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Introduction and aim. Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, presents a formidable challenge in the realm of autoimmune diseases. Granulomatosis, characterized by vasculitis and granuloma formation, exhibits diverse clinical manifestations. The rarity of GPA is evident, with an estimated incidence between 0.4 and 11.9 cases per 1 million person-years. The aim of this report is to show the complex diagnostic challenges inherent in GPA, demonstrating the diagnostic process from initial symptoms. Description of the case. This case report unfolds the diagnostic journey of a 52-year-old Caucasian male. The presented case, initially suspected as a respiratory infection, led to a comprehensive investigation owing to persistent symptoms, abnormal blood counts, and elevated inflammatory markers. This narrative aims to depict the patient's diagnostic journey. Key diagnostic tools include ANCA testing, imaging studies, and tissue biopsy. Pulmonary nodules, lymphangitic changes, and renal involvement culminating in a GPA diagnosis confirmed by positive ANCA and anti-PR3 antibodies. The successful management of this case involved a tailored therapeutic regimen, including cyclophosphamide and methylprednisolone, addressing both vasculitic and renal components. Conclusion. This case contributes to the understanding of atypical presentations of GPA, emphasizing the importance of a holistic and dynamic diagnostic approach. [ABSTRACT FROM AUTHOR]

The article describes a case of a female patient who experienced complications after the treatment of her granulomatosis with polyangiitis using rituximab and avacopan. Topics mentioned include the manifestation of severe prolonged liver abnormality with jaundice, the results of viral serological tests for hepatitis B virus and hepatitis virus C, and the histological examination of liver biopsy.

Coronavirus disease 2019 (COVID-19) was declared a global pandemic and a public health emergency worldwide in March 2020. COVID-19 presents with non-specific symptoms of the upper airway and pulmonary system, which can overlap with other diseases involving the respiratory system as granulomatosis with polyangiitis (GPA). Both diseases have high morbidity and mortality rates and it is important to promptly differentiate and treat them. Real-time reverse transcriptase polymerase chain reaction (RT-PCR) is currently the recommended method for diagnosing COVID-19. Antibody-based tests are used to diagnose both pat and current COVID-19 infections. We present a previously healthy thirteen-year-old girl who was admitted with upper airway symptoms and pulmonary involvement, and progressed to acute kidney failure. Laboratory findings showed leukocytosis, anemia, elevated kidney function tests and 2+ proteinuria. Computed tomography (CT) of the lungs showed multiple nodules, cavities, and ground-glass opacities (GGOs). We performed RT-PCR tests for COVID-19 for three times. Results were all negative, but the COVID-19 immunoglobulin (Ig)M test sent simultaneously was positive. Based on the cytoplasmic antineutrophilic cytoplasmic antibody (c-ANCA) positivity, upper airway, pulmonary, and renal involvement, she was diagnosed as GPA. This report highlights that COVID-19 antibody tests can be false-positive in patients with autoimmune diseases including GPA. [ABSTRACT FROM AUTHOR]

Objectives This study applied the 2022 criteria for granulomatosis with polyangiitis (GPA) proposed by the ACR and EULAR (the 2022 ACR/EULAR criteria) to Korean patients with previously diagnosed GPA to investigate the number of patients who could be reclassified as having GPA. Methods Sixty-five patients with GPA, who met the 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides and the 2007 European Medicines Agency algorithm for GPA, were included in this study. They were reclassified based on the 2022 ACR/EULAR criteria. Results Of the 65 patients, 48 patients (73.8%) were reclassified as having GPA. A patient could not be reclassified as having GPA if the patient did not have a total score of 5 despite granulomas on biopsy or clear GPA surrogate markers. Among the 17 patients unclassified as having GPA, 16 patients were reclassified as having MPA and one as having unclassifiable vasculitis, and furthermore, 94.1% of them harboured MPO-ANCA (or perinuclear (P)-ANCA). Conclusion The concordance rate between the 2022 ACR/EULAR criteria for GPA and the previous criteria in patients with previously diagnosed GPA was 73.8%. Although the 2022 ACR/EULAR criteria are the product of the most advanced methodologic process, it should be noted that there were some consequences of distorting the CHCC definition, and further discussion is required, especially with respect to the weightage of the items. [ABSTRACT FROM AUTHOR]

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Acute respiratory distress syndrome (ARDS) can be associated with a wide multiplicity of causes, including diffuse alveolar haemorrhage (DAH). Vasculitis associated with anti-neutrophil cytoplasmic antibody (ANCA), particularly granulomatosis with polyangiitis (GPA), is one of the most common causes of DAH, primarily affecting small and medium-sized vessels in the lungs and kidneys. Diagnosing GPA can be challenging, and it should be considered a potential cause of DAH, even in the absence of other organ involvement. Given the high mortality associated with this condition, early diagnosis and aggressive treatment are crucial for improving outcomes. We present the case of a young patient with severe ARDS who did not show significant alveolar haemorrhage on bronchoscopy or renal involvement but exhibited extensive gastrointestinal involvement and had a predominant laboratory diagnosis of positive cytoplasmic anti-neutrophil cytoplasmic antibody and proteinase 3 antibodies., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Purpose: This study aimed to investigate whether the serum extracellular newly identified receptor for advanced glycation end products binding protein (EN-RAGE) and the soluble form of RAGE (sRAGE) measured at diagnosis are associated with all-cause mortality in patients with microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA)., Materials and Methods: Serum EN-RAGE and sRAGE were measured in 75 immunosuppressive drug-naïve MPA and GPA patients using an immunoassay, with their clinical and laboratory data reviewed. The optimal cut-off point of EN-RAGE and sRAGE was calculated by finding the threshold with the maximum sum of sensitivity and specificity. In addition, the least absolute shrinkage and selection operator regression was adopted to select variables included in the multivariable Cox proportional hazards (PH) regression model., Results: The median age of the patients was 67.0 years, and 34% were male. Neither serum EN-RAGE nor sRAGE at diagnosis was correlated with the Birmingham Vasculitis Activity Score. Furthermore, no correlation was observed between serum EN-RAGE and sRAGE. Deceased patients had significantly lower serum EN-RAGE and higher serum sRAGE at diagnosis compared to surviving patients. Patients with serum EN-RAGE at diagnosis ≤84.37 ng/mL and serum sRAGE at diagnosis ≥1.82 ng/mL showed significantly lower survival probabilities compared to those without. In multivariable Cox PH regression model, only serum sRAGE at diagnosis ≥1.82 ng/mL, rather than serum EN-RAGE at diagnosis ≤84.37 ng/mL, was independently associated with all-cause mortality (hazard ratio 7.094)., Conclusion: This study is the first to demonstrate that serum sRAGE at diagnosis may independently predict all-cause mortality during follow-up in patients with MPA and GPA., Competing Interests: The authors have no potential conflicts of interest to disclose., (© Copyright: Yonsei University College of Medicine 2024.)

Hypereosinophilic syndrome (HES) and eosinophilic granulomatosis with polyangiitis (EGPA) are complex disorders defined by blood and tissue eosinophilia and heterogeneous clinical manifestations. Historically, the mainstay of therapy for both conditions has been systemic glucocorticoids. However, recent availability of biologics that directly or indirectly target eosinophils has provided new avenues to pursue improved outcomes with decreased toxicity. In this article, we summarize the evidence supporting the use of specific biologics in HES and/or EGPA and provide a framework for their clinical use in patients., Competing Interests: Disclosure The authors have nothing to disclose. This work was supported in part by the Division of Intramural Research, NIAID, NIH., (Published by Elsevier Inc.)

Introduction: Aspergillosis is an opportunistic infection that can complicate any situation of immunosuppression. The primary manifestations are pulmonary, and more rarely, in cases of severe immunosuppression, the infection can become invasive with extra-pulmonary involvement., Observation: We report the case of a 76-year-old female patient, experiencing a relapse of granulomatosis with polyangiitis treated with corticosteroids, rituximab and cyclophosphamide, who presented with diffuse erythematous nodular skin lesions. A biopsy with histological analysis confirmed a diagnosis of invasive cutaneous aspergillosis. Treatment with voriconazole led to a favorable outcome., Conclusion: The appearance of skin lesions in an inflammatory context in a patient receiving immunosuppressive therapy should prompt a comprehensive microbiological assessment for opportunistic pathogens, as well as a skin biopsy to investigate for invasive cutaneous aspergillosis., (Copyright © 2024 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.)

Idiopathic orbital inflammation (IOI) is a diagnosis of exclusion, but the exclusion of other inflammatory diseases of the orbit is broad and relies on clinician experience, response to corticosteroid, or biopsy. This study aimed to investigate the presence of granulomatosis with polyangiitis (GPA) in patients initially diagnosed as IOI and describe its clinicopathological features, ANCA status, treatment, and outcome. We performed a retrospective case series study of children diagnosed with limited GPA (L-GPA) in patients with IOI. A systematic review of the literature was performed in children with GPA and orbital mass. Eleven of 13 (85%) patients with IOI had L-GPA. Two additional patients with orbital mass and L-GPA were included in this analysis. The median age was 10 years, and 75% were female. Twelve cases were ANCA positive and 77% were MPO-pANCA positive. Most patients had a poor response to treatment and had a high relapse rate. Based on literature review, 28 cases were found. Most (78.6%) were female with a median age of 9 years. Three patients were misdiagnosed as IOI. Patients with L-GPA more frequently had MPO-pANCA positivity (35%) than children with systemic GPA (18%) and were less often PR3-cANCA positive than patients with systemic GPA (18% vs. 46%). L-GPA accounts for a high prevalence of children diagnosed as IOI. The high prevalence of MPO-pANCA observed in our study may be related to L-GPA rather than with the orbital mass. Long-term follow-up, orbital biopsy, and serial ANCA testing are necessary to exclude GPA in patients with IOI., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

ANCA-associated vasculitides (AAV) comprise granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis. All forms may involve different organ systems, yet kidney and lung involvement are common and fatal in many cases. Here, we aimed to determine the predictive value of pulmonary disease manifestation and individual CT findings in AAV patients. Available CT scans and clinical information on mortality, renal outcomes, occurrence of relapses and damage scores were analysed retrospectively from a tertiary rheumatology center in Germany. We included a total of 94 AAV patients (49 with GPA, 41 with MPA). Forty-four patients had lung involvement with available CT scans, 70.5% of which with GPA and 72.7% with renal involvement. Nodule formation and cavities were more frequent among GPA patients, whereas ground-glass opacities (GGO), ILD and pleural effusion were observed predominantly in MPA patients. Over a median follow-up of 37 months, GPA patients had a slightly higher overall mortality, whereas end-stage kidney failure rates were significantly increased in MPA patients. Relapse frequencies were comparable between both entities. The presence of GGO and pleural effusion were associated with higher relapse rates, whereas nodules were negatively correlated with relapses. Notably, RTX-treated patients had less infections as compared to individuals under different therapies. Our data demonstrate the outstanding importance of characteristic CT patterns in AAV diagnosis assessment. Especially certain CT patterns including GGO and pleura effusion may help to identify patients who are at higher risk for relapsing disease., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Severe eosinophilic asthma (SEA) may be the prodromal phase of eosinophilic granulomatosis with polyangiitis (EGPA). Nevertheless, few studies have tried to recognize EGPA in the early stages of the disease., Objective: To identify a panel of clinical and biological markers to detect which severe asthmatic patient might be considered in a prodromal phase of EGPA and crafting a strategy for diagnostic decision-making., Methods: A total of 30 patients with EGPA and 49 with SEA were enrolled. A complete pulmonary, ear, nose, and throat, and rheumatologic assessment were made. Blood (eosinophil count, eosinophilic cationic protein, IL-5, IL-4, total-IgE, IgG 4 , and antineutrophil cytoplasmic antibody), sputum (eosinophils count, periostin, IL-8, and granulocyte-monocyte colony-stimulating factor [GM-CSF]), and nasal smear (eosinophilia) biomarkers were assessed. Asthma Control Test, Short Form-36, SinoNasalOutcome Test-22, and Asthma Quality of Life Questionnaire were also used., Results: Patients with SEA had poorer asthma control (P < .001) and a higher level of sputum eosinophils (P < .002), whereas patients with EGPA reported higher levels of blood eosinophils in the past. Sputum GM-CSF was the only biomarker significantly increased in patients with EGPA compared with those with SEA (P < .0001). Among patients with SEA, those with some suggestive but not diagnostic criteria of EGPA, particularly tissue eosinophilic infiltrates, presented higher levels of sputum GM-CSF (P < .0005), blood, and sputum eosinophils (P < .0006 and P < .011) than the other patients., Conclusion: Sputum GM-CSF and eosinophils might be useful biomarkers to support early diagnosis and treatment choices in patients with SEA, suspected of having EGPA., (Copyright © 2024 American Academy of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.)

Background : Ocular involvement is relatively common in granulomatosis with polyangiitis (GPA); however, choroidal involvement is rare. We present a case of serous retinal detachment resulting from choroidal involvement in GPA. Case presentation : A 55-year-old male presented to our clinic with bilateral eye redness and pain. Ocular examination revealed bilateral conjunctival injection, and dilation of the episcleral and scleral vessels. Slit-lamp examination revealed anterior chamber cells. Optical coherence tomography (OCT) confirmed serous retinal detachment (SRD) in the left eye. The patient had recently been diagnosed with GPA following a lung biopsy and had received immunosuppressive therapy, including systemic steroids, cyclosporine, mycophenolate mofetil, and rituximab. Five weeks after treatment, the clinical symptoms of the patient, including SRD, improved with alleviation of systemic symptoms. However, tapering systemic steroids and immunosuppressants and discontinuing rituximab led to disease reactivation. OCT demonstrated a recurrence of subretinal fluid, which had previously resolved, and slit-lamp examination showed mild bilateral engorged scleral vessels. Conclusions : Choroidal involvement can present as SRD and may indicate disease activity in patients with GPA.

Objectives: Ceruloplasmin is an inhibitor of myeloperoxidase (MPO) activity that plays an important role in the pathophysiology of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV). This study aimed to evaluate the prognostic impact of serum level of ceruloplasmin at diagnosis in patients with anti-MPO antibody-positive AAV., Methods: This retrospective monocentric study in Caen University Hospital involved all consecutive adult anti-MPO antibody-positive patients with microscopic polyangiitis or granulomatosis with polyangiitis, diagnosed between January 2010 and January 2022 with available serum sample at inclusion. Patients outcomes were analyzed from two subgroups constituted according to the median serum level of ceruloplasmin. The same analyses were then performed in anti-proteinase 3 (PR3) antibody-positive patients., Results: Within the 92 patients analyzed, 50 patients had anti-MPO antibodies with a median ceruloplasmin level of 0.44 [quartiles 1-3, 0.40-0.49] g/L and a median Birmingham Vasculitis Activity Score of 19 [14-22]. After a median follow-up period of 40 [22-86] months, 13 (26%) patients had died: 10 (40%) in the low ceruloplasmin group and 3 (12%) in the high ceruloplasmin group (p = 0.03), with a significantly worse survival rate in the low ceruloplasmin group (p = 0.021). No significant differences in relapse rate or renal failure was observed between the two groups. The same analyses performed in the group of AAV patients with anti-PR3 antibody did not show any differences., Conclusion: In anti-MPO AAV patients, serum level of ceruloplasmin at diagnosis seems to be associated with a significant impact on survival., Competing Interests: The authors have declared that no competing interests exist., (Copyright: © 2024 Camboulive et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.)

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare form of ANCA-associated vasculitis (AAV) within the group of small vessel vasculitides. It is defined by vasculitis of small and medium-sized vessels with granulomatous inflammation and blood and tissue eosinophilia. Almost all patients have allergic symptoms with bronchial asthma and rhinosinusitis symptoms. Further clinical manifestations vary depending on the localisation, severity, and type of disease manifestation. Eosinophilic infiltration and inflammation may result in rhinosinusitis, pneumonitis, gastrointestinal involvement, and cardiomyopathy. The latter, in particular, is associated with a poorer prognosis. As a necrotising pauci-immune small-vessel vasculitis, EGPA, similar to the other AAVs, can cause pulmonary infiltrates with alveolar haemorrhage, glomerulonephritis, cutaneous vasculitis with purpura as well as central and peripheral neurologic injuries. The presence of perinuclear ANCA (pANCA) with specificity against myeloperoxidase (MPO) is observed in approximately one-third of patients but is not specific to EGPA. MPO-ANCA-positive patients are more likely to have peripheral neurologic involvement and glomerulonephritis, whereas ANCA-negative patients are more likely to have cardiac and pulmonary involvement. What is frequently challenging in the clinical routine is to differentiate EGPA from the hypereosinophilic syndrome (HES). The therapeutic approach to EGPA depends on whether the severity of the disease is potentially organ or life-threatening. For severe forms of EGPA, acute therapy mainly includes glucocorticoids in combination with cyclophosphamide. Rituximab has come to be mentioned as an alternative treatment option in the guidelines. Various immunosuppressive therapies are available for remission maintenance. In EGPA without severe organ involvement, IL-5 blockade with mepolizumab is an approved treatment., Competing Interests: N. Venhoff hat Vortragshonorare und Beraterhonorare von GSK, Chugai/Roche und Vifor erhalten. V. Schindler gibt an, dass in Bezug zu diesem Artikel kein Interessenkonflikt besteht., (Thieme. All rights reserved.)

Purpose: To report a patient with bilateral scleritis who was initially treated for infectious scleritis, and subsequently diagnosed as granulomatosis with polyangiitis (GPA)., Method: Retrospective chart review., Result: A 48-year-old female with a known history of diabetes presented with pain, redness, and blurring of vision in the right eye. She was diagnosed as scleritis with retinal detachment and underwent vitrectomy, with silicone oil tamponade and intravitreal injections of antibiotics, and antifungal agents. She presented with active scleritis with additional multiple pus points. Several scleral biopsies failed to yield any microorganism and finally, the painful blind eye was enucleated. The enucleated specimen grew gram-positive bacteria which were identified as Staphylococcus arlettae by polymerase chain reaction (PCR)-based sequencing of the 16S rRNA gene. Nine months after the onset of symptoms in the right eye, the patient developed necrotizing scleritis in the left eye. Laboratory investigation revealed a positive cytoplasmic- anti-neutrophil cytoplasmic autoantibody, which was previously negative during the right eye involvement. She was diagnosed with GPA by a rheumatologist and treated accordingly., Conclusion: Retinal detachment may be associated with scleritis, and ANCA testing may not detect GPA in its early stages. Once infection has been excluded, clinicians should not be afraid to use high-dose immunosuppression instead of surgery to treat retinal detachment associated with scleritis.

Competing Interests: Declaration of competing interest The authors declare they have no conflict of interest.

Background: Eosinophilic granulomatosis with polyangiitis (EGPA), is a rare ANCA-associated systemic vasculitis. Its overlapping features with other vasculitic or eosinophilic diseases, and the wide and heterogeneous range of clinical manifestations, often result in a delay to diagnosis., Objective: To identify red flags that raise a suspicion of EGPA to prompt diagnostic testing and to present an evidence-based clinical checklist tool for use in routine clinical practice., Methods: Systematic literature review and expert consensus to identify a list of red flags based on clinical judgement. GRADE applied to generate a strength of recommendation for each red flag and to develop a checklist tool., Results: 86 studies were included. 40 red flags were identified as relevant to raise a suspicion of EGPA and assessed by the experts as being clinically significant. Experts agreed that a diagnosis of EGPA should be considered in a patient aged ≥6 years with a blood eosinophil level >1000 cells/µL if untreated and >500 cells/µL if previously treated with any medication likely to have altered the blood eosinophil count. The presence of asthma and/or nasal polyposis should reinforce a suspicion of EGPA. Red flags of asthma, lung infiltrates, pericarditis, cardiomyopathy, polyneuropathy, biopsy with inflammatory eosinophilic infiltrates, palpable purpura, digital ischaemia and ANCA positivity, usually anti-myeloperoxidase, among others, were identified., Conclusion: The identification of a comprehensive set of red flags could be used to raise a suspicion of EGPA in patients with eosinophilia, providing clinicians with an evidence-based checklist tool that can be integrated into their practice., Competing Interests: Declaration of competing interest Iñigo Rúa-Figueroa: Lectures and advisors boards for GSK. No other conflicts of interest. Roser Solans Laqué: Honoraria for consultancy and conferences from GSK, and conferences from CSL- Vifor. Marina Blanco Aparicio declares to have received payment or honoraria for lectures, presentations, speakers’ bureaus, or educational events and support for attending meetings and/or travel for AstraZeneca, GSK, Sanofi, Chiesi, TEVA and Faes. Ismael García Moguel: Consultant and speaker: Novartis, AstraZeneca, Teva, Novartis, GSK, Sanofi Genzyme, Chiesi, Allergy therapeutics, Leti, Stallergenes, ALK-Abelló, Mundipharma, Pfizer, and Orion Pharma. PI clinical trials: Novartis, GSK, AstraZeneca, Sanofi Genzyme. Ricardo Blanco: Grants/research support from AbbVie, MSD, and Roche, and had consultation fees/participation in a company-sponsored speaker's bureau from AbbVie, Pfizer, Roche, Lilly, UCB, Bristol-Myers, GSK, Janssen, Novartis and MSD. Francisco Pérez Grimaldi: Honoraria for conferences from AstraZeneca, GSK, Sanofi, Novartis, Chiesi, Teva, Esteve, Menarini y Boehringer-Ingelheim. Ana Noblejas Mozo: Colaboration with CSL Vifor, Gebro Pharma, Astra Zeneca. Colaboration in a vasculitis meeting with patients: CSL Vifor. Honoraria for consultancy from GSK. Moises Labrador Horrillo: Payment for lectures including service on speakers’ bureaus from: AstraZeneca, GSK, Novartis and Sanofi. Consultancy from: AstraZeneca, Novartis and GSK. J.M. Álvaro-Gracia: Honoraria for lecturing, advisories and Congress attendance from AbbVie, AstraZeneca, Galapagos, Gilead, Pfizer, Novartis, GSK, Lilly, MSD y UCB. Christian Domingo Ribas. CDR has acted as consultant for GSK, AstraZeneca and Sanofi and has received funding for travel or speaker fees from ALK, Almirall, AstraZeneca, Boehringer Ingelheim, Chiesi, GSK, Menarini, MSD, Novartis, Stallergenes and Pfizer. Georgina Espígol-Frigolé: Honoraria for consultancy from Janssen, GSK and CSL-Vifor. Congress attendance from Boehringer-Ingelheim. Fernando Sánchez Toril. I have received personal fees (payments for presentations, advisory boards, etc.) from: ALK, AstraZeneca, Bial, Boehringer Ingelheim, Chiesi, FAES, Gebro Pharma, GSK, Menarini, Novartis, Pfeizer, TEVA and Sanofi. I have participated in clinical trials and received research grants from: AstraZeneca, Chiesi, GSK, Novartis, TEVA, Sanofi. I am not employed (including part-time) in any pharmaceutical industry. I do not maintain any relationship with the tobacco industry. Francisco Ortiz Sanjuán: Advisor, Review Panel member or Consultant fee from GSK, Grünenthal and received financial aid for attendance at medical conferences from Lilly. Ebymar Arismendi: Honoraria for consultancy, advisory board and/or conferences from GSK, AstraZeneca, MSD, Novartis, Chiesi, Gebro Pharma and Sanofi-Genzyme. Maria C Cid: Consulting fees from GSK, AbbVie, CSL- Vifor and AstraZeneca; royalties from UpToDate, and a research grant from Kiniksa Pharmaceuticals Corp., (Copyright © 2024. Published by Elsevier B.V.)

Children with anti-neutrophil cytoplasmic antibody-associated vasculitis benefit immensely from avacopan as it reduces the requirement for steroids. However, descriptions of adverse drug reactions in children are lacking, and the dosage and follow-up intervals are unclear. A 10-year-old boy with initial granulomatosis and polyangiitis presented with diffuse pulmonary hemorrhage. Rituximab and 30 mg avacopan were administered twice daily as induction therapy following methylprednisolone pulse therapy. However, sudden liver function test abnormalities were observed on day 31 of avacopan treatment, despite liver enzyme levels being within the normal range 5 days earlier. A drug-induced lymphocyte stimulation and various infectious disease tests yielded negative results. Discontinuation of rituximab and avacopan resulted in improved liver function; no change in the Birmingham Vasculitis Activity Score during liver function test abnormalities was observed. Avacopan-associated abnormalities in liver function tests suggest that drug-induced liver injury may occur rapidly in children, and appropriate dosing strategies should be reconsidered., (© 2024. The Author(s), under exclusive licence to International Pediatric Nephrology Association.)

Granulomatosis polyangiitis (GPA) is an autoimmune condition causing inflammation of small blood vessels. It is a rare disorder that may affect various parts of the body. The diagnosis is often based on clinical examination, laboratory investigations and tissue biopsy. In about 10-20% of patients, the anti-neutrophilic cytoplasmic antibody (ANCA) can be negative, and histology maybe inconclusive, which can lead to diagnostic uncertainty. Failure to treat vasculitis can lead to morbidity and even mortality. We present a case report of a gentleman who was presented with an airway emergency with inflammation of the nasal cavity and subglottic involvement amounting to airway stenosis. His ANCA was negative and tissue biopsy from the subglottis was inconclusive. He underwent urgent dilatation of his airway, local therapies to the nose and was commenced on 10 cycles of cyclophosphamide. A follow-up of over 4 years has not shown any relapse of his disease clinically or biochemically. We discuss the clinical findings, diagnostic dilemma and multidisciplinary management of this life-threatening condition., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Background and objectives : The initial symptom that triggers granulomatosis with polyangiitis (GPA) diagnosis is rarely ocular. We describe a case with a single ocular lesion identified as probable GPA due to proteinase 3 (PR3)-antineutrophil cytoplasmic antibody (ANCA)-positivity according to the diagnostic criteria of the Ministry of Health in Japan; the lesion repeatedly worsened. Materials and methods : A 25-year-old female visited the Department of Ophthalmology, Asahi General Hospital, with upper eyelid swelling and conjunctival and episcleral hyperemia of the left eye. Both hordeolum and eyelid cellulitis were suspected, as the condition was resistant to treatment with antibiotic eye drops. Episcleritis was suspected due to localized hyperemia in the upper part of the eye. Upon treatment with antibacterial agents and steroid eye drops, the swelling and the hyperemia repeatedly worsened every week. Results : Blood samples were positive for PR3-ANCA, and GPA with an isolated ocular lesion was considered. After oral steroid treatment, the patient had no recurrence for 4 years. There was no systemic involvement in the upper respiratory tract, lungs, or kidneys. Conclusions : Diagnosing GPA with ocular symptoms as initial manifestations is challenging. GPA should be considered in treatment-resistant eyelid, orbital, and episcleral lesions, even at a young age.

Rationale: Pathological featuring by necrotizing granulomatous inflammation of peripheral blood and tissues with increased eosinophils infiltrating small and medium vessels, eosinophilic granulomatosis with polyangiitis (EGPA), a family of rare antineutrophil cytoplasmic antibody (ANCA) associated with systemic vasculitis. With low morbidity, diverse clinical manifestations, and difficult early diagnosis, the majority of patients are confirmed after multiple organ damages, thus missing the best treatment time and having a poor prognosis. About 25% to 30% of EGPA cases have been reported to suffer from the renal disease, and there are few studies on EGPA complicated with kidney damage, most of them on ANCA-positive patients. Generally, the initial diagnosis of EGPA on maintenance hemodialysis is even rare. We report a case of a patient with maintenance hemodialysis for 5 years and then was diagnosed with EGPA., Patient Concerns: The female patient, 54-year-old, having maintenance hemodialysis for 5 years consecutively, was hospitalized for the recurring rash in the past 3 years and then exacerbation in the last 2 months. With the previous history of bronchial asthma having attacked frequently recently, it could be observed from peripheral blood that the eosinophils increased, from the cardiac color ultrasound that it was prone to eosinophilic endocarditis, from 5 tests for vasculitis that P-ANCA and MPO-AB were positive., Diagnoses: The patient's onset is renal dysfunction, with maintenance hemodialysis for 5 years, recurrent lung infections, combined with eye lesions, scattered skin rashes, P-ANCA positive, MPO-AB positive, asthma present, eosinophil absolute value 1.60 × 109/L, total score >6 points, diagnosis considering EGPA., Interventions: Due to multiple organ damage, the patient received treatment with a combination of steroids and cyclophosphamide., Outcomes: After 2 days, the patient's rash significantly darkened compared to before, wheezing improved, and eosinophils returned to normal levels., Lessons: The ANCA test shall be put on the high agenda for patients presenting with kidney failure at first. Meanwhile, the neglected immune monitoring for patients with dialysis tells us that it is of great significance for this kind of patient to have immune monitoring in the early diagnosis of EGPA., Competing Interests: The authors declare that there is no conflict of interest in writing this case., (Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.)

Rationale: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare autoimmune disease that can affect multiple organ systems. The standard treatment mainly relies on glucocorticoids and immunosuppressive agents. In our study, we present an EGPA patient who had pulmonary tuberculous mycobacteria infection, such cases are rarely reported., Patient Concerns: A 71-year-old male patient was diagnosed with EGPA (systemic type) and pulmonary tuberculosis simultaneously., Diagnoses: The Five-Factor score indicated that the patient required glucocorticoids combined with immunosuppressive agents for induction therapy, however, the use of immunosuppressive agents would significantly inhibit antituberculosis treatment. Nowadays, treating active autoimmune disease in patients with infections remains a clinical challenge., Interventions: Considering the patient did not show life-threatening or severe organ involvement and reduced the effect of antituberculosis immunity, we used glucocorticoids alone., Outcomes: Finally, the patient had no adverse events, the eosinophil counts were markedly decreased and symptoms of EGPA were relieved., Lessons: The patient of EGPA combined with pulmonary tuberculosis successfully treated with glucocorticoids alone may provide significant support in selecting the appropriate treatments for similar cases in the future., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.)

Objective: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is divided into granulomatosis with polyangiitis (GPA), microscopic polyangiitis, and eosinophilic GPA. It is one of the most severe and potentially fatal autoimmune inflammatory conditions. The etiology and pathology of AAV are complex and poorly understood. Since the onset of the Coronavirus Disease 2019 (COVID-19) pandemic, numerous reports have documented GPA cases following COVID-19, suggesting a potential link between COVID-19 and the development of GPA. This case report discusses a 16-year-old East Asian boy who developed GPA with diffuse alveolar hemorrhage after contracting COVID-19. Additionally, a literature review was conducted to gain a deeper understanding of this disorder., Methods: The study involved a retrospective analysis of the data of a case of GPA post-COVID-19 infection, aiming to summarize the clinical characteristics of GPA post-COVID-19 infection through a search of databases (PubMed, Wanfang Data, and CNKI), supplemented by standard searches in Google Scholar, Cochrane, Scopus, and LitCovid, and to conduct a comprehensive analysis of the literature., Results: A total of 12 cases were identified and, when combined with the present case, yielded 13 cases of GPA post-COVID-19 infection, comprising 5 males and 8 females with an average age of (40.6 ± 19.5) years. The interval between COVID-19 infection and the diagnosis of GPA varied from 1 day to 3 months across all cases. Mortality was reported at 7.7% (1/13). The most common clinical manifestations included cough (69.2%) and dyspnea (46.1%). Computed tomography scans revealed ground-glass opacities and multifocal pulmonary nodules. In all cases, positive findings for c-ANCA and protease 3-antibody were observed. Renal involvement was observed in more than half of the patients., (© 2024 The Authors. Immunity, Inflammation and Disease published by John Wiley & Sons Ltd.)

A 78-year-old woman with a history of intractable otitis media presented with a fever, hearing impairment, thigh pain, and a skin rash. She had renal dysfunction, positive myeloperoxidase-antineutrophil cytoplasmic autoantibody, otitis media, and multiple nodules in both lungs. She was diagnosed with granulomatosis with polyangiitis, crescentic glomerulonephritis, and interstitial nephritis, which was confirmed in a kidney biopsy specimen. Induction therapy with rituximab and avacopan without glucocorticoids promptly resolved her fever and thigh pain and improved her auditory acuity and nodule in the right lung. The patient experienced no adverse effects with rituximab or avacopan.

Objective: Granulomatosis with polyangiitis (GPA) is an ANCA-associated vasculitis. The 2022 ACR/EULAR-endorsed classification criteria for GPA was derived using data only from adult patients. We aimed to assess the performance of the ACR/EULAR classification criteria for GPA in paediatric patients and compare it with the EULAR/Pediatric Rheumatology International Trials Organization (PRINTO)/Pediatric Rheumatology European Society (PReS)-endorsed Ankara 2008 criteria for GPA., Methods: Retrospective data of paediatric patients with GPA in 20 centres from 9 countries were evaluated. The diagnosis of GPA was made according to the expert opinion. The sensitivity, specificity, positive predictive value, and negative predictive value of the criteria sets were evaluated., Results: The study included 77 patients with GPA and 108 controls [IgA vasculitis (n = 44), Takayasu's arteritis (n = 20), microscopic polyangiitis (n = 16), polyarteritis nodosa (n = 14), Behçet's disease (n = 12), eosinophilic granulomatosis with polyangiitis (n = 1) and Cogan's syndrome (n = 1)] with a median age of 17.8 and 15.2 years, respectively. Among patients with GPA, constitutional symptoms (85.7%) and ENT involvement (79.2%) were the most common presentations. In the GPA group, 73 patients fulfilled the Ankara 2008 criteria and 69 the ACR/EULAR classification criteria. Sensitivities of the Ankara 2008 criteria and the ACR/EULAR classification criteria were 94.8% and 89.6%, while specificities were 95.3% and 96.3%, respectively. No significant difference was found between sensitivities and specificities of both classification criteria (P = 0.229 and P = 0.733, respectively)., Conclusion: In children, both the ACR/EULAR and EULAR/PRINTO/PReS Ankara 2008 classification criteria for GPA perform well and similarly., (© Crown copyright 2023.)

Competing Interests: The author declares no conflicts of interest.

Competing Interests: Disclosures Dr Welsh and Dr Schuldt have no conflicts of interest to report.

Granulomatosis with polyangiitis (GPA) is a rare, small-vessel vasculitis that often presents with upper and lower respiratory tract symptoms along with nonspecific symptoms, including fever and weight loss. Although it is more likely to present in adulthood, GPA can present at all ages and affect multiple organ systems, making its recognition exceedingly difficult and leading to delayed diagnosis. This is particularly deleterious in the pediatric population in which delayed diagnosis and treatment are associated with a significantly worse prognosis, significant hematologic complications, and irreversible organ damage. We discuss a 14-year-old boy with GPA who presented with noncardiac chest pain and polyarthralgias in whom a detailed review of systems later revealed a 2-year history of fevers, malaise, and failure to thrive. Our report reveals the importance of early recognition and treatment of GPA during childhood. [ABSTRACT FROM AUTHOR]

The authors discuss the article "Reclassification of Previously Diagnosed GPA Patients Using the 2022 ACR/EULAR Classification Criteria" by Jung Yoon Pyo et al. Topics include purpose of the Diagnostic and Classification Criteria in Vasculitis (DCVAS) study, reason that classification criteria for diagnosis should be avoided, and the American College of Rheumatology/European Alliance of Associations for Rheumatology ACR/EULAR classification criteria for granulomatosis with polyangiitis (GPA).

Objective To describe disease presentation and long-term outcome of granulomatosis with polyangiitis (GPA) patients according to blood eosinophils count (Eos) at vasculitis diagnosis. Methods Data from newly diagnosed GPA patients registered in the French Vasculitis Study Group database with available eosinophil count at diagnosis were reviewed. Disease characteristics, rate and type of relapses, and overall survival were analysed according to Eos, categorized as normal (<500/mm3), mild-to-moderate hypereosinophilia (HE) (between 500 and 1500/mm3) and severe HE (>1500/mm3). Results Three hundred and fifty-four patients were included. At diagnosis, 90 (25.4%) patients had HE ≥500/mm3; they were more likely male (73% vs 56%, P  = 0.006) and had more frequent cutaneous manifestations (49% vs 33%, P  = 0.01), peripheral neuropathy (32% vs 17%, P  = 0.004) and higher BVAS (21 vs 18, P  = 0.01), compared with those with Eos <500/mm3. Patients with severe HE (n  = 28; median Eos 2355, range 1500–9114) had more frequent renal function worsening at presentation (P  = 0.008). After a median follow-up of 3.95 (interquartile range 1.95–6.76) years, no difference was found in overall relapse rates according to baseline Eos, but those with HE experienced more neurological (P  = 0.013) and skin (P  = 0.024) relapses and had more frequently peripheral neuropathy as damage at last follow-up (P  = 0.02). Overall survival was not significantly different in patients with normal Eos or HE at diagnosis. (P  = 0.08). Conclusions Blood HE at diagnosis, observed in about one-quarter of GPA patients, identifies a subgroup of patients with a more severe disease and higher rate of skin and neurological involvement both at presentation and during follow-up. [ABSTRACT FROM AUTHOR]

Fibroinflammatory lesions of the sinonasal tract include inflammatory polyps (chronic rhinosinusitis), various infectious, sarcoidosis, and NK/T-cell lymphoma as examples of the most commonly encountered lesions. However, the differential diagnosis includes several less frequently encountered entities, such as granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis (Churg-Strauss), eosinophilic angiocentric fibrosis considered part of IgG 4 -related disease, and Rosai-Dorfman disease. This review focuses on these latter entities providing an update on clinical, laboratory, imaging, histology, and ancillary testing employed to reach an actionable diagnosis., Competing Interests: Disclosure The author declares that he has no conflict of interest as it relates to this review. This review article was performed in accordance with ethical standards and did not require informed consent. Consent to participate or for publication was waived as it is a review article without any uniquely identifying personal data. The opinions or assertions contained herein are the private views of the author., (Published by Elsevier Inc.)

Background: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare form of autoimmune vasculitis. The involvement of IgG4 and HBsAg in EGPA is less common but can occur and may present unique challenges in management., Case Presentation: We present a case study of a 70-year-old female diagnosed with EGPA confirmed via renal biopsy. She initially presented with recurrent purpura, diarrhea and progressive numbness in the hands and feet, accompanied by general weakness. Complete remission was achieved with a one-year course of prednisone acetate and cyclophosphamide treatment. However, upon discontinuation of self-medication, the disease relapsed, manifesting as a generalized rash and weakness in the extremities.Skin biopsy revealed eosinophil infiltration, with inflammatory cells predominantly surrounding blood vessels. Notably, during treatment, the patient's hepatitis B markers transitioned from negative to positive for HBsAg. Subsequent administration of entecavir, along with monitoring for a decrease in HBV DNA levels, preceded the initiation of steroids and rituximab to attain remission once more. Among the remaining 15 patients analyzed, all exhibited elevated serum IgG4 levels, with none testing positive for hepatitis B. Notably, only one patient was diagnosed with immunoglobulin G4-related disease (IgG4-RD), suggesting that elevated IgG4 levels alone may not necessarily indicate IgG4-RD., Conclusions: Our case report highlights the first instance of recurrent EGPA accompanied by elevated IgG4 and positivity for hepatitis B, which was successfully treated with rituximab. In cases of concurrent hepatitis B, rituximab treatment may be considered once viral replication is under control. However, emphasis on maintenance therapy is crucial following the induction of disease remission.

Background: Granulomatosis with polyangiitis is a systemic inflammatory disease characterized by necrotizing vasculitis that affects small- and medium-sized blood vessels. Granulomatous inflammation affects the lungs, ears, nose, and throat, and commonly affects the kidneys, although the retroperitoneal tissue is rarely affected. Several studies have reported an increased risk of venous thromboembolism. Early diagnosis and treatment are of vital importance due to the rapid progression of the disease.Case Presentation: We present the case of a 66-year-old Moroccan man followed for bilateral jugular thrombosis. Cavitary pulmonary nodules and retroperitoneal fibrosis with thrombosis involving several vascular territories were detected on thoracoabdominopelvic computerized tomography scan. Laboratory analyses revealed that the patient was positive for cytoplasmic antineutrophilic antibodies. The diagnosis of granulomatosis with polyangiitis was retained. Treatment with glucocorticoids and immunosuppressive agents resulted in significant clinical and radiological improvement over the following months.Conclusions: We describe the diagnostic steps and the difficulty of managing this patient. Rare manifestations, such as retroperitoneal fibrosis, have been reported in the literature in association with granulomatosis with polyangiitis, and should not delay the diagnosis and treatment of granulomatosis with polyangiitis owing to its severity. [ABSTRACT FROM AUTHOR]

Wegener' granulomatosis is an autoimmune diseases, often involving the lung and kidney, has a high mortality rate in nontreatment patients. The low incidence and nonspecific features, often lead to misdiagnosis and delayed treatment. This paper reported the diagnosis and treatment of a 55-year-old female patient with primary Wegener' granuloma of the lung diagnosed by percutaneous lung biopsy of pulmonary nodules, and reviews the relevant literature. [ABSTRACT FROM AUTHOR]

Purpose: This study investigates the accuracy of either computerized tomography (CT) or magnetic resonance imaging (MRI) for the evaluation of various orbital diseases., Methods: We collected 126 CT scans and 65 MRI scans from 144 subjects and asked two radiologists to interpret the images without clinical information. Images included 14 with a clinical diagnosis of orbital infection, 144 with orbital inflammation, and 33 with orbital neoplasm. The inflammatory diseases included thyroid eye disease (TED, n = 69), non-specific orbital inflammation (NSOI, n = 44), IgG4-related disease (IgG4-RD, n = 15), sarcoidosis (Sarcoid, n = 9), granulomatosis with polyangiitis (GPA, n = 5), and Erdheim-Chester disease (ECD, n = 2)., Results: The balanced accuracy (BA) for the two radiologists ranged from 0.87 to 0.90 for cellulitis, 0.81 to 0.86 for inflammation, and 0.82 to 0.85 for neoplasm. Radiologists were excellent at recognizing GPA (BA = 0.98 to 0.99) and very good for TED (BA = 0.80 to 0.86). They also did well identifying IgG4-RD (BA = 0.75 to 0.77), but slightly less well for NSOI (BA = 0.69 to 0.75) and poorly for Sarcoid (BA = 0.48 to 0.50)., Conclusions: CT or MRI scanning contributes to the evaluation of patients with orbital disease, but accuracy does varies based depending on the diagnosis. We could not evaluate issues such as determination of disease activity, variability based on the unit used for imaging or the skills beyond those of our two specialized neuroradiologists. Future studies should directly compare the two imaging modalities and assess the utility of imaging to determine disease activity., Competing Interests: At the time that this study was conceived and data collected, James T. Rosenbaum was a consultant to Janssen, Abbvie, Roche, UpToDate, Novartis, Gilead, UCB, Santen, Horizon, Corvus, Celldex and Eyevensys, and had received research funding from Alcon Research Institute and Pfizer. He is now employed by Corvus Pharmaceuticals and is no longer receiving research support or serving as a consultant. Min Joung Lee is a consultant of Thyroscope. Bobby S. Korn is a consultant to Amgen, Viridian, Immunovant, and has received Royalties from Elsevier. Don O. Kikkawa is a consultant to Amgen (Horizon), Immunovant, Lassen, Thyroscope, and has received Royalties from Elsevier. The following authors have no financial disclosures: Rohan Verma, Bronwyn E. Hamilton, David Pettersson, Dongseok Choi, and Eun Soo Kim.” This does not alter our adherence to PLOS ONE policies on sharing data and materials., (Copyright: © 2024 Lee et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.)

Competing Interests: Competing interests: None declared.

Background: Granulomatosis with polyangiitis (Wegener's granulomatosis) causes progressive nasal collapse, nasal obstruction, and central face deformity. It is not known whether cartilaginous nasal reconstruction should be performed immediately or delayed until after disease "burnout.", Objectives: The aims of this research regarding nasal collapse due to Wegener's granulomatosis were to (1) assess the functional and aesthetic outcomes following immediate vs delayed nasal reconstruction; and (2) measure the impact of psychosocial well-being (anxiety, depression, social isolation) in immediate vs delayed nasal reconstruction., Methods: Wegener's patients with either immediate or delayed nasal surgery (n = 61) were compared. Functional and aesthetic severity were compared with the validated Standard Cosmesis and Health Nasal Outcome Survey (SCHNOS) score (t test). In addition, Patient-Reported Outcomes Measurement Information System (PROMIS) perioperative and 1-year follow-up surveys were analyzed., Results: At initial consultation, SCHNOS score severity types were similar for both groups (immediate vs delayed): mild 15% vs 15%; moderate 59% vs 60%, and severe 26% vs 25%. Over a 30 ± 4 month period, delayed surgery patients' conditions deteriorated, with a shift from mild to more severe SCHNOS scores, from 25% severe at initial consultation to 85% before surgery. PROMIS scores at presentation were high compared to the general public; by the time of delayed surgery, patients had significantly worsened: anxiety from 28 to 73; depression from 18 to 62; and social isolation from 20 to 80. Although both immediate and delayed groups improved after surgery in functional and psychosocial scores, the immediate surgery group's improvement was superior., Conclusions: Data showed superior functional and aesthetic scores and superior psychosocial indicators with immediate cartilaginous nasal reconstruction, compared with waiting until disease burnout to undergo surgery., (© The Author(s) 2024. Published by Oxford University Press on behalf of The Aesthetic Society. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com.)

This case report describes a 40-year-old male patient with severe cardiac failure due to eosinophilic granulomatosis with polyangiitis (EGPA) and myocarditis. The fast diagnostic approach with cardiac MRI (CMR) and immunosuppressive treatment with glucocorticoid and cyclophosphamide near-normalized the patient's cardiac function. Myocarditis due to EGPA is rare, however life-threatening, so a systematic approach and early CMR should be considered in patients with known asthma presenting with eosinophilia and cardiac involvement., (Published under Open Access CC-BY-NC-BD 4.0. https://creativecommons.org/licenses/by-nc-nd/4.0/.)

Granulomatosis with polyangiitis (GPA) is a rare autoimmune disease with multi-system involvement. It involves the upper respiratory tract, lungs and kidneys. A 36-year-old female patient presented to a tertiary care referral hospital in Central India in 2023 with complaints of low-grade fever, dry cough and loss of appetite initially followed by dyspnoea, purpuric skin lesions, right lower limb swelling with pain and redness. Her chest radiograph revealed right upper lobe cavitary lesion with consolidation in the right lower lobe. Mycobacterium tuberculosis was detected in sputum and broncho alveolar lavage via cartridge based nucleic acid amplification assay. Later, computed tomography pulmonary angiography revealed bilateral pulmonary artery thromboembolism. Furthermore, her cytoplasmic-antineutrophil cytoplasmic antibody test was positive, serum creatinine was rising, urine microscopy had red cell casts and lower limb venous doppler revealed deep venous thrombosis. Histopathological examination of the skin lesion revealed vasculitis. Based on these findings, the patient was diagnosed with GPA. The patient improved with pulse steroids, cyclophosphamide, anticoagulants and anti-tuberculous therapy., (© Copyright 2024, Sultan Qaboos University Medical Journal, All Rights Reserved.)

Background: Antineutrophil cytoplasmic antibody-associated vasculitides (AAV) is a group of systemic necrotizing small vessel autoimmune diseases, with microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA) being the two most common. The co-existence of AAV with different immune-mediated diseases (autoimmune disesases - AID) might affect the clinical presentation of the primary disease. The purpose of the study was to assess the co-existence of AAV with AID and to investigate whether it affects the characteristics and the course of AAV., Methods: A retrospective single-center study was performed to identify patients with a diagnosis of MPA or GPA and concomitant AID, and to investigate their clinical features and characteristics. The group consisted of consecutive unselected AAV patients treated at a large university-based hospital, since 1988 with follow-up until 2022., Results: Among 284 patients diagnosed either with GPA (232) or MPA (52), 40 (14,1%) had co-existing AIDs. The most frequent were: Hashimoto thyroiditis (16 cases), rheumatoid arthritis (8 cases), followed by psoriasis (6 cases), pernicious anemia (3 cases), and alopecia (3 cases). Patients with autoimmune comorbidities had a significantly longer time between the onset of symptoms and the diagnosis (26 vs. 11 months, p < 0.001). Laryngeal involvement (20.0% vs. 9.0%, p = 0,05), peripheral nervous system disorders (35.0% vs. 13.9%, p < 0.001), and neoplasms (20.0% vs. 8.6%, p = 0,044) were more common in patients with AID comorbidities, compared to subjects without AID. In contrast, renal involvement (45.0% vs. 70.9%, p = 0.001) and nodular lung lesions (27.5% vs. 47.5%, p = 0.044) were significantly less frequent in patients with co-morbidities. Following EUVAS criteria, patients with autoimmune co-morbidities had a generalized form of the disease without organ involvement (52.5% vs. 27.2%, p = 0.007), while the others had a higher percentage of generalized form with organ involvement (38.3% vs. 20.0%, p = 0.007)., Conclusions: The coexistence of AAV with different autoimmune diseases is not common, but it might affect the clinical course of the disease. Polyautoimmunity prolonged the time to diagnosis, but the AAV course seemed to be milder. Particular attention should be paid to the increased risk of cancer in these patients. It also seems reasonable that AAV patients should receive a serological screening to exclude the development of overlapping diseases., (© 2024. The Author(s).)