Splenic infarction in a patient with Wegener's granulomatosis

Roy, Debasish; George, Ajith John; Chattopadhyay, C.; Grennan, D M

doi:10.1093/rheumatology/38.11.1162

Cited by 9 publications

(4 citation statements)

References 4 publications

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“…The spleen is particularly predisposed to infarction due to occlusive infarction of distal parenchymal splenic arteries and arterioles, which are end-vessels that lack collaterals [11]. Clinically apparent splenic infarction appears to be a rarer phenomenon with few reported cases, but typically presenting with left sided abdominal pain [16, 17]. In contrast, in the case reported here the patient reported flank and back pain as the predominant presenting complaint, which probably represented symptomatic renal and splenic wedge infarction and was initially misdiagnosed as a urinary tract infection.…”

Section: Discussionmentioning

confidence: 99%

PR3 vasculitis presenting with symptomatic splenic and renal infarction: a case report and literature review

2019

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Background ANCA-associated vasculitis is a life-threatening, systemic autoimmune disease. There is an increased risk of organ infarction but in many cases this is asymptomatic. We described here the first reported case of PR3 vasculitis presenting with symptomatic bilateral renal wedge infarction. Case presentation A 19-year old Caucasian woman with no past medical history presented on a number of occasions over a number of weeks with progressively more severe back pain, fevers and arthralgia. On the final presentation she was noted to have developed splinter haemorrhages and her blood tests revealed impaired renal function along with elevated inflammatory markers. She was subsequently found to have high titres of serum PR3 antibodies and focal necrotising glomerulonephritis on renal biopsy, consistent with a diagnosis of PR3 ANCA-associated vasculitis. Cross-sectional imaging revealed multiple wedge infarcts of her spleen and both kidneys, confirmed on contrast-enhanced ultrasound. Large vessel, cardiac and thrombophilic causes of thromboembolism were excluded. She was treated with high-dose corticosteroids and CD20 monoclonal antibodies (rituximab) and at time of writing, 4 months after initial presentation, has entered clinical remission. Conclusions Here we describe the first reported case of PR3 vasculitis presenting with symptomatic renal wedge infarction. In patients with vasculitis who present with flank or back pain, infarction of abdominal organs should be considered in the differential. Both splenic and renal infarctions are likely underdiagnosed in the setting of ANCA-associated vasculitis but may have clinical impact in contributing to infection risk and the degree or renal recovery, respectively.

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Section: Discussionmentioning

confidence: 99%

PR3 vasculitis presenting with symptomatic splenic and renal infarction: a case report and literature review

2019

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“…However, isolated case reports of patients with WG and incomplete septal cirrhosis, 184 primary biliary cirrhosis, 185 and portal inflammatory granuloma, vasculitis, and fibrosis 186 have been published. Splenic involvement is rarely diagnosed antemortem, [187][188][189][190] but splenic infarcts have been documented at necropsies 191,192 or on abdominal CT (low attenuation lesions with peripheral enhancement). 187,193 OTHER ORGAN INVOLVEMENT Constitutional symptoms (e.g., malaise, fatigue, fever, weight loss) occur in 30 to 80% of patients with WG and may be the presenting features.…”

Section: Gastrointestinal Involvementmentioning

confidence: 99%

Wegener's Granulomatosis: Evolving Concepts in Treatment

Lynch

White

Tazelaar

et al. 2004

Seminars in Respiratory and Critical Care Medicine

171

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Wegener's granulomatosis (WG), the most common of the pulmonary granulomatous vasculitides, typically involves the upper respiratory tract, lower respiratory tract (bronchi and lung), and kidney, with varying degrees of disseminated vasculitis. Major histological features include a necrotizing vasculitis involving small vessels, extensive "geographic" necrosis, and granulomatous inflammation. Clinical manifestations of WG are protean; virtually any organ can be involved. Further, the spectrum and severity of the disease is heterogeneous, ranging from indolent disease involving only one site to fulminant, multiorgan vasculitis leading to death. The pathogenesis of WG has not been elucidated, but both cellular and humoral components are involved. Circulating antineutrophil cytoplasmic antibodies (cANCA) likely play a role in the pathogenesis and often correlate with activity of the disease. Treatment strategies are evolving. Cyclophosphamide (CYC) plus corticosteroids (CS) is the mainstay of therapy for generalized, multisystemic WG. Historically, the combination of CYC plus CS was used for a minimum of 12 months, but concern about late toxicities associated with CYC has led to novel treatment approaches. Currently, short-course (3-6 months) induction treatment with CYC plus CS, followed by maintenance therapy with less toxic agents (e.g., methotrexate, azathioprine) is recommended. Further, recent studies suggest that methotrexate combined with CS may be adequate for limited, non-life threatening WG. The role of other immunomodulatory agents (including trimethoprim-sulfamethoxazole) is also explored.

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“…Some cases of splenic infarction in GPA have been reported, [8][9][10] although splenic rupture is uncommon. Splenic infarction can be asymptomatic in some cases, unlike in the present case.…”

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confidence: 99%

“…Unless there are signs of imminent rupture of the spleen or bleeding, a conservative approach with aggressive immunosuppressive treatments appears to be justified. 8,9 It is also important to note that severe splenic bleeding caused by GPA-related vasculitis has been reported in some patients receiving antithrombotic treatment. 11 The clinical lesson to be learnt from our case is that comprehensive evaluation at the time of diagnosis and early recognition of the presentation of abdominal lesions, including those in the spleen, can contribute to appropriate and timely management of this potentially fatal complication.…”

mentioning

confidence: 99%