Perinuclear Antineutrophil Cytoplasmic Antibodies Research Articles

Isolated Gastric Crohn's Disease: A Growing Tendency.

This case highlights the challenges and complexities of diagnosing and managing isolated gastric Crohn's disease (IGCD), a rare manifestation of Crohn's disease confined to the stomach.Serological tests such as the anti-Saccharomyces cerevisiae antibody (ASCA) test and the perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA) test help in distinguishing Crohn's disease from other conditions.This case emphasises the importance of considering IGCD in patients with unexplained gastric symptoms, and the need for individualised treatment plans due to the lack of specific guidelines for IGCD.

Prognostic factors and outcomes in anti-neutrophil cytoplasmic antibody-associated glomerulonephritis: A retrospective single-center study from southern China.

To investigate the prognostic factors and outcomes in patients with anti-neutrophil cytoplasmic antibody-associated glomerulonephritis (ANCA-GN) in Southern China. A retrospective analysis of medical records of patients with ANCA-GN admitted to Shenzhen Hospital of Southern Medical University and Nanfang Hospital of Southern Medical University between September 2011 and September 2021 was performed. The clinical presentation, biological, and renal pathology were collected. In addition, the risk factors for end-stage renal disease (ESRD) and short-term overall survival in patients with ANCA-GN were analyzed. A total of 93 patients with ANCA-GN were included in the study. Of them, 91.4%, were perinuclear anti-neutrophil cytoplasmic antibodies (MPO-pANCA)-positive. Approximately one-quarter (24.7%) of patients had progressed to ESRD, and 7.5% died within six months. Most patients presented with hematuria (94.6%), proteinuria (78.5%), elevated serum creatinine (86.0%), anemia (90.3%), and increased erythrocyte sedimentation rate (ESR) (44.1%). The majority (94.6%) of patients presented with crescent formations at histopathological examination. Serum creatinine, hemoglobin, and Birmingham vasculitis activity score (BVAS) were all independent factors for ESRD (P<0.05). Moreover, while ANCA renal risk score (ARRS) has an impact on prognosis of nephropathy, it did not influence ESRD independently (P>0.05). The effect of Berden's histopathologic classification on ESRD has not been confirmed. Age at onset, ESR and cardiovascular involvement were all independent factors affecting short-term overall survival of patients with ANCA-GN (P<0.05). Serum creatinine, hemoglobin, and BVAS were all independent risk factors of ESRD, while ARRS and Berden's histopathologic classification were not. Age at onset, ESR, and cardiovascular involvement were independent risk factors for the overall six-month survival rate in patients with ANCA-GN.

Unmasking of Churg–Strauss Syndrome in an Asthmatic

Abstract Churg–Strauss syndrome, also known as eosinophilic granulomatosis, is one of the antineutrophilic cytoplasmic antibody (ANCA)-associated vasculitis associated with asthma and peripheral eosinophilia. Here, we present a case of a 50-year-old male, known asthmatic presenting with cough, dyspnea, fever, and lower limb purpura. On evaluation found to have perinuclear ANCA positivity; a nerve conduction study showed mononeuritis multiplex, and he was diagnosed with Churg–Straus syndrome.

A Systematic Review of Perinuclear Antineutrophil Cytoplasmic Antibody-Associated Glomerulonephritis Following Coronavirus Disease 2019 Vaccination: A 2024 Update.

Antineutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis (GN) is an immune-mediated kidney disease characterized by the inflammation of small blood vessels in the kidney, leading to renal impairment and potentially irreversible damage. Concerns have been raised over the reports of myeloperoxidase/perinuclear (MPO/p) ANCA GN following the coronavirus disease 2019 (COVID-19) vaccination. Our study provides a comprehensive insight into perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA) GN after COVID-19 vaccination. We conducted a comprehensive literature search on PubMed, Cochrane Library, and EMBASE using the Medical Subject Headings (MeSH) terms related to "covid-19 vaccine," "glomerulonephritis," "p-ANCA," and "MPO-ANCA" up to March 5, 2024, to include cases of p-ANCA-associated GN following COVID-19 vaccination. Of the 4,102 articles, we included 29, reporting 35 patients demonstrating COVID-19 vaccine-induced p-ANCA GN, with 23 (65.7%) females and a median age of 69 years (mean ± SD = 63.22 ± 16). Twenty-six (74.28%) patients received the mRNA vaccine (Pfizer = 19, Moderna = 7). Seventeen (48.57%) patients presented with p-ANCA GN after the second dose of the COVID-19 vaccine, with a median gap of 19 days (1-84 days). Constitutional symptoms (54.28%) and acute kidney injury (42.85%) were the most reported initial presentations, and elevated serum creatinine (mean peak serum creatinine = 4.98 ± 5.02 mg/dL), hematuria, and proteinuria were the laboratory findings. MPO/p-ANCA was positive in 31 (88.6%) patients. All patients underwent renal biopsy, and crescentic GN was the most common finding among 27 (77.14%) patients. Management of p-ANCA GN included steroids in 30 (85.71%) patients, followed by rituximab (28.57%), and plasmapheresis (22.86%). Most patients responded well to treatment, with complete remission in 29 (82.86%) and relapse in four (11.42%) patients. Two patients did not achieve remission and became dialysis dependent. ANCA-associated GN is a rare and life-threatening complication of the COVID-19 vaccine, necessitating urgent evaluation and management. COVID-19 vaccine-induced p-ANCA GN should be included in the differential diagnoses of patients presenting with kidney injury after vaccination.

Inflammatory Bowel Disease and Its Association With Perinuclear Antineutrophil Cytoplasmic Antibodies: A Systematic Review.

An idiopathic condition known as inflammatory bowel disease (IBD) is characterized by a dysregulated immune response to the intestinal flora of the host. It falls into one of two primary categories: ulcerative colitis or Crohn's disease. A wide range of disorders, both clinically and genetically, can cause IBD. The purpose of this thorough analysis is to determine the significance and reliability of the correlation between perinuclear antineutrophil cytoplasmic antibodies (p-ANCA) and IBD, as well as the implications of this correlation for the diagnosis and treatment of IBD. Ten pertinent studies were identified from a starting pool of 20 articles in this systematic review, which was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) 2020 guidelines. These studies addressed treatment, complications, limitations, and outcome in addition to the presence or lack of p-ANCA in patients with IBD. In conclusion, p-ANCA is more strongly linked to inflammatory bowel illness than Crohn's disease, primarily ulcerative colitis. Some evidence suggests that there is a decrease in p-ANCA to some extent with medical or surgical interventions, but the exact intervention is not yet clear. There is less evidence suggesting that the medical or surgical treatments used in patients with IBD cause an increase or decrease in p-ANCA.

Antineutrophil cytoplasmic antibody in children with nephrotic syndrome treated with levamisole: a cross-sectional cohort study.

Levamisole is a commonly used steroid-sparing agent (SSA), but the reported incidence of antineutrophil cytoplasmic antibody (ANCA) positivity has been concerning. Observational cross-sectional study wherein children aged 2 to 18years with frequently relapsing/steroiddependent nephrotic syndrome (FRNS/SDNS) on levamisole for ≥ 12months were tested for ANCA. A total of 210 children (33% female), median age of 7.3 (IQR: 5.6-9.6) years, and a median duration of levamisole exposure of 21 (IQR: 15-30) months were tested. ANCA was positive in 18% (n = 37): 89% (n = 33) perinuclear ANCA (pANCA), 3% (n = 1) cytoplasmic ANCA (cANCA), and 8% (n = 3) both. Of ANCA-positive children, none had reduced eGFR or abnormal urinalysis. The majority of these children were asymptomatic (81%, n = 30). Rash was more common among ANCA-positive children [6/37 (16%) vs. 3/173 (2%), p = 0.0001]. On multivariate analysis, higher age (OR = 1.02, [95th CI: 1.01 to 1.03], p = 0.007) and longer duration of levamisole exposure (OR = 1.05, [95th CI: 1.02 to 1.08], p = 0.0007) were associated with ANCA positivity. Levamisole was stopped in ANCA-positive children with the resolution of any clinical manifestations if present. Repeat ANCA testing was performed in 54% (20/37), and all were ANCA negative by 18months. Children with FRNS/SDNS on longer duration of levamisole were associated with increasing prevalence of ANCA positivity, but most of these children were clinically asymptomatic. Prospective studies are required to determine the chronology of ANCA positivity and its clinical implication.

Hepatitis C virus-related autoimmunity before and after viral clearance: a single center, prospective, observational study.

Hepatitis C virus (HCV) chronic infection is frequently associated to autoimmune manifestations. The aim of this study was to prospectively evaluate the occurrence of clinical and/or laboratory features of autoimmunity in a cohort of 140 consecutive HCV chronically infected patients treated with direct-acting antiviral agents (DAAs) and followed-up for 96 weeks. All patients were screened for cryoglobulins, rheumatoid factor (RF), C3, C4, antinuclear antibody (ANA), anti-smooth muscle (ASMA), anti-liver kidney microsome type 1 (anti-LKM1), anti-mitochondrial antibodies (AMA), anti-neutrophil cytoplasmic antibodies (ANCA), and anti-liver cytosol type 1/soluble liver antigen (anti-LC1/SLA) autoantibodies before therapy and 12, 48 and 96 weeks after treatment. They were then grouped according to the expression of laboratory findings and related autoimmune diseases. At baseline, autoimmune manifestations were found in 70 patients: 83% of them were cryoglobulinemic, whereas ANA, AMA, perinuclear ANCA (pANCA) and LKM/LC1 autoantibodies were found in the remaining 17%. An autoimmune disease was diagnosed in 9 cases, two of them featuring an autoimmune liver disease (AILD). At the end of follow-up, despite viral clearance and regression of vasculitis, cryoglobulins persisted in 12 patients (21%), and autoantibodies disappeared or decreased in most of cases but, with the exception of the 2 patients diagnosed as AILD, associated autoimmune diseases remained stable. In one patient with relapsing cryoglobulinemia and ANA positivity, type-1 autoimmune hepatitis was defined. Conversely, autoantibodies first appeared after viral clearance in 5 patients, of whom one was diagnosed with type-1 autoimmune hepatitis and one with pANCA+ primary sclerosing cholangitis. Following DAA-induced viral clearance, cryoglobulins may persist or reappear. Autoantibodies changed dynamically in step with the disappearance of a previously diagnosed or the occurrence of a new AILD. A longer follow-up will be necessary to establish the possible diagnosis of a newly onset AILD, the reactivation of cryoglobulinemic vasculitis and even its progression to non-Hodgkin lymphoma.

Glomerulonephritis as a renal manifestation in a patient with systemic sclerosis overlapped with anti-neutrophil cytoplasmic antibody-associated vasculitis

Introduction and Importance: Systemic sclerosis (SSc) is a systemic immune disorder that may overlap with other rheumatologic disease; however, overlapping with antineutrophil cytoplasmic antibody-associated vasculitis is rare. Case Presentation: A 28-year-old Syrian male patient with SSc diagnosed according to the American College of Rheumatology/European League against Rheumatism 2013 criteria with a disease duration of 4 years, was admitted to the hospital complaining of palpable purpura in the lower limbs and hemoptysis and later, a rise in creatinine level. Laboratory tests showed high levels of perinuclear antineutrophil cytoplasmic antibodies (p-ANCA). The renal biopsy results were consistent with the diagnosis of glomerulonephritis. He was treated with methylprednisolone, cyclophosphamide, and rituximab, as he was diagnosed with SSc overlapping antineutrophil cytoplasmic antibody-associated vasculitis. Clinical Discussion: SSc most commonly renal manifestations are proliferative vasculopathy leading to scleroderma renal crisis. However, other types of renal involvement were also reported in SSc patients with comorbid autoimmune diseases such as glomerulonephritis and signs of concurrent vasculitis. SSc may overlap with rheumatoid arthritis, systemic lupus erythromatosus, polymyositis/dermatomyositis (PM/DM), and Sjogren Syndrome. Overlapping with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is mentioned in rare cases. The authors reported a rare case of overlapping SSc with antineutrophil cytoplasmic antibody-associated vasculitis with renal involvement. Conclusion: The authors revealed a rare case of overlapping SSc with antineutrophil cytoplasmic antibody-associated vasculitis with renal involvement. In SSc, renal involvement as glomerulonephritis is infrequent and should be detect in other rheumatologic disease such as systemic lupus erythematosus or antineutrophil cytoplasmic antibody-associated vasculitis.

Levamisole-Adulterated Cocaine-Induced Mucous Membrane Pemphigoid: Case Reports and Literature Review.

The aim of this study was to report 2 cases of levamisole-adulterated cocaine-induced mucous membrane pemphigoid. This study is a review of case reports and literature. Two patients presented with bilateral severe purulent conjunctivitis, corneal ulceration, and rapidly progressive forniceal shortening. Both patients were active cocaine users. A complete blood analysis showed a positive antineutrophil cytoplasmic antibody immunofluorescence with a mixed perinuclear antineutrophil cytoplasmic antibody and cytoplasmic-staining antineutrophil cytoplasmic antibody pattern. Direct immunofluorescence examination of conjunctival tissue showed linear deposition of component 3 and immunoglobulins at the basal membrane. A diagnosis of levamisole-adulterated cocaine-induced mucous membrane pemphigoid was made. In case 1, this suspicion was confirmed by investigating remnants of cocaine on the patient's debit card using mass spectrometry, which contained traces of levamisole. In both cases, aggressive immunosuppressive therapy combining systemic corticosteroids and rituximab was able to control the disease. However, by the time these therapies were initiated, significant corneal injury had occurred requiring corneal grafts in both patients. Given the rising abuse of cocaine, it is important that ophthalmologists are made aware of its association with severe atypical cicatricial conjunctivitis. To the best of our knowledge, we present the first case proving the causal relationship between levamisole and ocular cicatricial pemphigoid.

Activated phosphoinositide 3-kinase δ syndrome caused by PIK3CD mutations: expanding the phenotype

BackgroundGermline heterozygous gain-of-function (GOF) mutations in the PIK3CD gene lead to a rare primary immunodeficiency disease known as activated phosphoinositide 3-kinase (PI3K) δ syndrome type 1(APDS1). Affected patients present a spectrum of clinical manifestations, particularly recurrent respiratory infections and lymphoproliferation, increased levels of serum immunoglobulin (Ig) M, Epstein-Barr virus (EBV) and cytomegalovirus (CMV) viremia. Due to highly heterogeneous phenotypes of APDS1, it is very likely that suspected cases may be misdiagnosed.MethodsHerein we reported three patients with different clinical presentations but harboring pathogenic variants in PIK3CD gene detected by trio whole-exome sequencing (trio-WES) and confirmed by subsequent Sanger sequencing.ResultsTwo heterozygous mutations (c.3061G > A, p.E1021K and c.1574 A > G, p.E525G) in PIK3CD (NM_005026.3) were identified by whole exome sequencing (WES) in the three patients. One of two patients with the mutation (c.3061G > A) presented with abdominal pain and diarrhea as the first symptoms, which was due to intussusception caused by multiple polyps of colon. The patient with mutation (c.1574 A > G) had an anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV)-like clinical manifestations, including multisystemic inflammation, acute nephritic syndrome, and positive perinuclear ANCA (p-ANCA), thus the diagnosis of ANCA-AAV was considered.ConclusionsOur study expands the spectrums of clinical phenotype and genotype of APDS, and demonstrates that WES has a high molecular diagnostic yield for patients with immunodeficiency related symptoms, such as respiratory infections, multiple ecchymosis, ANCA-associated vasculitis, multiple ileocecal polyps, hepatosplenomegaly, and lymphoid hyperplasia.Trial registrationRetrospectively registered.

Measurement of Neutrophil Extracellular Traps as a Biomarker for the Differential Diagnosis Between Antineutrophil Cytoplasmic Antibody-Positive Individuals With Antineutrophil Cytoplasmic Antibody-Associated Vasculitis and Nonautoimmune Diseases.

Neutrophil extracellular traps (NETs) have a correlation with disease activity in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). However, it is not known whether there is an association between NETs and the presence of ANCA in other diseases. This study aimed to assess the occurrence of NETs in individuals with ANCA and whether serum NET quantitation is capable of distinguishing them with regard to the diagnosis. This was a cross-sectional, observational study. From the positive ANCA by indirect immunofluorescence, 94 individuals were divided into groups: AAV, infectious diseases, and neoplastic diseases. Healthy controls served for comparisons. Neutrophil extracellular traps were evaluated through the investigation of NET remnants, by detecting cell-free DNA bound to proteins such as histone, myeloperoxidase, and neutrophil elastase (NE). In patients with perinuclear ANCA (p-ANCA) the detection of NETs by NE was able to distinguish AAV from infection/neoplasia and healthy controls. Receiver operating characteristic curves for serum NETs by NE in patients with p-ANCA were drawn in 2 situations: AAV versus infection/neoplasia, showing a sensitivity of 0.65 and specificity of 0.84, with an area under the curve of 65%; and AAV versus controls, showing a sensitivity of 0.84 and a specificity of 0.88, with an area under the curve of 96%. For p-ANCA-positive individuals, we found higher serum NETs detected by NE-DNA in those with chronic infectious and neoplastic diseases than in AAV individuals and healthy controls. This allows us to infer that the evaluation of serum NETs may be of value as a biomarker for differential diagnosis.

Angiotensin II Type 2 Receptor Antibodies in Glomerular Diseases.

We evaluated the concentration of AT2R antibodies in 136 patients with primary and secondary glomerular diseases: membranous nephropathy (n = 18), focal and segmental glomerulosclerosis (n = 25), systemic lupus erythematosus (n = 17), immunoglobulin A (IgA) nephropathy (n = 14), mesangial (non-IgA) proliferative nephropathy (n = 6), c-ANCA vasculitis (n = 40), perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA) vasculitis (n = 16), and compared it with a healthy control group (22 patients). Serum creatinine levels, proteinuria, serum albumin, and total protein concentrations were prospectively recorded for 2 years. The mean levels of AT2R antibodies in the lupus nephropathy group were significantly higher compared to the control group, 64.12 ± 26.95 units/mL and 9.72 ± 11.88 units/mL, respectively. There was no association between this level and the clinical course of the disease. The AT2R levels in other kinds of glomerular disease were no different from the control group. We found significant correlations between AT1R and AT2R in patients with membranous nephropathy (r = 0.66), IgA nephropathy (r = 0.61), and c-ANCA vasculitis (r = 0.63). Levels of AT2R antibodies in systemic lupus erythematosus are higher compared to other types of glomerulonephritis, vasculitis, and a healthy control group. Levels of AT2R antibodies correlate with AT1R antibodies in the groups of patients with membranous nephropathy, IgA nephropathy, and c-ANCA vasculitis. These kinds of AT2R antibodies have a stimulative effect on AT2R, but we have not found the influence of these antibodies on the clinical course of glomerular diseases.

Incidence of Systemic Vasculitis and Clinical Outcomes in Systemic Sclerosis: 2-Years Follow-Up of Asymptomatic Positive for Anti-Neutrophilic Cytoplasmic Antibody.

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. 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. 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. 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.

Diagnostic utility, disease activity, and disease phenotype correlation of serum ASCA, pANCA, and PR3-ANCA in pediatric inflammatory bowel disease

ObjectiveThis study aimed to evaluate the diagnostic utility, disease activity, and phenotypic association of serum anti-Saccharomyces cerevisiae antibody (ASCA), perinuclear anti-neutrophil cytoplasmic antibody (pANCA), PR3-ANCA, and MPO-ANCA in pediatric patients with inflammatory bowel disease (IBD). MethodsPediatric patients diagnosed with IBD were recruited and classified as Crohn's disease (CD), ulcerative colitis (UC), and IBD-unclassified (IBD-U) through full investigation. The Paris classification was used to evaluate disease phenotypes of pediatric CD and UC. ResultsIn all, 229 pediatric patients with IBD (CD 147, UC 53, IBD-U 29) were included. The ASCA IgG seropositivity significantly differed among the three groups (CD 75.4%, UC 17.5%, and IBD-U 60.0%; p < 0.001). PR3-ANCA positive rates were the highest in UC (24.0%), followed by IBD-U (17.6%), and none in CD (p = 0.002); pANCA-positive rates were higher in IBD-U (33.6%), followed by UC (28.0%) than in CD (1.4%) (p < 0.001). Regarding disease phenotype, perianal disease revealed higher serum ASCA IgG titers (median 36.7 U/mL in P1 vs. 25.2 U/mL in P0, p = 0.019). Serum ASCA IgG and IgA cutoff values to distinguish CD were 32.7 (U/mL) and 11.9 (U/mL), respectively, with a specificity of 80.0%. ConclusionSerological biomarkers of ASCA IgG and IgA were effective for differentiating CD in pediatric IBD patients, and serum pANCA and PR3-ANCA, but not MPO-ANCA, were effective in distinguishing UC and IBD-U. Furthermore, measuring serological titers of ASCA IgG and IgA may help differentiate CD and evaluate the disease activity and phenotype of pediatric IBD in practice.

Two Cases of Perinuclear Anti-neutrophil Cytoplastic Antibodies Associated Hypertrophic Pachymeningitis Presented with Visual Loss

Pachymeningitis is an inflammatory process mainly involving the dura mater and is possibly associated with infectious, neoplastic, or autoimmune disease. However, in most cases, the etiology remains unknown. Clinical manifestations of pachymeningitis can vary, ranging from headache, cranial neuropathy, cerebellar dysfunction, to seizure. Here we report two cases of perinuclear anti-neutrophil cytoplasmic antibodies associated with pachymeningitis, in both the visual loss attributed to optic neuropathy was the primary sign.

Clinical significance of serum levels of Saccharomyces cerevisiae IgA, IgG and perinuclear antineutrophil cytoplasmic antibodies in the differential diagnosis of inflammatory bowel diseases

Clinical significance of serum levels of Saccharomyces cerevisiae IgA, IgG and perinuclear antineutrophil cytoplasmic antibodies in the differential diagnosis of inflammatory bowel diseases

Autoantibodies to beta tubulin in autoimmune liver diseases-Relation to pANCA and clinical relevance.

There was evidence that perinuclear antineutrophil cytoplasmic antibodies (pANCA) in autoimmune liver diseases react with human beta-tubulin-5 (TBB5). Here, we reevaluate the specificity and clinical relevance of anti-TBB5 antibodies. Patients with untreated autoimmune hepatitis (AIH; n = 53), AIH under immunosuppressive therapy (AIH-IS; n = 125), primary sclerosing cholangitis (PSC; n = 40), primary biliary cholangitis (PBC; n = 250), nonautoimmune liver diseases (n = 158), inflammatory bowel diseases (IBD; n = 30), and healthy individuals (n = 62) were tested by enzyme-linked immunosorbent assay for IgG- and IgA-antibodies against recombinant human TBB5. pANCA were detected by immunofluorescence test. Sera were absorbed with TBB5 coupled to cyanogen bromide-activated sepharose. Prevalence and reactivity of IgG anti-TBB5 were significantly higher in patients with untreated AIH (68%; arbitrary units [AU] median: 369) than in PSC (28%; AU median: 84, P < 0.001), other liver diseases (14%; AU median: 185, P < 0.0001), IBD (3%; AU median: 111, P < 0.0001), and healthy controls (3%; AU median: 135; P < 0.0001). Anti-TBB5 did not correlate with pANCA, and immunoprecipitation with TBB5 did not abolish pANCA reactivity. In untreated AIH, anti-TBB5-reactivity was significantly higher than in AIH-IS. Transaminases decreased under IS preferentially in anti-TBB5-negative patients. There was no correlation between anti-TBB5-reactivity and histological stages. IgA-anti-TBB5 was mainly found in alcohol-associated liver disease (ALD; 39%). Our data do not support TBB5 as an autoantigenic target of pANCA. However, IgG-anti-TBB5 showed high specificity for (untreated) AIH. While they did not correlate with histological and laboratory parameters, their presence may indicate a poor response to IS.

SAT216 Multiple Reasons Of Secondary Hyperthyroidism And Unexpected Results

Abstract Disclosure: I. Dzherieva: None. N. Volkova: None. S. Brovkina: None. I. Reshetnikov: None. L. Gritsenko: None. T. Mamedova: None. U. Ismailov: None. Background: Revealing the cause of secondary hyperparathyroidism might be a difficult problem especially if a patient has a few diseases to lead to this condition. Clinical Case: A 52-year-old-man was referred to our service for revealing the cause of weakness which started after COVID 19. When the patient was treated by rivaroxaban there was an unusual feature such as a hemoptysis, so the medicine was canceled. In medical history only the Achilles tendovaginitis was performed. The patient plays football twice a weak regularly, does not smoke, does not use drags. The physical examination showed nothing special. An arterial blood pleasure was 120/80 mm Hg, Body Mass Index was 25,4 kg/m2. Laboratory workup showed high erythrocyte sedimentation rate (ESR)(33mm/hr), leukocytosis (11000mm3) and a normocytic anemia(Hb 115 g/l),hematuria (16650 RBCsper hpf). Creatinine was 163µmol/L, glomerular filtration rate (GFR) was 43 ml/min/1.73m 2 , total serum calcium corrected for the patient's albumin concentration was 2.47 mM/L (2.15-2.50 mM/L), parathyroid hormone (PTH-intact) was 130 pg/mL (10-65 pg/mL),the level of 25-hydroxyvitamin D was 14.4 ng/mL(30.0-100.0 ng/mL). Chest radiograph findings were as follows: bilateral irregular, nodular, and patchy opacities. After vitamin D deficiency correction level of PTH-intact became normal (65 ng/mL). Serological tests : antinuclear antibody [ANA] and double-stranded DNA [dsDNA], ANCA were performed. Antineutrophil cytoplasmic antibody (ANCA) test result revealed perinuclear ANCA related to myeloperoxidase with the level more than 80 IgG. According to this finding Microscopic polyangiitis was diagnosed. Conclusion: This case has shown that sometimes there are two reasons for development of secondary hyperthyroidism and only successive actions help to choose right way for diagnosis. Presentation: Saturday, June 17, 2023

Propylthiouracil induced ANCA-positive vasculitis in a patient with Graves’ disease; a case report

Anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis is rare, but it can be triggered by chemicals, infections, and drugs. Patients who use anti-thyroid drugs are prone to involve with ANCA-associated vasculitis. Perinuclear ANCA (p-ANCA) is almost always positive in these patients. Patients have various presentations and symptoms such as (arthritis, edema) and this disorder usually resolves with discontinuation of the drug, however, some patients require high-dose steroids, immunosuppressive or plasmapheresis. A 38-year-old woman with a history of Graves’ disease was on long-term treatment with propylthiouracil (PTU), presented with severe bone pain, arthritis and edema in both feet. The patient’s manifestations were resolved with discontinuation of PTU, iodine therapy, and corticosteroid administration.

Cocaine-induced pyoderma gangrenosum with negative anti-neutrophil cytoplasmic antibodies: A case report

Introduction: Cutaneous pyoderma gangrenosum (PG) is an ulcerating autoinflammatory neutrophilic dermatosis often associated with inflammatory bowel disease, hematologic diseases, or arthritis. PG secondary to systemic medications is less commonly reported. PG is a diagnosis of exclusion that requires investigation of other causes of cutaneous ulcers prior to final diagnosis. Case presentation: We herein describe a case of cocaine-induced PG with atypical features (recurrent ulcerations limited to the lower extremities and perinuclear anti-neutrophil cytoplasmic antibody negativity) following years of abstinence from cocaine. Discussion: PG is a rare autoinflammatory neutrophilic dermatosis and a diagnosis of exclusion because of its nonspecific clinical presentation. Drug-induced PG is less common but presents an opportunity to further research the pathogenesis of PG. Conclusion: This unusual case reinforces that drug-induced PG may have non-classical features and may serve as an indicator of past or present substance abuse, allowing for patient education and counseling.