Spontaneous splenic rupture complicating granulomatosis with polyangiitis (Wegener's granulomatosis)

Nagasu, Akiko; Sasae, Yumi; Hirano, Hajime; Nishimura, Hirotake; Aoyama, Yuko; Urakami, Atsushi; Morita, Yoshitaka

doi:10.1111/1756-185x.12151

Cited by 1 publication

(5 citation statements)

References 11 publications

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“…In three of those cases [reported by Hawley et al (1996), Franssen et al (1993), andMcCain et al (2002)], the initial sign of a GPA was a splenic rupture. This contrasts with the other study conducted by Nagasu et al (2013), where splenic rapture occurred following the diagnosis of GPA and during corticosteroid treatment. In our case, the splenic rupture occurred before diagnosis and treatment.…”

Section: Discussioncontrasting

confidence: 85%

“…In our case, the splenic rupture occurred before diagnosis and treatment. The mechanisms of splenic rupture in three reports were different [ 6 , 7 , 8 ]; two showed no signs of vasculitis or infarction but rather a neutrophilic infiltration at the rupture site [ 6 , 8 ]. Another study exhibited severe necrosis but no histological signs of vasculitis [ 7 ].…”

Section: Discussionmentioning

confidence: 99%

“…The mechanisms of splenic rupture in three reports were different [ 6 , 7 , 8 ]; two showed no signs of vasculitis or infarction but rather a neutrophilic infiltration at the rupture site [ 6 , 8 ]. Another study exhibited severe necrosis but no histological signs of vasculitis [ 7 ]. However, our case is similar to the Hawley case [ 5 ], where a biopsy showed necrosis and central arteritis in the resected spleen.…”

Section: Discussionmentioning

confidence: 99%

“…Spontaneous splenic rupture is an extremely rare complication of systemic vasculitis, and there have been only four reported cases of spontaneous splenic rupture in GPA patients worldwide [5,6,7,8]. In three of those cases [reported by Hawley et al (1996), Franssen et al (1993), andMcCain et al (2002)], the initial sign of a GPA was a splenic rupture.…”

Section: Discussionmentioning

confidence: 99%

“…Vasculitis, fibrinoid blood vessel alterations, necrotizing granulomatous inflammation, and infarction are some of the pathologic splenic changes linked to GPA [4]. Spontaneous splenic rupture can develop as a consequence of GPA; however, it is exceedingly rare, and only four such cases have been published in the literature [5][6][7][8]. Due to its rarity, early and accurate detection coupled with vigorous therapy is necessary to slow the progression of the disease.…”

Section: Introductionmentioning

confidence: 99%

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A Rare Case of Granulomatosis With Polyangiitis Complicated by Splenic Rupture

Awad,

Srour,

Mekdad

et al. 2024

Cureus

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Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis (WG), is a condition marked by necrotizing vasculitis of the small-medium vessels that results in necrotizing granulomatous inflammation. Splenic involvement in GPA is a potentially life-threatening consequence of connective tissue disease and is rarely described as the main presenting feature. We present a case of a patient with perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA) who experienced spontaneous splenic rupture. A CT scan of the abdomen, an ANCA test, and a splenic biopsy were employed to identify ANCA-associated vasculitis (AAV) splenic rupture. Our patient's splenic rupture could be attributed to GPA. Nonetheless, since it may alter patient follow-up and therapy, a patient with spontaneous splenic rupture without an obvious explanation should be promptly evaluated for connective-tissue disease. This report highlights the intricacy and unpredictability of the clinical symptoms linked to AAV, as well as the possibility of misinterpreting them.

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