Granulomatosis with polyangiitis masquerading as renal mass: Case report and literature review

Tiwari, V.; Raman, Abhishek; Gupta, Anurag; Gupta, Parikshaa; Joshi, Ameeta; Bhargava, Vinant; Malik, Manish; Gupta, Ashwani; Bhalla, Anil Kumar; Rana, Devinder Singh

doi:10.4103/ijn.ijn_325_20

Cited by 4 publications

(4 citation statements)

References 19 publications

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“…Finally, data were extracted from 41 articles, resulting in a total of 42 GPA patients presenting with renal masses: 23 (54.8%) were men and the median age was 45.3 (29.8–59.8) years [ 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 45 , 46 , 47 , 48 , 49 , 50 ].…”

Section: Resultsmentioning

confidence: 99%

Cutting-Edge Strategies for Renal Tumour-like Lesions in Granulomatosis with Polyangiitis: A Systematic Review

Iorio,

Pizzi,

Cecchin

et al. 2024

Diagnostics

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Background: Granulomatosis with polyangiitis (GPA) is characterised by granulomatous inflammation and small-to-medium vessel necrotising vasculitis, mainly affecting respiratory tract and kidneys. Renal involvement presenting as tumour-like lesions poses diagnostic and treatment challenges. Methods: Following the observation of a GPA patient presenting with multiple renal tumour-like lesions, we conducted a systematic literature review on MEDLINE/PubMed, EMBASE, and Cochrane databases. Data gathered from the literature were analysed to summarise the diagnostic approach, management, and outcome of renal GPA-related tumour-like lesions. Results: a 49-year-old female presented with persistent constitutional symptoms and multiple bilateral renal lesions. Renal biopsy showed chronic interstitial inflammation with necrotising granulomas. Laboratory tests disclosed positive anti-proteinase 3 (PR3) anti-neutrophil cytoplasmic antibody (ANCA) leading to a final diagnosis of GPA. She was effectively treated with high-dose glucocorticoids and rituximab. Literature search yielded 41 articles, concerning 42 GPA patients with renal masses, presenting bilaterally in 23.8% of the cases. Positive PR3-ANCA was observed in 86.5% of the cases. Half of 42 patients showed kidney abnormalities. Treatment with glucocorticoids (83.3%) and immunosuppressive agents (80.9%) resulted in an overall good remission rate and favourable prognosis. Conclusions: GPA should be considered in the differential diagnoses of kidney tumour-like lesions. The diagnosis is challenging, and histological examination greatly contributes to the diagnostic work-up.

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Section: Resultsmentioning

confidence: 99%

Cutting-Edge Strategies for Renal Tumour-like Lesions in Granulomatosis with Polyangiitis: A Systematic Review

Iorio,

Pizzi,

Cecchin

et al. 2024

Diagnostics

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“…8 In the present report, even our patient was presented with typical symptoms of GPA involving ear, nose, and throat as reported in previous case reports and reviews. 1,3,6,[8][9][10] However, establishing the disease initially based on typical symptoms as GPA was a challenge without a proper diagnostic workup. An event of tests was performed one after the other as per patient symptoms.…”

Section: Discussionmentioning

confidence: 99%

“…2 Its systematic vasculitides involves various organs such as the sinuses, upper respiratory tract, the lungs, and the kidneys. 3 The prevalence of GPA ranges from 2.5 to 146 cases/per million, with an incidence of 0.5 to ~12 cases/per million person-years. 4 Approximately ~90% of patients with GPA present with ear, nose, and throat involvement with manifestations such as purulent/bloody nasal discharge, persistent rhinorrhea, otorrhea, earache, polychondritis, etc.…”

Section: Introductionmentioning

confidence: 99%

Diagnostic dilemma of Wegener’s granulomatosis and its effective management: A rare case report and update

Modi

Devi

2022

Chest Dis Rep

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Granulomatosis with Polyangiitis (GPA) is an uncommon condition belongs to the group of ANCA-associated necrotizing vasculitides. It is characterized by necrotizing granulomatosis of upper and lower respiratory system with coexisting glomerulonephritis. Its signs and symptoms are largely varied due to a wide spectrum of involvement sites. In the present case report, we describe a case of 26-year-old female presented to our centre with history of bilateral ear discharge, earache, and dry cough with a deviated mouth angle to left. Diagnostic work up was done and patient was treated with pulse methylprednisolone, plasmapheresis, and rituximab. Timely diagnosis and prompt initiation of the treatment has prevented the GPA progression and helped the patient to improve quickly. Currently, the patient is doing well and is in remission.

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“…The incidence of thoracic involvement in GPA is up to 75% [41], and the lung lesions may be multiple or rarely solitary [42]. Renal involvement may be absent at presentation [2,42].…”

Section: Clinical Findingsmentioning

confidence: 99%

Noninfectious Granulomatous Lung Disease: Radiological Findings and Differential Diagnosis

Lassandro,

Picchi,

Corvino

et al. 2024

JPM

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Granulomatous lung diseases (GLDs) are a heterogeneous group of pathological entities that can have different clinical presentations and outcomes. Granulomas are histologically defined as focal aggregations of activated macrophages, Langerhans cells, and lymphocytes, and may form in the lungs when the immune system cannot eliminate a foreign antigen and attempts to barricade it. The diagnosis includes clinical evaluation, laboratory testing, and radiological imaging, which especially consists of high-resolution computed tomography. bronchoalveolar lavage, transbronchial needle aspiration or cryobiopsy, positron emission tomography, while genetic evaluation can improve the diagnostic accuracy. Differential diagnosis is challenging due to the numerous different imaging appearances with which GLDs may manifest. Indeed, GLDs include both infectious and noninfectious, and necrotizing and non-necrotizing granulomatous diseases and the imaging appearance of some GLDs may mimic malignancy, leading to confirmatory biopsy. The purposes of our review are to report the different noninfectious granulomatous entities and to show their various imaging features to help radiologists recognize them properly and make an accurate differential diagnosis.

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Granulomatosis with polyangiitis masquerading as renal mass: Case report and literature review

Cited by 4 publications

References 19 publications

Cutting-Edge Strategies for Renal Tumour-like Lesions in Granulomatosis with Polyangiitis: A Systematic Review

Cutting-Edge Strategies for Renal Tumour-like Lesions in Granulomatosis with Polyangiitis: A Systematic Review

Diagnostic dilemma of Wegener’s granulomatosis and its effective management: A rare case report and update

Noninfectious Granulomatous Lung Disease: Radiological Findings and Differential Diagnosis

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