Otitis media with effusion as an initial manifestation of granulomatosis with polyangiitis

Granulomatosis with polyangiitis is a systemic vasculitis of unknown etiology, characterized by necrotizing granulomas. It is an autoimmune disease affecting small- and medium-sized vessels of upper and lower respiratory tract, kidneys, and other organs. We described a case of a patient with otitis media with effusion as the first manifestations of granulomatosis with polyangiitis. A 54-year-old female presented as an urgent case with history of a severe otalgia, hearing loss, vertigo, and fever. The patient was treated with diagnosis of otitis media with effusion and acute rhinosinusitis, but without significant success. She developed an acute kidney dysfunction as a sign of glomerulonephritis with rapidly progressive renal failure. Diagnosis of granulomatosis with polyangiitis was confirmed after the histopathological analysis of kidney tissue, not by analysis of middle ear and paranasal sinus mucosa specimens. The patient was treated according to generally accepted protocol, and over time, there was an almost complete recovery.

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Tension Pneumothorax as Initial Manifestation of Granulomatosis with Polyangiitis (GPA)

European Journal of Case Reports in Internal Medicine ◽

10.12890/2021_002304 ◽

2021 ◽

Author(s):

Rúben Reis ◽

Anneke Joosten ◽

Francelino Ferreira ◽

Magda Silva ◽

Catarina Parente ◽

...

Keyword(s):

Granulomatosis With Polyangiitis ◽

Kidney Biopsy ◽

Systemic Vasculitis ◽

Systemic Disease ◽

Tension Pneumothorax ◽

Upper Airway ◽

Antineutrophil Cytoplasmic Antibodies ◽

Lower Airway ◽

Initial Manifestation ◽

Pulmonary Cavity

Granulomatosis with polyangiitis (Wegener’s granulomatosis) is a systemic vasculitis that primarily affects small and medium vessels. Its manifestations are usually confined to the upper airway, lower airway and kidney. It can also affect other organs and systems, although this is unusual. We describe the case of a 67-year-old woman who presented with a tension pneumothorax due to rupture of a pulmonary cavity. This pulmonary cavity proved to be secondary to systemic disease which also caused a tumour in her kidney. Biopsy showed non-necrotizing granulomatosis, and even though antineutrophil cytoplasmic antibodies (ANCA) were negative, the diagnosis of granulomatosis with polyangiitis was made.

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GRANULOMATOSE EOSINOPHILIQUE AVEC POLYANGEITE: PRESENTATIONS INHABITUELLES. EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS: UNUSUAL PRESENTATIONS

International Journal of Advanced Research ◽

10.21474/ijar01/13667 ◽

2021 ◽

Vol 9 (10) ◽

pp. 1199-1203

Author(s):

Fadwa Mekouar ◽

◽

Naoual Elomri ◽

Mohamed Jira ◽

Naoufal Assoufi ◽

...

Keyword(s):

Granulomatosis With Polyangiitis ◽

Systemic Vasculitis ◽

Pulmonary Involvement ◽

Military Hospital ◽

Unknown Etiology ◽

Eosinophilic Granulomatosis With Polyangiitis ◽

Peripheral Eosinophilia ◽

Medicine Department ◽

The Third ◽

Eosinophilic Granulomatosis

Introduction:Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis of unknown etiology, affecting small and medium-sized vessels and associating asthma,pulmonary involvement, peripheral eosinophilia and extravascular eosinophilic granulomas. We report three GEPA sightings of unusual revelations. Methods:These are three cases of GEPA from the internal medicine department B, Mohammed V military hospital in Rabat. Results:Three patients aged between 27 and 70 years. The most frequently found manifestations were pulmonary, cutaneous, neurological and digestive. All patients presented with peripheral eosinophilia. The ANCA were negative. All patients were treated with corticosteroids in one case, immunosuppressive therapy were used. Conclusion:In this article, GEPA was revealed by an acute surgical abdomen (appendicitis) in one case, a febrile rash in the second and liver damage in the third patient. Our patients responded well to corticosteroid therapy but the treatment must be adapted depending on the case.

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Granulomatous Inflammation in ANCA-Associated Vasculitis

International Journal of Molecular Sciences ◽

10.3390/ijms22126474 ◽

2021 ◽

Vol 22 (12) ◽

pp. 6474

Author(s):

Antje Müller ◽

Bettina Krause ◽

Anja Kerstein-Stähle ◽

Sara Comdühr ◽

Sebastian Klapa ◽

...

Keyword(s):

Respiratory Tract ◽

Granulomatosis With Polyangiitis ◽

Local Development ◽

Systemic Vasculitis ◽

Kidney Tissue ◽

Granulomatous Inflammation ◽

Giant Cells ◽

Eosinophilic Granulomatosis With Polyangiitis ◽

Factors Affecting ◽

Anca Associated Vasculitis

ANCA-associated vasculitis (AAV) comprises granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). While systemic vasculitis is a hallmark of all AAV, GPA is characterized by extravascular granulomatous inflammation, preferentially affecting the respiratory tract. The mechanisms underlying the emergence of neutrophilic microabscesses; the appearance of multinucleated giant cells; and subsequent granuloma formation, finally leading to scarred or destroyed tissue in GPA, are still incompletely understood. This review summarizes findings describing the presence and function of molecules and cells contributing to granulomatous inflammation in the respiratory tract and to renal inflammation observed in GPA. In addition, factors affecting or promoting the development of granulomatous inflammation such as microbial infections, the nasal microbiome, and the release of damage-associated molecular patterns (DAMP) are discussed. Further, on the basis of numerous results, we argue that, in situ, various ways of exposure linked with a high number of infiltrating proteinase 3 (PR3)- and myeloperoxidase (MPO)-expressing leukocytes lower the threshold for the presentation of an altered PR3 and possibly also of MPO, provoking the local development of ANCA autoimmune responses, aided by the formation of ectopic lymphoid structures. Although extravascular granulomatous inflammation is unique to GPA, similar molecular and cellular patterns can be found in both the respiratory tract and kidney tissue of GPA and MPA patients; for example, the antimicrobial peptide LL37, CD163+ macrophages, or regulatory T cells. Therefore, we postulate that granulomatous inflammation in GPA or PR3-AAV is intertwined with autoimmune and destructive mechanisms also seen at other sites.

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A Case Series of Granulomatosis With Polyangiitis Primarily Diagnosed by Otological Manifestations

Annals of Otology Rhinology & Laryngology ◽

10.1177/0003489418815517 ◽

2018 ◽

Vol 128 (3) ◽

pp. 263-266 ◽

Cited By ~ 1

Author(s):

Ronald Sahyouni ◽

Omid Moshtaghi ◽

Mehdi Abouzari ◽

Phuonganh Le ◽

Jack Birkenbeuel ◽

...

Keyword(s):

Hearing Loss ◽

Otitis Media ◽

Granulomatosis With Polyangiitis ◽

Otitis Media With Effusion ◽

False Negative ◽

Previous History ◽

Acute Otitis Media ◽

Case Series ◽

Repeated Testing ◽

Mixed Hearing Loss

Objective: To describe a case series of previously undiagnosed granulomatosis with polyangiitis (GPA) patients who presented primarily with otological manifestations. Method: We report a series of patients visited at a neurotology clinic who were eventually diagnosed with GPA based on their otologic complaints and had no prior knowledge of having this condition. Results: In this series, 10 (91%) patients presented with hearing loss (HL), more than half of which were bilateral (60%). Upon audiometric examination, all but 1 patient had mixed, conductive, or sensorineural HL. All patients presented with eustachian tube dysfunction (ETD), otitis media with effusion (OME), or both. Nasal endoscopy showed intranasal pathology in 3 (27%) patients. Otologic symptoms were improved in all patients after treatment with an average of 4 in-office follow-up appointments. Conclusion: GPA should be included in the differential diagnosis of adults with unexplained mixed hearing loss, new onset serous effusion, or acute otitis media in the absence of a previous history of ETD. Laboratory tests (ie, anti-neutrophil cytoplasmic autoantibody, erythrocyte sedimentation rate, and C-reactive protein) along with a urinalysis can aid in screening these patients. In cases in which the index of suspicion is high, repeated testing could reduce the risk of false negative findings.

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Polyps, grommets and eosinophilic granulomatosis with polyangiitis

The Journal of Laryngology & Otology ◽

10.1017/s0022215117002444 ◽

2018 ◽

Vol 132 (3) ◽

pp. 236-239 ◽

Cited By ~ 2

Author(s):

F G Kavanagh ◽

W Hasan ◽

D A Smyth ◽

J E Fenton

Keyword(s):

Observational Study ◽

Diagnostic Criteria ◽

Otitis Media ◽

Nasal Polyps ◽

Nasal Polyposis ◽

Granulomatosis With Polyangiitis ◽

Otitis Media With Effusion ◽

Eosinophilic Granulomatosis With Polyangiitis ◽

The Mean ◽

Eosinophilic Granulomatosis

AbstractObjective:To explore the link between nasal polyposis, refractory otitis media with effusion and eosinophilic granulomatosis with polyangiitis.Methods:A retrospective observational study was carried out of patients diagnosed with refractory otitis media with effusion necessitating grommet insertion and who had nasal polyps. Patients were evaluated to determine if they fulfilled the diagnostic criteria of eosinophilic granulomatosis with polyangiitis.Results:Sixteen patients (10 males and 6 females) were identified. The mean age of grommet insertion was 45.4 years. The mean number of grommets inserted per patient was 1.6. The mean number of nasal polypectomies was 1.7. All 16 patients had paranasal sinus abnormalities and otitis media with effusion, 14 had asthma, 9 had serological eosinophilia and 7 had extravascular eosinophilia. Nine patients met the diagnostic criteria for eosinophilic granulomatosis with polyangiitis.Conclusion:The co-presence of nasal polyps and resistant otitis media with effusion should raise the possibility of eosinophilic granulomatosis with polyangiitis.

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Effects of surgery and topical medication on eosinophilic granulomatosis with polyangiitis with otitis media and sinusitis: a case report

Journal of International Medical Research ◽

10.1177/0300060520920049 ◽

2020 ◽

Vol 48 (4) ◽

pp. 030006052092004

Author(s):

Zijing Wang ◽

Lin Han ◽

Lisheng Yu

Keyword(s):

Otitis Media ◽

Granulomatosis With Polyangiitis ◽

Otitis Media With Effusion ◽

Granulomatous Inflammation ◽

Sinus Surgery ◽

High Dose ◽

Eosinophilic Granulomatosis With Polyangiitis ◽

Strauss Syndrome ◽

Ear Symptoms ◽

Eosinophilic Granulomatosis

Eosinophilic granulomatosis with polyangiitis (EGPA), also known as Churg–Strauss syndrome, is eosinophil-rich, necrotizing granulomatous inflammation often involving the respiratory tract. Furthermore, EGPA is necrotizing vasculitis that predominantly affects small to medium vessels and is associated with asthma and eosinophilia. Most patients with EGPA have sinusitis and some complain of hearing loss and refractory otitis media with effusion. Systemic use of immunosuppressants and glucocorticoids is currently recommended, despite the inevitable associated side effects. However, systemic treatment is not always effective for nasal and ear symptoms. We report a case of EGPA with refractory otitis media and chronic sinusitis, which were resistant to systemic high-dose steroids and immunosuppressants. However, these symptoms responded well to functional endoscopic sinus surgery and myringotomy and grommet insertion. We also administered budesonide nasal irrigation and glucocorticoid injection by intratympanic and postaural methods in this patient. The therapeutic effect was satisfactory.

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GRANULOMATOSIS WITH POLYANGIITIS (WEGENER): FEATURES OF DIAGNOSIS IN CONDITIONS OF COVID-19 INFECTION (CLINICAL CASE)

Journal of the Grodno State Medical University ◽

10.25298/2221-8785-2021-19-3-331-334 ◽

2021 ◽

Vol 19 (3) ◽

pp. 331-334

Author(s):

N. V. Hlutkina ◽

Keyword(s):

Respiratory Failure ◽

Otitis Media ◽

Clinical Case ◽

Granulomatosis With Polyangiitis ◽

Systemic Vasculitis ◽

Organ Damage ◽

Multiple Organ ◽

State Of Health ◽

Moderate Severity ◽

The Given

Background. Multivascular and multiple organ damage in COVID-19 infection poses difficulties in the diagnosis of comorbidities, especially vasculitis. Aim. The goal is to analyze a clinical case of diagnosing granulomatosis with polyangiitis (Wegener) in conditions of COVID-19 infection. Material and methods. An example of the difficulty of diagnosing granulomatosis with polyangiitis (Wegener) in conditions of COVID-19 infection is given. Results. Within three months, the patient received treatment for sinusitis, otitis media. However, against the background of the antibacterial therapy, the state of health did not improve. On the fourth month from the onset of the disease, the patient was admitted to the pulmonology department with a diagnosis of a viral infection caused by SARS Cov-2 (IgM +, IgG-), moderate severity, respiratory failure 0 ?, after the examination, the diagnosis was made: granulomatosis with polyangiitis (Wegener's), acute course, high activity associated with antibodies to Pr-3 (79 n/ml), advanced stage with lesions of ENT organs in the form of exudative otitis media, bilateral mastoiditis. The damage to the lungs in the form of endobronchitis, pulmonary disseminated form (partly with disintegration). respiratory failure 1. Reconvalescent of SARS Cov-2 infection. Conclusions. Based on the given example of this clinical case, the difficulty of diagnosing systemic vasculitis, namely granulomatosis with polyangitis (Wegener's), in conditions of COVID-19 infection is demonstrated.

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