Three atypical manifestations of granulomatosis with polyangiitis: lateral medullary syndrome, anterior cheek mass and melting scleritis of eye

Shenavandeh, Saeedeh; Petramfar, Peyman

doi:10.5114/reum.2017.68915

Cited by 8 publications

(8 citation statements)

References 16 publications

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“…Subcutaneous nodules associated with GPA have previously been reported in 1.1%-14.21% of patients with GPA ( 4 , 5 ). These nodules are reportedly located in the back, right armpit, and forehead of patients with GPA ( 5 ), whereas subcutaneous lesions in the anterior cheek have very rarely been reported ( 9 ). Shenavandeh et al reported the involvement of GPA in a patient who developed severe sinusitis, hearing loss, and a left cheek mass.…”

Section: Discussionmentioning

confidence: 99%

Subcutaneous Cheek Nodule Associated with Granulomatosis with Polyangiitis

et al. 2021

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We herein report an unusual case of granulomatosis with polyangiitis (GPA) in a 65-year-old man in whom relapsed disease manifested as an anterior cheek nodule. Magnetic resonance imaging indicated the differential diagnoses of the subcutaneous nodule in the patient's anterior cheek to be inflammatory granulomatous lesions with GPA, malignancy, or infectious disease. A histopathological examination ruled out malignancy and infectious diseases, and necrotizing vasculitis was suspected. The subcutaneous nodule was successfully treated using rituximab, suggesting that it was associated with GPA, secondary to vasculitis. Clinicians should be aware of the possibility of such a rare manifestation of GPA.

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Section: Discussionmentioning

confidence: 99%

Subcutaneous Cheek Nodule Associated with Granulomatosis with Polyangiitis

et al. 2021

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“…A total of 35.8% (34/95) of patients received just 1 cycle of RTX, leading to a positive response with disease remission in 84.3% (29/34), treatment failure in 14.7% (5/34), and disease relapse in 6.9% (2/29). The remaining 64.2% (61/95) of treated patients received between two to 15 cycles of RTX at intervals ranging from 1 to 6 months, with 18.0% (11/61) receiving one retreatment for disease relapse [48,61,68,70]. However, the rationale of designated treatment intervals and indications for retreatment in other reports were unclear.…”

Section: Uveitis Of Indeterminate Etiologymentioning

confidence: 99%

Rituximab for non-infectious Uveitis and Scleritis

Cunningham

2021

J Ophthal Inflamm Infect

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Purpose To provide a comprehensive review of rituximab use for the treatment of non-infectious uveitis and scleritis. Methods Review of literature through December 2020. Results Individual data was available for 229 patients with refractory non-infectious uveitis (n = 108) or scleritis (n = 121) who received treatment with rituximab (RTX). Rituximab was generally utilized as third-line or later treatment (uveitis: 67/90, 74.4%; scleritis: 90/96, 93.8%) at a mean of 33.5 months following the diagnosis of uveitis (range = 0 to 168.0 months; median = 24.0 months) and 39.4 months after diagnosis of scleritis (range = 1.0 to 168.0 months; median = 21.0 months). Patients with non-infectious uveitis and scleritis either received prior treatment with corticosteroids only (uveitis: 18/90, 20%; scleritis: 4/94, 4.3%), or with one (uveitis: 19/90, 21.1%; scleritis: 30/94, 31.9%), two (uveitis: 11/90, 12.2%; scleritis 27/94, 28.7%), or three or more (uveitis: 37/90, 41.1%; scleritis: 31/94, 33.0%) corticosteroid-sparing immunosuppressive agents with or without corticosteroids before initiation of RTX treatment. The rheumatologic protocol (two infusions of 1 gram of RTX separated by 14 days) was utilized most frequently (uveitis: 45/87, 51.7%; scleritis: 87/114, 76.3%), followed by the Foster protocol (eight weekly infusions of 375 mg/m2 RTX; uveitis: 18/87, 20.7%; scleritis: 10/114, 8.8%), and the oncologic protocol (four weekly infusions of 375 mg/m2 RTX; uveitis: 5/87, 5.7%; scleritis: 6/114, 5.3%). Various other off-label regimens were used infrequently (uveitis: 19/87, 21.8%; scleritis 11/114, 9.6%). Rituximab treatments resulted in a positive therapeutic response for the majority of patients with non-infectious uveitis (81/97, 83.5%). Commonly treated uveitic diagnoses included non-paraneoplastic autoimmune retinopathy (30/107, 28.0%), juvenile idiopathic arthritis (21/107, 19.6%), Vogt-Koyanagi-Harada disease (12/107, 11.2%), and Behçet disease (11/107, 10.3%). Cases of non-infectious scleritis were most commonly attributed to granulomatosis with polyangiitis (75/121, 62.0%) and rheumatoid arthritis (15/121, 12.4%), and showed an even greater rate of positive therapeutic response (112/120, 93.3%) following RTX treatment. No side effects were reported in 76.3% (74/97) of uveitis and 85.5% (71/83) scleritis cases. Of those cases associated with RTX-induced adverse events, the most common were infusion reactions of various severity (11/35, 31.4%). Conclusions Overall, RTX appeared to be both effective and well-tolerated as second or third-line therapy for patients with non-infectious uveitis and scleritis.

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“…Atypical lesions are often the presenting feature in GPA, including mass lesion. This manifestation may present as parapharyngeal mass, parotid mass, sinonasal and maxillary sinus lesions, and subglottic paratracheal mass [66][67][68][69] . Typically, masses are associated with PR3-ANCA and occur at early stage of the disease, usually as part of a systemic disease (lung and kidney).…”

Section: Tumor-like Manifestationsmentioning

confidence: 99%

Ear, nose, and throat in ANCA-associated vasculitis: a comprehensive review

Padoan¹,

Campaniello²,

Felicetti³

et al. 2021

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Ear, nose, and throat (ENT) involvement is a common feature in antineutrophil cytoplasmic antibody (ANCA)associated vasculitis (AAV), particularly in granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis. Over the last decade, substantial advancement has been made in understanding AAV pathogenesis, classification, and treatment. Typical ENT symptoms may include sinonasal, otologic, pharyngeal, and laryngeal manifestations. The otolaryngologic symptoms of AAV sometimes might be misdiagnosed in etiology as infectious or allergic. Thus, rapid recognition and early diagnosis of AAV as the cause of the symptoms prevent the risk of irreversible organ damage. The high impact of ENT symptoms on the quality of life of AAV patients confirms the importance of their early treatment through specific local and systemic approaches. Appropriate interdisciplinary management to early recognition of AAV and initiation of treatment may reduce morbidity in these patients. The purpose of this comprehensive review is to describe the clinical, histological, and radiological findings of ENT involvement in AAV and to update their surgical and therapeutic management, with a focus also on the role of a multidisciplinary team, involving the otorhinolaryngologist.

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Three atypical manifestations of granulomatosis with polyangiitis: lateral medullary syndrome, anterior cheek mass and melting scleritis of eye

Cited by 8 publications

References 16 publications

Subcutaneous Cheek Nodule Associated with Granulomatosis with Polyangiitis

Subcutaneous Cheek Nodule Associated with Granulomatosis with Polyangiitis

Rituximab for non-infectious Uveitis and Scleritis

Ear, nose, and throat in ANCA-associated vasculitis: a comprehensive review

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