Renal Solid Mass as a Rare Presentation of Wagener's Granulomatosis: A Case Report

Sichani, Mehrdad Mohammadi; Hadi, Mazaher; Talebi, Ardeshir; Khalighinejad, Pooyan

doi:10.1155/2012/793014

Cited by 5 publications

(4 citation statements)

References 6 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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“…1,2 Renal mass is a rare presentation of GPA and its clinical and radiologic manifestations are similar to a renal abscess, tumor, or an inflammatory process. 3 Maguire et al reported one patient had renal mass out of 31 patients presenting with GPA. 4 Fig.…”

Section: Discussionmentioning

confidence: 98%

“…Renal mass is a rare presentation of GPA and its clinical and radiologic manifestations are similar to a renal abscess, tumor, or an inﬂammatory process. 3…”

Section: Discussionmentioning

confidence: 99%

Granulomatosis with Polyangiitis Presenting as a Renal Mass: A Scarce Case Report with a Review of the Literature

Kumar¹,

Barwal²,

Sharma³

et al. 2023

Ann Natl Acad Med Sci

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Wegener granulomatosis (WG) now known as granulomatosis with polyangiitis (GPA) is an uncommon autoimmune disorder of undivulged etiology affecting the respiratory tract including paranasal sinuses, nasal cavity, lungs, and kidneys predominantly. GPA presenting as a solitary renal mass is rarely seen. We present a case report of a 27-year-old female presenting with a right renal mass along with pain, low-grade fever, and arthralgia. Computed tomography scan of the abdomen revealed a hypodense low attenuated renal mass with indistinct margins. Ultrasound-guided biopsy revealed features typical of GPA. She was started on oral steroids (prednisolone 40 mg) and azathioprine. She developed pain, vomiting, and diarrhea after starting treatment with azathioprine. Azathioprine was stopped and rituximab 1 g weekly was started for 4 weeks followed by 500 mg 6 monthly injections. She got symptomatic relief at 4 weeks with a diminution of renal mass at 6 months follow-up. We report this rare entity of WG presenting as renal mass. Suspecting and diagnosing renal mass as a part of GPA prevented us from undertaking unnecessary surgical treatment in this patient. Medical treatment with steroids and rituximab is effective in inducing remission and maintenance.

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“…In 15 cases reported so far, one case had progressed to ESRD as he refused the treatment, one case was lost to follow up after the initial presentation and returned with advanced renal failure, and one case expired due to massive pulmonary hemorrhage before starting the treatment. [ 14 18 11 ] Even though, in cases where only the kidney is involved initially, without immunosuppression disease may progress and can involve other systems. In a report by smith et al ., patient underwent total nephrectomy in view of renal mass, which was consistent with granulomas without any evidence of vasculitis.…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2021

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Wegener's granulomatosis or granulomatosis with polyangiitis (GPA) is multisystemic vasculitis. Kidney involvement in GPA often presents with rapidly progressive renal failure and requires urgent treatment. A 60-year-old female presented with prolonged history of fever, generalized weakness, decreased appetite, and weight loss over 4 months. Her renal function was normal; urine culture was sterile. On further evaluation, she was found to have large, hypodense solid lesion in mid pole of the right kidney on CECT. CT guided renal biopsy was done, which showed granulomatous interstitial nephritis with focal crescents. On further evaluation, she was found to have high titers of anti-MPO antibody. She was started on steroid and methotrexate with subsidence of fever. Follow-up after 12 months showed resolution of the lesion. GPA solely presenting as solid mass like lesion in the kidney is extremely rare presentation. Early diagnosis and prompt initiation of the treatment can prevent the progression of the disease.

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Renal Solid Mass as a Rare Presentation of Wagener's Granulomatosis: A Case Report

Cited by 5 publications

References 6 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

Granulomatosis with Polyangiitis Presenting as a Renal Mass: A Scarce Case Report with a Review of the Literature

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

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