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Introduction: Eosinophilic granulomatosis with polyangiitis (EGPA) was formerly known as Churg-Strauss syndrome. The condition is characterized by disseminated necrotizing vasculitis with extravascular granulomas associated with hypereosinophilia. The vasculitides affect small vessels and are associated with antineutrophil cytoplasmic antibodies (ANCAs) detectable in the blood. Distinguishing between type 2-mediated chronic airway inflammation such as chronic rhinosinusitis with nasal polyps (CRSwNP) without vasculitis can be clinically challenging and should be considered., Materials and Methods: Immunological background, diagnosis, and therapy of EGPA were identified through literature searches in Medline, PubMed, as well as national and international studies (ClinicalTrials.gov) and the Cochrane Library. Human studies published up to and including 10/2023 on the topic were considered., Results: In cases of deteriorating general health with previously known eosinophilic inflammation of the upper and lower airways, EGPA and its interdisciplinary investigation should be considered. Various types of eosinophilic inflammation and syndromes must be considered differentially., Conclusion: Characterization of mucosal airway inflammation through biomarker determination is meaningful and occasionally makes the difference for targeted therapy., Competing Interests: J. Hagemann received grants from GSK, Sanofi, HAL Allergy, Novartis Pharma GmbH for lectures and consultations, outside the submitted work. M. Laudien received funds and/or fees from Olympus Deutschland GmbH, Olympus Europa SE & CO. KG, Novartis Pharma GmbH, Sanofi-Aventis Deutschland GmbH, Brainlab Sales GmbH, CSL Vifor, GlaxoSmithKline GmbH & Co. KG, Medtronic, Fiagon AG Medical Technologies, AEDA. He is board member of John Grube Foundation and chair of AG Rhinologie/Rhinochirurgie DGHNO. M. Cuevas has received honoraria from AstraZeneca, GSK, Sanofi, and Novartis, outside the submitted work. L. Klimek has received research grants and/or lecture fees from Allergy Therapeutics/ Bencard, UK/Germany; ALK-Abelló, Denmark; Allergopharma, Germany; ASIT Biotech, Belgium; AstraZeneca, Sweden, Bionorica, Germany; Biomay, Austria, Boehringer Ingelheim, Germany, Circassia, USA; Stallergene, France; Cytos, Switzerland; Curalogic, Denmark; HAL, Netherlands; Hartington, Spain; Lofarma, Italy; MEDA/Mylan, Sweden/USA; Novartis, Switzerland, Leti, Spain; ROXALL, Germany; GlaxoSmithKline (GSK), UK; Sanofi, France outside the submitted work and/or acted in an advisory capacity for the above-mentioned pharmaceutical companies. L. Klimek is editor of Allergo Journal and Allergo Journal International and he is the current President of AeDA (Ärzteverband Deutscher Allergologen) and Vice President of EAACI. R. Kianfar and F. Klimek report no conflicts of interest in connection with the present work. Table 1.Diseases involving the respiratory tract and hypereosinophilia [1]. Adult-onset eCRSwNPEGPAN-ERDAdult-onset asthmaABPAPrevalence (asthma/CRS patients)3 – 10%0.6%5 – 10%21 – 48%2.5%M : W2 : 11 : 21 : 22 : 31 : 1Appearance at the age of...30 – 505635 – 3630 – 4625 – 55   under 12RareRareRaren.a.Rare   over 60RarePossibleRarePossiblePossibleClinical peculiarityNasal polyps, loss of smellSystemic vasculitis, granulomatosisSalicylate intoleranceRapid decrease in LuFuMucus plugs (bronchial tubes)Biomarkers   Blood-Eo↑↑↑↑↑↑↑↑↑↑   Serum IgE (IU/mL)Variable100 – 300VariableVariable1,000 – 10,000   FeNO↑↑↑↑↑   Serum periostin↑↑→↑↑↑↑   U LTE4↑↑↑↑↑↑   EETs/EETosis++Lumen/tissuePossible++Lumen/tissue++Lumen   Charcot-Leyden crystals++Lumen/tissuePossible++Lumen/tissue++Lumen   OtherCST-1, eotaxin-3, IgG4ANCA, eotaxin-3, lösIL-2RMast cell/ tile markerReversibility in LuFu for diagnosisResponse to biologicsanti-IgE+++++anti-IL-5(R)+++++++++anti-IL-4R++++++anti-TSLP(+)++eCRSwNP = eosinophilic chronic rhinosinusitis with nasal polyps; EGPA = eosinophilic polyangiitis with granulomatosis; N-ERD = non-steroidal-exacerbated respiratory disease; ABPA = allergic bronchopulmonary aspergillosis; LuFu = lung function test; FeNO = fractional expiratory NO; leukotriene E4 = LTE4; EETs/EETosis = extracellular eosinophil traps; ANCA = antineutrophil cytoplasmic antibody. Table 2.The following criteria are only to be applied in the case of histological evidence of small vessel vasculitis. Clinical criteriaObstructive airway disease+3Nasal polyps / CRSwNP+3Mononeuritis multiplex+1Laboratory and biopsy criteriaBlood eosinophilia of >1,000 cells/µL or 1 × 109/L (blood)+5Extravascular, predominantly eosinophilic inflammation (biopsy)+2cANCA + / anti-PR3 + (blood)–3Hematuria–1A score of > 6 makes EGPA likely. CRSwNP = chronic rhinosinusitis with nasal polyps; cANCA = antineutrophil cytoplasmic antibody.., (© Dustri-Verlag Dr. K. Feistle.)

Goodpasture's syndrome (GS) is a rare and organ-specific autoimmune disease that is mediated by anti-glomerular basement membrane (anti-GBM) antibodies and has pathology characterized by crescentic glomerulonephritis with linear immunofluorescent staining for IgG on the GBM. It typically presents as acute renal failure caused by a rapidly progressive glomerulonephritis, accompanied by pulmonary hemorrhage that may be life-threatening. It was first described as a distinctive syndrome by Pasture in 1919. Autoimmune Inner Ear Disease (AIED) may be associated. The etiology of GS is unknown. Researchers hypothesized a genetic predisposition HLA-associated. Complex immunological mechanisms are in the pathogenesis. The disease is caused by autoantibodies against the NC1 domain of the alpha 3 chain of type IV collagen. The limited presence of this molecule in the body explains the interest confined to specific target organs, such as the lung and kidney. It occurs when the immune system attacks the walls of the lungs and the tiny filtering units in the kidneys. Without prompt diagnosis and treatment, the disease can lead to bleeding in the lungs, kidney failure, and even death. [ABSTRACT FROM AUTHOR]

Systemic lupus erythematosus (SLE) and small-sized vessel vasculitis are usually two distinguishable autoimmune diseases. However, a vasculitis may be found in the course SLE but rarely corresponds to an ANCA-associated vasculitis (AAV). We report four cases of de novo SLE associated with AAV, our aim being to discuss the clinical significance of this association. We included four patients fulfilling the criteria for both SLE and AAV and followed in two different university hospitals between 1996 and 2009. In light of a 20-year literature review (25 described clinical cases), we discussed the etiopathogeny of such an association. All patients presented a severe renal involvement (creatininemia ranging from 120 to 370 μmol/l) and thrombopenia (ranging from 45,000 to 137,000 platelets/mm). The other main clinical symptoms were arthritis ( n = 3), serositis ( n = 2) and intra-alveolar hemorrhage ( n = 2). An inflammatory syndrome was noticed at diagnosis in all cases. ANCAs were MPO-ANCAs in all cases. Two out of these four patients were also diagnosed with antiphospholipid syndrome. The frequency of this association seems not fortuitous. Although the etiopathogenic mechanisms of such an association remain to be more precisely described, several clinical, histological and immunological features support the hypothesis of the existence of a SLE-AAV overlapping syndrome. Moreover, clinicians must be aware of such an overlapping syndrome, notably because its initial presentation can be very severe. [ABSTRACT FROM AUTHOR]