Uncommon presentations of primary systemic necrotizing vasculitides: the Great Masquerades

Sharma, Aman; Gopalakrishan, Dharmesh; Nada, Ritambhra; Kumar, Susheel; Dogra, Sunil; Aggarwal, Mayank; Gupta, Rajesh; Minz, Ranjana W.; Kakkar, Nandita; Vashishtha, Rakesh K; Singh, Surjit

doi:10.1111/1756-185x.12223

Cited by 21 publications

(8 citation statements)

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“…One patient had a relapse in cardiac dysfunction which responded to reinduction with intravenous methylprednisolosne and intravenous pulses of cyclophosphamide. Four patients also developed deep venous thrombosis (DVT) and one patient had chronic Budd‐Chiari syndrome at presentation . This patient had thrombosis of the hepatic and portal veins and was positive for antiphospholipid antibodies.…”

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confidence: 99%

Polyarteritis nodosa in north India: clinical manifestations and outcomes

et al. 2016

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Section: Resultsmentioning

confidence: 99%

Polyarteritis nodosa in north India: clinical manifestations and outcomes

et al. 2016

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“… 10 Renal infarction is rare and often asymptomatic in ANCA-associated vasculitis; however, adequate attention should be paid to this possible diagnosis because of the huge impact of renal cortex infarction on renal function. 11 , 12 The prognosis may be worse in patients with bilateral infarctions. 13 Information on the use of anticoagulant and/or antiplatelet therapy in ANCA-associated vasculitis is lacking, because of potential hemorrhagic complications.…”

Section: Discussionmentioning

confidence: 99%

Multiple renal infarctions in a patient caused by granulomatosis with polyangiitis

Yuan

2020

J Int Med Res

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Granulomatosis with polyangiitis (GPA) is a small-vessel vasculitis that is highly associated with anti-neutrophil cytoplasmic antibodies. GPA carries an increased risk of organ infarction, but renal infarction is rare. We herein describe a case of multiple renal infarctions caused by GPA. A 66-year-old man presented with hearing loss, nasal discharge, fatigue, and weight loss for several months. Cross-sectional contrast-enhanced computed tomography images revealed multiple low-attenuation areas in both kidneys. He subsequently developed fever and impaired renal function. Blood serum was positive for cytoplasmic anti-neutrophil cytoplasmic antibody and a renal biopsy showed granulomatous necrotizing vasculitis. He was diagnosed with GPA and treated with high-dose corticosteroids, plasma exchange, and cyclophosphamide. The patient ultimately entered clinical remission.

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“…Other less commonly involved organs were skin (25%‐30%), heart (0%‐6%) and gastrointestinal tract (8%‐12%) . Uncommon presentations like tumefactive subcutaneous mass over the thigh and prostate involvement have also been reported . Table shows the comparison of clinical features of GPA reported from different geographical locations.…”

Section: Clinical Featuresmentioning

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Is granulomatosis with polyangiitis in Asia different from the West?

et al. 2018

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The incidence and clinical features of antineutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) have been shown to vary according to geographical areas, with granulomatosis with polyangiitis (GPA) being more common in northern Europe and microscopic polyangiitis (MPA) being more common in Asian countries.The annual incidence of GPA among Asians varies between 0.37/million to 2.1/million population. The prevalence of GPA has been estimated to be 1.94/100 000 in a Chinese population. Polymorphisms in class II major histocompatibility genes and ETS1 proto-oncogene has been shown in Asian patients with GPA. There is a difference in mean age at onset and proteinase 3 (PR3) or myeloperoxidase (MPO) positivity in GPA patients from different Asian countries. Those from India had mean age of 40 years and those from Japan had mean age of 65 years. Sixty percent of GPA patients from China and Japan were MPO ANCA positive while the majority of patients from India and Korea were PR3 positive. Geographical variation with lower frequency of renal involvement in Indian studies and higher frequency in Chinese patients has also been noted. Treatment outcomes have been similar to those reported from other parts of the world. Remission was achieved in about two-thirds of patients while relapses were noted in one-third to half of the patients. Apart from minor differences in the organ systems involved, MPO-ANCA GPA and PR3-ANCA GPA had similar rates of remission and relapses. K E Y W O R D S Asia, granulomatosis with polyangiitis, vasculitisWe searched PubMed using the search terms "Granulomatosis with polyangiitis", "GPA" and "Wegener's granulomatosis". We screened for articles published from Asian countries and describing the epidemiology, pathogenesis, clinical features and outcomes of GPA patients. Case reports and small case series of less than 5 patients were not included. The basis of diagnosis of GPA in different studies was either histopathology suggestive of GPA or fulfilling the American College of Rheumatology (ACR) criteria for GPA or classified according to European Medicines Evaluation Agency classification. We also included large case series on GPA from Europe and USA to compare them with Asian studies. | EPID EMIOLOGYThe incidence and prevalence of GPA shows a regional and ethnical difference with higher incidence of GPA in northern Europe and lower incidence in Asian countries. 1 The precise estimates of incidence and prevalence of AAV and GPA are not available from Asian countries. In the epidemiological study by Fujimoto et al, 1 the annual incidence of GPA in Japan was calculated to be 2.1/million population, which was much lower than that noted in the UK (annual incidence of 14.3/million). The annual incidence of MPA was higher in Japan than the UK and the overall incidence of all AAV was similar between Japan and the UK. Liu et al 7 used DIS-MOD II software to estimate prevalence based on the inpatient data of GPA patients in 100 hospitals across China. They estimated the prevalence of GPA to be 1...

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Uncommon presentations of primary systemic necrotizing vasculitides: the Great Masquerades

Abstract: Systemic necrotizing vasculitides may present with uncommon manifestations and a high index of suspicion is required for early diagnosis and prompt treatment to prevent adverse outcomes.

Cited by 21 publications

References 66 publications

Polyarteritis nodosa in north India: clinical manifestations and outcomes

Polyarteritis nodosa in north India: clinical manifestations and outcomes

Multiple renal infarctions in a patient caused by granulomatosis with polyangiitis

Is granulomatosis with polyangiitis in Asia different from the West?

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