Granulomatosis with polyangiitis presenting as a solitary renal mass: a case report.
Case report
Granulomatosis with polyangiitis
Inflammatory pseudotumor
Kidney
Solitary mass
Vasculitis
Journal
BMC nephrology
ISSN: 1471-2369
Titre abrégé: BMC Nephrol
Pays: England
ID NLM: 100967793
Informations de publication
Date de publication:
28 07 2023
28 07 2023
Historique:
received:
09
02
2022
accepted:
20
07
2023
medline:
31
7
2023
pubmed:
29
7
2023
entrez:
28
7
2023
Statut:
epublish
Résumé
Granulomatosis with polyangiitis (GPA) is characterized by necrotizing granulomatous vasculitis involving small-sized vessels in the upper airways, lower airways, and kidneys. Renal pathology is usually characterized by focal segmental necrotizing glomerulonephritis, which often leads to rapidly progressive renal failure. This type of renal involvement is usually unapparent on radiography. The presence of a renal mass in a patient with GPA, although extremely rare, is recognizable. Herein, we report a rare case of GPA presenting as a solitary renal mass and present a review of the literature. A 75-year-old woman presented with a solitary right kidney mass measuring 4 × 3.5 cm detected by enhanced computed tomography. There was no history of sinusitis, rhinitis, cough, or pneumonia suggestive of systemic GPA. Nephrectomy was performed based on the suspicion of renal cell carcinoma or malignant lymphoma. Three months later, she was admitted because her serum creatinine levels increased from 54.81 μmol/L to 405.76 μmol/L accompanied by a high C-reactive protein level of 159 mg/L. Anti-neutrophil cytoplasmic antibodies against myeloperoxidase and anti-proteinase 3 were negative. Histological examinations of the solitary renal mass revealed necrotizing granulomatous arteritis in the cortex and medullary vasa recta, surrounded by interstitial fibrosis, and focal segmental necrotizing glomerulonephritis in the localized lesion; however, signs of vasculitis were not observed in areas other than the solitary mass. Therefore, the patient was diagnosed with granulomatosis with polyangiitis (GPA). Despite treatment with prednisolone (30 mg/day), the patient developed oliguria with an elevation of her serum creatinine level to 583.44 μmol/L, which required hemodialysis within one month after the initiation of steroid therapy. The patient could successfully discontinue hemodialysis 21 months later, following a decrease in her serum creatinine level to 251.06 μmol/L. GPA should be considered as one of the differential diagnoses of a solitary renal mass. Furthermore, patients with solitary renal masses associated with GPA may exhibit a favorable response to steroid or immunosuppressive treatment.
Sections du résumé
BACKGROUND
Granulomatosis with polyangiitis (GPA) is characterized by necrotizing granulomatous vasculitis involving small-sized vessels in the upper airways, lower airways, and kidneys. Renal pathology is usually characterized by focal segmental necrotizing glomerulonephritis, which often leads to rapidly progressive renal failure. This type of renal involvement is usually unapparent on radiography. The presence of a renal mass in a patient with GPA, although extremely rare, is recognizable. Herein, we report a rare case of GPA presenting as a solitary renal mass and present a review of the literature.
CASE PRESENTATION
A 75-year-old woman presented with a solitary right kidney mass measuring 4 × 3.5 cm detected by enhanced computed tomography. There was no history of sinusitis, rhinitis, cough, or pneumonia suggestive of systemic GPA. Nephrectomy was performed based on the suspicion of renal cell carcinoma or malignant lymphoma. Three months later, she was admitted because her serum creatinine levels increased from 54.81 μmol/L to 405.76 μmol/L accompanied by a high C-reactive protein level of 159 mg/L. Anti-neutrophil cytoplasmic antibodies against myeloperoxidase and anti-proteinase 3 were negative. Histological examinations of the solitary renal mass revealed necrotizing granulomatous arteritis in the cortex and medullary vasa recta, surrounded by interstitial fibrosis, and focal segmental necrotizing glomerulonephritis in the localized lesion; however, signs of vasculitis were not observed in areas other than the solitary mass. Therefore, the patient was diagnosed with granulomatosis with polyangiitis (GPA). Despite treatment with prednisolone (30 mg/day), the patient developed oliguria with an elevation of her serum creatinine level to 583.44 μmol/L, which required hemodialysis within one month after the initiation of steroid therapy. The patient could successfully discontinue hemodialysis 21 months later, following a decrease in her serum creatinine level to 251.06 μmol/L.
CONCLUSIONS
GPA should be considered as one of the differential diagnoses of a solitary renal mass. Furthermore, patients with solitary renal masses associated with GPA may exhibit a favorable response to steroid or immunosuppressive treatment.
Identifiants
pubmed: 37507681
doi: 10.1186/s12882-023-03276-1
pii: 10.1186/s12882-023-03276-1
pmc: PMC10375652
doi:
Substances chimiques
Creatinine
AYI8EX34EU
Immunosuppressive Agents
0
Types de publication
Review
Case Reports
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
223Informations de copyright
© 2023. The Author(s).
Références
Urology. 2008 Mar;71(3):547.e1-2
pubmed: 18342212
Clin Exp Rheumatol. 2014 May-Jun;32(3 Suppl 82):S138
pubmed: 24021545
Urology. 2005 Apr;65(4):798
pubmed: 15833545
Arthritis Rheumatol. 2022 Mar;74(3):393-399
pubmed: 35106964
Am J Surg Pathol. 2014 Oct;38(10):1444-8
pubmed: 25025447
Arthritis Rheum. 2013 Jan;65(1):1-11
pubmed: 23045170
Pediatr Radiol. 2011 Sep;41(9):1212-5
pubmed: 21674284
Mod Rheumatol. 2019 Jan;29(1):20-30
pubmed: 30001655
Br J Urol. 1993 Dec;72(6):980-1
pubmed: 8306175
Radiol Case Rep. 2021 Jan 17;16(3):736-741
pubmed: 33510827
Arthritis Res Ther. 2015 Nov 02;17:305
pubmed: 26525413
Medicine (Baltimore). 2000 Nov;79(6):349-59
pubmed: 11144033
Case Rep Urol. 2012;2012:793014
pubmed: 22919535
Am J Med. 2002 Jan;112(1):82-3
pubmed: 11812418
Int J Clin Exp Pathol. 2017 Jul 01;10(7):7734-7742
pubmed: 31966620
J Rheumatol. 1999 Feb;26(2):457-8
pubmed: 9972987
Ann Rheum Dis. 2007 Feb;66(2):222-7
pubmed: 16901958
AJR Am J Roentgenol. 1978 Feb;130(2):233-8
pubmed: 414571
Rheumatol Int. 2009 Apr;29(6):679-83
pubmed: 18828022
Medicine (Baltimore). 2012 Mar;91(2):67-74
pubmed: 22391468
AJR Am J Roentgenol. 2000 Jun;174(6):1597-8
pubmed: 10845490
Clin Exp Nephrol. 2012 Aug;16(4):580-8
pubmed: 22350463
AJR Am J Roentgenol. 2001 Jan;176(1):116-8
pubmed: 11133548
Scand J Rheumatol. 1992;21(4):204-5
pubmed: 1529289
Am J Med. 2015 Oct;128(10):e15-8
pubmed: 26021813
Nephrol Dial Transplant. 2004 Apr;19(4):984-7
pubmed: 15031361
J Intern Med. 1998 Sep;244(3):209-16
pubmed: 9747743
Eur Radiol. 2000;10(8):1265-7
pubmed: 10939487
Rheumatology (Oxford). 2006 Dec;45(12):1554
pubmed: 17118941
Clin Exp Rheumatol. 2006 Sep-Oct;24(5):584-6
pubmed: 17181931
Arthritis Res Ther. 2014 Apr 23;16(2):R101
pubmed: 24758294
Nephron. 1994;68(4):500-4
pubmed: 7870236
Urology. 1986 Oct;28(4):307-9
pubmed: 3765241
Nephrology (Carlton). 2011 Sep;16(7):681-2
pubmed: 21884298
Indian J Nephrol. 2021 Jul-Aug;31(4):406-409
pubmed: 34584361