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Frontiers in Immunology 2024Severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) may trigger autoimmune disease (AD) through initial innate immune activation with subsequent aberrations in...
INTRODUCTION
Severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) may trigger autoimmune disease (AD) through initial innate immune activation with subsequent aberrations in adaptive immune cells leading to AD. While there are multiple reports of incident AD diagnosed after COVID-19, the risk in the context of key circulating strains is unknown.
METHODS
TriNetX, a global, federated, health research network providing access to electronic medical records across 74 healthcare organizations, was utilized to define an adult cohort between January 1, 2020, and March 3, 2023. Exposure was defined as COVID-19 diagnosis (ICD-10 code or positive laboratory test). Age- and sex-propensity score-matched controls never had COVID-19 diagnosed. Outcomes were assessed 1 month to 1 year after the index date. Patients with AD prior to or within 1 month after the index date were excluded from the primary analysis. Incidence and risk ratios of each AD were assessed.
RESULTS
A total of 3,908,592 patients were included. Of 24 AD patients assessed, adjusted risk ratios for eight AD patients who had COVID-19 were higher compared to those who had no COVID-19. Cutaneous vasculitis (adjusted hazard ratio (aHR): 1.82; 95% CI 1.55-2.13), polyarteritis nodosa (aHR: 1.76; 95% CI 1.15-2.70), and hypersensitivity angiitis (aHR: 1.64; 95% CI 1.12-2.38) had the highest risk ratios. Overall, psoriasis (0.15%), rheumatoid arthritis (0.14%), and type 1 diabetes (0.13%) had the highest incidence during the study period, and of these, psoriasis and diabetes were more likely after COVID-19. The risk of any AD was lower if COVID-19 was diagnosed when Omicron variants were the predominant circulating strains. A positive antinuclear antibody was more likely and predictive of AD after COVID-19.
DISCUSSION
SARS-CoV-2 may be a potential trigger for some AD, but the risk for AD may decrease with time given the apparent lower risk after infection with Omicron variants.
Topics: Adult; Humans; COVID-19; SARS-CoV-2; COVID-19 Testing; Autoimmune Diseases; Psoriasis
PubMed: 38390319
DOI: 10.3389/fimmu.2024.1337406 -
Vascular Medicine (London, England) Jun 2024
Topics: Humans; Polyarteritis Nodosa; Female; Hepatic Artery; Aneurysm; Adult; Treatment Outcome; Computed Tomography Angiography
PubMed: 38351513
DOI: 10.1177/1358863X231213362 -
Arthritis & Rheumatology (Hoboken, N.J.) Jul 2024We describe the demographics, clinical features, disease course, and survival of polyarteritis nodosa (PAN) through an international collaboration (GLOBAL-PAN). (Observational Study)
Observational Study
OBJECTIVE
We describe the demographics, clinical features, disease course, and survival of polyarteritis nodosa (PAN) through an international collaboration (GLOBAL-PAN).
METHODS
Patients with PAN were recruited between 1990 and 2020 from observational cohorts of nine countries across Europe, Japan, and North America. Eligibility was retrospectively defined using the European Medicines Agency classification algorithm. Patients with PAN related to hepatitis B virus (n = 12) and two monogenic diseases mimicking PAN, deficiency of adenosine deaminase 2 enzyme (n = 16) or familial Mediterranean fever (n = 11), were excluded. Data regarding organ involvement, relapse, disease-related damage, and survival were analyzed.
RESULTS
Three hundred fifty-eight patients (female:male ratio 174:184), including those with systemic PAN (sPAN, n = 282) and cutaneous PAN (n = 76), were included. Twenty-five were pediatric onset. Mean ± SD age at diagnosis was 44.3 ± 18.1 years. Constitutional symptoms (71.5%), cutaneous involvement (70.5%), musculoskeletal findings (69.1%), and neurologic features (48.0%) were common manifestations. Among patients with sPAN, gastrointestinal involvement and proteinuria over 400 mg/day were reported in 52.2% and 11.2%, respectively. During a median (interquartile range) 59.6 (99.5) months of follow-up, relapse occurred in 48.5% of patients. One, 5- and 10-year survival rates for sPAN were 97.1%, 94.0%, and 89.0%, respectively. Predictors of death for sPAN included age ≥65 years at diagnosis, serum creatinine at diagnosis >140 μmol/L, gastrointestinal manifestations, and central nervous system (CNS) involvement.
CONCLUSION
The spectrum of PAN remains a complex, multifaceted disease. Relapse is common. Age ≥65 years and serum creatinine >140 μmol/L at diagnosis, as well as gastrointestinal and CNS involvement, are independent predictors of death in sPAN.
Topics: Humans; Polyarteritis Nodosa; Male; Female; Adult; Middle Aged; Retrospective Studies; Europe; Aged; North America; Japan; Young Adult; Proteinuria; Recurrence; Survival Rate
PubMed: 38343337
DOI: 10.1002/art.42817 -
Modern Rheumatology Feb 2024Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis that predominantly affects medium-sized arteries. With advances in our understanding of the pathogenesis...
Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis that predominantly affects medium-sized arteries. With advances in our understanding of the pathogenesis and classification of vasculitis, PAN and microscopic polyangiitis (MPA), a disease of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV), were separated from the group of diseases previously diagnosed as periarteritis nodosa (PN) at the Chapel Hill Consensus Conference (CHCC) in 1994 (1).
PubMed: 38343272
DOI: 10.1093/mr/roae010 -
Scandinavian Journal of Rheumatology Feb 2024Inflammation markers, e.g. C- reactive protein (CRP) and sedimentation rate, can be normal despite active vasculitis. Von Willebrand factor (vWF) is secreted from...
OBJECTIVE
Inflammation markers, e.g. C- reactive protein (CRP) and sedimentation rate, can be normal despite active vasculitis. Von Willebrand factor (vWF) is secreted from endothelial cells in response to vascular damage. Some reports suggest increased vWF levels in vasculitis. This study aimed to evaluate vWF serum concentration in vasculitis patients as a possible biomarker of disease activity and to review the current literature.
METHOD
Adult patients with systemic vasculitis were prospectively enrolled. Disease activity was recorded using the Birmingham Vasculitis Activity Score (BVAS) version 3. Blood group-adjusted vWF antigen serum level was evaluated at diagnosis and, when available, after treatment.
RESULTS
Twenty-five vasculitis patients were compared to 15 healthy controls. The mean age of patients was 56 ± 17 years and 56% were women. Forty percent had anti-neutrophil cytoplasmic autoantibody-associated vasculitis, 20% giant cell arteritis, 16% polyarteritis nodosa, 8% Takayasu arteritis, and the rest had other vasculitides. The mean disease duration was 3.4 ± 4.8 years. Mean vWF was higher in patients with active vasculitis than in healthy controls (212 ± 81% vs 106 ± 26%, p < 0.001). vWF levels directly correlated with BVAS. In 13 patients with active vasculitis who reached remission or low disease activity after treatment, vWF level at follow-up decreased significantly. In three out of five patients who were treated with interleukin-6 inhibitors, vWF was elevated despite normal CRP levels, while vasculitis was clinically active.
CONCLUSION
vWF antigen serum level is increased in active vasculitis and could potentially serve as a biomarker for active disease.
PubMed: 38314784
DOI: 10.1080/03009742.2024.2302679 -
International Journal of Surgical... Feb 2024Primary angiitis of central nervous system (PACNS) is a rare idiopathic vasculitis that typically involves small arteries. An 18-year-old woman was operated on for...
Primary angiitis of central nervous system (PACNS) is a rare idiopathic vasculitis that typically involves small arteries. An 18-year-old woman was operated on for resection of a ruptured aneurysm in a cerebral artery. Multiple aneurysms of cerebral arteries had been detected by neuroimaging examinations since the age of 12, and she had been administered drugs following a diagnosis of PACNS since the age of 15. The resected aneurysm was a ruptured saccular aneurysm occurring in a medium-sized artery. Histologically, necrotizing arteritis of the polyarteritis nodosa (PAN) type was noted in the aneurysmal wall. It consisted of an admixture of acute and healing stages. In the acute stage, fibrinoid necrosis in the intima and media and intense inflammatory cell infiltrate in the entire wall were present. The inflammatory cells mainly consisted of lymphocytes, including plasma cells, neutrophils, and macrophages. In the healing stage, disappearance of fibrinoid necrosis, fibrosis in the intima and media, and scarce inflammatory cells were noted. The acute stage was mainly present near the ruptured site. From these findings, the aneurysm was considered to have been caused by necrotizing arteritis of the PAN type. Although saccular aneurysmal formation in a medium-sized artery is rare in PACNS, an understanding of aneurysms produced by necrotizing arteritis of the PAN type offers useful information for the diagnosis and treatment of PACNS.
PubMed: 38303147
DOI: 10.1177/10668969241228292 -
Innere Medizin (Heidelberg, Germany) Feb 2024The immune-mediated small vessel vasculitis is known as Schoenlein-Henoch purpura predominantly from pediatrics and in these cases occurs more frequently after... (Review)
Review
The immune-mediated small vessel vasculitis is known as Schoenlein-Henoch purpura predominantly from pediatrics and in these cases occurs more frequently after infections of the upper airways. In adults, immunoglobulin A (IgA) vasculitis often proceeds more severely und recurrently with the classical tetrad of skin manifestations in the sense of leukocytoclastic vasculitis, joint affection, gastrointestinal involvement and IgA nephritis, in contrast to the mostly mild and self-limiting course in children. The background of this systemic vasculitis with formation of IgA immune complexes is considered to be an altered glycosylation of IgA, as this causes the exposure of binding sites for autoantibodies so that an immune complex reaction can be elicited. This ultimately leads to perivascular deposition of IgA and a further activation of neutrophils. Groundbreaking in the diagnostics is the histological detection of leukocytoclastic vasculitis and in cases of renal manifestations a kidney biopsy with characteristic deposits of immune complexes, which cannot be clearly differentiated from IgA nephropathy. The treatment is aimed at the respective manifestation and is mostly based on consensus recommendations due to the lack of randomized studies. In addition to immunosuppressive medication, in the presence of a chronic kidney disease general nephroprotection is becoming increasingly more important also by inhibition of sodium-glucose transporter 2 (SGLT2). The type and extent of kidney involvement and also rare cardiac manifestations are the main determinants of the prognosis. Continuous medical accompaniment of those affected is necessary due to the possible progression of the disease and the risk of recurrence.
Topics: Adult; Humans; Child; IgA Vasculitis; Antigen-Antibody Complex; Immunoglobulin A; Vasculitis; Polyarteritis Nodosa; Vasculitis, Leukocytoclastic, Cutaneous
PubMed: 38236411
DOI: 10.1007/s00108-023-01650-7 -
Endocrinology, Diabetes & Metabolism... Jan 2024Diabetes foot ulcer (DFU) is a common long-term complication of diabetes. Intractable chronic wounds to standard care of diabetic foot raise the question of whether...
SUMMARY
Diabetes foot ulcer (DFU) is a common long-term complication of diabetes. Intractable chronic wounds to standard care of diabetic foot raise the question of whether other factors intervene in disease development. We report a case of a 54-year-old male patient who came to Sardjito General Hospital with leg pain and previous history of multiple debridement and amputation for DFU referred from a remote hospital yet no improvement was evident in the surrounding lesion following treatment. Consequently, a histopathological examination was carried out proving the presence of other aetiologic factors, vasculitis and panniculitis existing in the lesion. In this case, we report a rare type of causative factor of foot ulcers among diabetic patients. Vasculitis suspected for polyarteritis nodosa accompanied by panniculitis is considered in this patient. The treatment of choice is corticosteroids or immunosuppressants based on the clinical condition, contrary to usual wound care in DFU. Based on the evidence, clinicians need to consider other causes than only macrovascular complications in a diabetic patient with DFU that is intractable to standard wound care. In this patient, vasculitis may be considered in forming diabetic foot ulcers alongside macrovascular complications.
LEARNING POINTS
A thorough examination is essential to rule out other processes in intractable DFU patients. Prompt management based on proper diagnosis is crucial to prevent peripheral arterial disease complications. Vasculitis and macrovascular complication are inseparable processes forming DFU in this patient.
PubMed: 38189794
DOI: 10.1530/EDM-22-0397 -
BMC Cardiovascular Disorders Jan 2024Spontaneous coronary artery dissection is a rare and important cause of myocardial infarction, especially in young women without other coronary artery disease. This...
BACKGROUND
Spontaneous coronary artery dissection is a rare and important cause of myocardial infarction, especially in young women without other coronary artery disease. This arterial dissection can occur within or between any of the 3 layers. Its predisposing factors include connective tissue diseases (Marfone syndrome, Ehlers-Danlos syndrome), vasculitis (polyarteritis nodosa, systemic lupus erythematosus, and Kawasaki disease), atherosclerosis and fibromuscular dysplasia. Clinical presentations of spontaneous coronary artery dissection are wide spectrum from asymptomatic to acute coronary disease, sustained ventricular arrhythmia and sudden cardiac death.
CASE PRESENTATION
We describe A 33-year-old man with history of Hodgkin's lymphoma five years earlier that became a candidate for Patent foramen ovale closure due to recurrent embolic cerebrovascular accident. Before the intervention, coronary angiography incidentally showed dissection in the left main and all major coronary arteries.
CONCLUSIONS
Based on our hypothesis, chemoradiotherapy-induced arteriopathies could be consider as a predisposing factor for spontaneous coronary artery dissection.
Topics: Male; Humans; Female; Adult; Coronary Vessels; Hodgkin Disease; Incidental Findings; Myocardial Infarction
PubMed: 38172682
DOI: 10.1186/s12872-023-03609-w -
Innere Medizin (Heidelberg, Germany) Feb 2024Polyarteritis nodosa (PAN) and Kawasaki syndrome (KS) are rare forms of primary vasculitis with heterogeneous manifestations and courses of the disease. According to the... (Review)
Review
Polyarteritis nodosa (PAN) and Kawasaki syndrome (KS) are rare forms of primary vasculitis with heterogeneous manifestations and courses of the disease. According to the Chapel Hill Consensus Conference 2012 they belong to the vasculitis of medium size vessels. In contrast to microscopic polyangiitis (MPA), PAN and KS do not affect microscopic vessels such as arterioles, venules or capillaries and are not associated with antineutrophil cytoplasmic antibodies (ANCA). The diagnostics are based on the typical constellation of clinical symptoms, on angiographic findings, the exclusion of other differential diagnoses and, in the case of PAN, in the histopathological confirmation. The therapeutic options of KS in childhood and PAN in adults and children, which are dependent on the severity and the prognosis, are presented.
Topics: Adult; Child; Humans; Polyarteritis Nodosa; Mucocutaneous Lymph Node Syndrome; Antibodies, Antineutrophil Cytoplasmic; Microscopic Polyangiitis; Prognosis
PubMed: 38168826
DOI: 10.1007/s00108-023-01637-4