation in paediatric connective... (Review)
Review
Cardiovascular involvement in juvenile rheumatic diseases is the primary manifestation in paediatric vasculitis and a major organ manifestation in paediatric connective tissue diseases. Though coronary vasculitis is the prototypical manifestation of Kawasaki disease, it can also be seen in patients with polyarteritis nodosa. Pericarditis is the most common manifestation seen in juvenile rheumatic diseases like systemic onset JIA, and lupus. Cardiac tamponade, valvular insufficiency, aortic root dilatation and arrhythmias are seen rarely. Cardiac involvement is often recognized late in children. The development of cardiac disease in juvenile systemic sclerosis is associated with a poor outcome. In long term, childhood onset of rheumatic diseases predisposes to diastolic dysfunction and premature atherosclerosis during adulthood. Key Points • Pericarditis is the most common cardiac manifestation in SLE and can lead to tamponade. • Conduction defects are common in juvenile mixed connective tissue disease and systemic sclerosis. • Pulmonary hypertension is a significant contributor to mortality in juvenile systemic sclerosis. • In Kawasaki disease, early treatment can reduce risk of coronary artery aneurysms.
Topics: Child; Humans; Adult; Mucocutaneous Lymph Node Syndrome; Heart Diseases; Pericarditis; Rheumatic Diseases; Scleroderma, Systemic; Vasculitis; Lupus Erythematosus, Systemic
PubMed: 37160484
DOI: 10.1007/s10067-023-06621-9