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Clinical, Cosmetic and Investigational... 2024Anti-neutrophilic cytoplasmic antibody (ANCA)-associated vasculitis (AAV) overlap with systemic sclerosis (SSc) is uncommon. We aimed to determine the incidence of AAV...
PURPOSE
Anti-neutrophilic cytoplasmic antibody (ANCA)-associated vasculitis (AAV) overlap with systemic sclerosis (SSc) is uncommon. We aimed to determine the incidence of AAV and define clinical outcomes relevant to asymptomatic screening positive for ANCA in SSc after 2 years of follow-up.
PATIENTS AND METHODS
The study was a cohort study of 185 Thai adult SSc patients testing for ANCA and having a 2-year follow-up at the Scleroderma Clinic, Khon Kaen University, Thailand. The incidence of AAV and outcomes of those who tested positive for ANCA were evaluated.
RESULTS
A total of 185 SSc patients were tested for ANCA, of whom 21.6% were positive for either cytoplasmic ANCA, perinuclear ANCA (p-ANCA), anti-myeloperoxidase (anti-MPO), or anti-proteinase3 antibody. Only one 52-year-old female patient with dcSSc, negative for initial ANCA test, developed AAV (microscopic polyangiitis) 7 months after the first ANCA test for an incidence of AAV of 0.27 per 100-person-years (95% CI 0.01-1.5). She was positive for p-ANCA and anti-MPO. Eight of those who had an initial test were positive for ANCA and underwent a repeated test. Only two cases persisted as positive for ANCA (1 anti-MPO and 1 anti-PR3) and had no clinicals suspicious of vasculitis. Four cases that had ANCA turned to a negative result.
CONCLUSION
AAV is a rare complication in SSc, so ANCA may not have any role as a screening test for AAV as it cannot predict the development of AAV in SSc. We suggest testing for ANCA only in SSc patients with clinicals suspicious of AAV.
PubMed: 38314147
DOI: 10.2147/CCID.S443069 -
Journal of Personalized Medicine Jan 2024Renal involvement is a common occurrence in patients with immuno-rheumatological diseases (IRDs). Several instances of glomerulonephritis (GN) occur in the setting of...
Renal involvement is a common occurrence in patients with immuno-rheumatological diseases (IRDs). Several instances of glomerulonephritis (GN) occur in the setting of IRD and complicate the clinical course of an underlying condition. The aim of this study was to observe the spectrum of nephropathies according to age, kidney function, history of IRD at the time of biopsy, and histopathological kidney diagnosis. We evaluated data relating to 699 consecutive kidney native biopsies (female 52.1%) with a median age of 48 years (IQR 34-62) performed in adult patients collected over 15 years. The study population was divided into three groups: patients with kidney histological findings correlated to underlying IRD (Group 1), patients with kidney histological findings not correlated to underlying IRD (Group 2), and patients with kidney histological findings compatible with "de novo" IRD (absent in personal medical history) (Group 3). Kidney involvement related to IRD was found in 25.2% of patients. Group 1 was mostly represented by lupus nephritis (76.6%), with a younger age than Group 3 ( < 0.001) and by a higher percentage of females than other groups ( < 0.001). Group 3 was the most represented by microscopic polyangiitis (50.8%) when compared with the other two groups ( < 0.001). Acute nephritic syndrome ( < 0.001), acute kidney injury (AKI), and abnormal urinalysis ( < 0.001) were more represented in Group 3 than the other groups. In conclusion, IRDs are characterized by different clinical presentations and heterogeneous histological findings. Kidney biopsy remains fundamental to achieving the correct diagnosis and starting targeted therapy.
PubMed: 38248794
DOI: 10.3390/jpm14010092 -
Cureus Dec 2023Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) refers to a group of disorders characterized by inflammation and destruction of small- and...
The Successful Treatment of Acute Kidney Injury Due to Antineutrophil Cytoplasmic Antibody (ANCA)-Associated Vasculitis Without Glomeruli Involvement by Using Rituximab: A Case Report.
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) refers to a group of disorders characterized by inflammation and destruction of small- and medium-sized blood vessels. It can be classified into various clinical disease phenotypes: granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA), and renal-limited AAV or serologic subtypes, which are myeloperoxidase (MPO)-AAV and proteinase 3 (PR3)-AAV. Renal involvement is a common manifestation in these types of vasculitis. MPO-AAV usually involves the glomeruli causing membranous changes and presents with glomerulonephritis. However, MPO-AAV renal type without glomeruli involvement is much rarer, and very few case reports of this condition have been reported in the literature. Once the diagnosis is confirmed by renal biopsy, the treatment of AAV involves high-dose steroids and cyclophosphamide to induce remission. Rituximab, a chimeric monoclonal antibody that targets against the pan-B-cell marker CD20, was the first monoclonal antibody to be approved for the treatment of vasculitis. It is now considered first-line therapy for ANCA vasculitis with kidney involvement thanks to the higher remission rates associated with it. We report a unique and rare case of acute kidney injury due to MPO-AAV without glomeruli involvement, which was successfully treated with rituximab over a period of 12 months and led to the remission of the disease in the kidneys.
PubMed: 38234965
DOI: 10.7759/cureus.50732 -
Respiratory Medicine Case Reports 2024One of the most frequent diffuse interstitial lung diseases is hypersensitivity pneumonitis. It is related to exposure to diverse antigens, causing fibrosis in advanced...
One of the most frequent diffuse interstitial lung diseases is hypersensitivity pneumonitis. It is related to exposure to diverse antigens, causing fibrosis in advanced stages, making the differential diagnosis with interstitial pulmonary fibrosis difficult as it overlaps with the usual interstitial pneumonia pattern. On the other hand, there are interstitial lung diseases associated with ANCA, such as microscopic polyangiitis, which is also related to the usual interstitial pneumonia pattern. We present the case of a 74-year-old male patient with chronic dyspnea, history of smoking and exposure to organic particles, in addition to a pattern suggestive of moderately severe restriction. The diagnosis was confirmed by histology of hypersensitivity pneumonitis by presenting granules, however, anti MPO and p-ANCA positivity was found, integrating the simultaneous diagnosis of microscopic polyangiitis. This is a case of difficult diagnosis since these pathologies have not been previously reported to coexist.
PubMed: 38223467
DOI: 10.1016/j.rmcr.2023.101957 -
Journal of Immunology Research 2024Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of autoimmune diseases with inflammation affecting small blood vessels and includes...
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of autoimmune diseases with inflammation affecting small blood vessels and includes granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). In this study, we investigated granulocyte and monocyte subsets in a large cohort of AAV patients with emphasis on disease activity and tendency to relapse. A cohort of 105 patients with GPA or MPA and 126 healthy controls (HCs) were included. Clinical and laboratory data were collected for all patients, including disease activity, tendency to relapse, and pharmacological treatment. Using flow cytometry, circulating eosinophils, basophils, neutrophils, and monocytes were assessed. The monocytes were subdivided into classical (CD14CD16), intermediate (CD14CD16), and nonclassical (CD14CD16) monocytes. Mature (CD16) or newly released (CD16) neutrophils were defined, as well as the frequency of CD177 neutrophils. AAV patients displayed increased frequencies of intermediate monocytes, mature and newly released neutrophils, and an expanded population of CD177 neutrophils compared to HC. MPA patients differed from GPA patients in terms of lower frequency of classical monocytes. No differences in cell frequencies regarding ANCA phenotype were observed. Paired data from 23 patients demonstrated that active disease was associated with an increased frequency of mature neutrophils and a decreased frequency of monocytes, in particular intermediate monocytes. Moreover, GPA patients with a tendency to relapse displayed an increased frequency of mature neutrophils with increased expression of CD177. Relapsing MPA patients, on the other hand, showed decreased frequency of intermediate monocytes. Finally, rituximab treatment was associated with increased frequencies of classical and intermediate monocytes. In conclusion, AAV patients exhibit a skewing of different neutrophil and monocyte subpopulations that are associated with disease subtypes, disease activity, rituximab treatment, and propensity to relapse. These changes may contribute to the inflammatory process and could potentially be used as biomarkers for relapse prediction.
Topics: Humans; Neutrophils; Monocytes; Granulomatosis with Polyangiitis; Rituximab; Antibodies, Antineutrophil Cytoplasmic; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis; Microscopic Polyangiitis; Recurrence
PubMed: 38213873
DOI: 10.1155/2024/6648265 -
Rheumatology International Mar 2024The aim of this study is to present the current care situation of patients with giant cell arteritis (GCA), Takayasu arteritis (TAK), ANCA-associated vasculitis (AAV)...
Trends in health care of patients with vasculitides, including giant cell arteritis, Takayasu arteritis, ANCA-associated vasculitis and Behçet's disease: cross-sectional data of the German National Database 2007-2021.
The aim of this study is to present the current care situation of patients with giant cell arteritis (GCA), Takayasu arteritis (TAK), ANCA-associated vasculitis (AAV) and Behçet's disease (BD). Trends over the last 15 years will reflect improvements and remaining deficits in the management of vasculitides. Consecutive cross-sectional data from patients with vasculitides from the German National Database (NDB) of the Collaborative Arthritis Centres between 2007 and 2021 were included. Medication, physician- and patient-reported outcomes on disease activity and disease burden, inpatient stays and occupational participation are compared for different vasculitis entities and over time. Employment rates were compared to German population rates. Between 502 and 854 vasculitis patients were annually documented. GCA and AAV were the most common vasculitides. Median disease duration ranged from 2 to 16 years. Over the years, glucocorticoids decreased in proportion and dose, most markedly in GCA and TAK, while biologic therapies increased up to 27%. Physicians rated disease activity as low for the vast majority of patients, while patients-reported moderate outcomes in many dimensions. PROs remained largely unchanged. The proportion of employed patients (< 65 years) increased from 47 to 57%. In recent years, biologics are increasingly used in patients with vasculitides, while glucocorticoids decreased significantly. PRO's have not improved. Work participation increased but remains lower than that in the German population.
Topics: Humans; Giant Cell Arteritis; Behcet Syndrome; Takayasu Arteritis; Cross-Sectional Studies; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis; Glucocorticoids; Delivery of Health Care; Giant Cells
PubMed: 38180499
DOI: 10.1007/s00296-023-05508-x -
Clinical and Experimental Rheumatology Apr 2024
Biopsy-proven giant cell arteritis in an elderly woman diagnosed 11 years earlier with microscopic polyangiitis: two different vasculitis in the same patient separated in time.
Topics: Humans; Giant Cell Arteritis; Microscopic Polyangiitis; Female; Biopsy; Aged; Treatment Outcome; Temporal Arteries; Time Factors
PubMed: 38179718
DOI: 10.55563/clinexprheumatol/r847a0 -
Arthritis Research & Therapy Jan 2024Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of illnesses that cause inflammation and alterations to small vessels in the body. Some...
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.
METHODS
Patients with a current diagnosis of AAV and a control group were identified for enrollment. Nailfold videocapillaroscopy images were used for a semiquantitative analysis on capillary density, morphology, dilation, and microhemorrhage after review by 2 rheumatologists. Disease characteristics, occurrence of recent disease flare, and presence of ANCA were recorded.
RESULTS
Thirty-three patients with a diagnosis of AAV and 21 controls were recruited. The AAV group had a median age of 59 and 17 (52%) were women. Granulomatosis with polyangiitis was the most common diagnosis (19 [58%]), followed by eosinophilic granulomatosis with polyangiitis (7 [21%]) and microscopic polyangiitis (7 [21%]). Twenty-seven patients (82%) had positive ANCA tests. After assessment of capillary density, dilation, morphology, microhemorrhages, and disorganization, there were no statistically significant differences between the 2 groups.
CONCLUSION
There was no evidence of differences in nailfold capillaroscopy abnormalities between those diagnosed with AAV and the control group. While this cohort was relatively small, we did not find a high enough prevalence or specific phenotype of capillary abnormalities that could aid in diagnosis or prognostication of these diseases in the clinical setting.
Topics: Humans; Female; Male; Antibodies, Antineutrophil Cytoplasmic; Granulomatosis with Polyangiitis; Microscopic Angioscopy; Churg-Strauss Syndrome; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis; Microscopic Polyangiitis; Inflammation
PubMed: 38167073
DOI: 10.1186/s13075-023-03227-z -
Cureus Nov 2023Primary nasal tuberculosis (TB) is a rare disease even in areas with high TB burden, possibly attributed to the protective mechanism of sinonasal mucosa. Its symptoms...
Primary nasal tuberculosis (TB) is a rare disease even in areas with high TB burden, possibly attributed to the protective mechanism of sinonasal mucosa. Its symptoms are subtle and can be mistaken for other granulomatous inflammatory conditions. We would like to report a case of a 70-year-old Indian lady who underwent a successful left endoscopic dacryocystorhinostomy three years ago and presented with recurrent left epiphora. During nasal endoscopy, multiple ulcerative masses with crusting were detected over the left nasal vestibule, anterior nasal septum, left inferior, and middle turbinate. Biopsy of the nasal mass revealed granulomatous inflammation without caseating necrosis. Initially, all TB-related tests were negative. As the patient had granulomatous nasal lesions with microscopic haematuria, granulomatosis with polyangiitis (GPA) was suspected. Regrettably, the patient did not respond to treatment. A repeated tissue culture at a later stage finally detected mycobacterium tuberculosis without the presence of pulmonary tuberculosis. Considering the current TB prevalence in the Southeast Asian region, it is crucial for otorhinolaryngologists to be aware of primary nasal TB when encountering unusual head and neck lesions, even in the absence of pulmonary TB.
PubMed: 38161936
DOI: 10.7759/cureus.49649 -
Archive of Clinical Cases 2023Antineutrophil cytoplasmic autoantibody associated vasculitides has 3 different types: Granulomatosis with polyangiitis, eosinophilic granulomatosis and polyangiitis and...
Antineutrophil cytoplasmic autoantibody associated vasculitides has 3 different types: Granulomatosis with polyangiitis, eosinophilic granulomatosis and polyangiitis and microscopic polyangiitis. These vasculitides manifest differently based on which area of small and medium size vessels in our bodies that it affects. In this case report, we discuss a unique case of microscopic polyangiitis diagnosed in a 75-year-old male who was relatively asymptomatic i.e. indolent, apart from nodules present in the lung with the use of the diagnostic criteria outlined by 2022 American College of Rheumatology and European Alliance of Associations for Rheumatology. In addition, we reviewed briefly about vasculitis, its epidemiology and the workup of microscopic polyangiitis.
PubMed: 38155998
DOI: 10.22551/2023.41.1004.10276