Vasculitis mimics

Molloy, Eamonn; Langford, Carol A.

doi:10.1097/bor.0b013e3282f1dcf2

Cited by 67 publications

(30 citation statements)

References 30 publications

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“…Ruling out the differentials or complications of therapy, including infections or malignancy, is mandatory during the entire disease course. Some drugs can induce (propylthiouracil is the most famous one) and/or mimic (levamisole-cocaine) ANCA-associated vasculitides (30)(31)(32)(33). Most routinely used methods for ANCA testing include indirect immunofluorescence (to detect c-ANCA with a cytoplasmic labeling pattern, p-ANCA with a perinuclear pattern, and sometimes x-ANCA for an atypical pattern) and enzyme-linked immunosorbent assay (ELISA; to detect PR3-and/or MPO-ANCA; non-routine ELISA tests can also detect ANCA with other specificities such as anti-elastase or anti-cathepsin G).…”

Section: Clinical and Biological Findings Diagnosismentioning

confidence: 99%

Updates in ANCA-associated vasculitis

2016

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Antineutrophil cytoplasm antibody (ANCA)-associated vasculitides are small-vessel vasculitides that include granulomatosis with polyangiitis (formerly Wegener's granulomatosis), microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome). Renal-limited ANCA-associated vasculitides can be considered the fourth entity. Despite their rarity and still unknown cause(s), research pertaining to ANCA-associated vasculitides has been very active over the past decades. The pathogenic role of antimyeloperoxidase ANCA (MPO-ANCA) has been supported using several animal models, but that of antiproteinase 3 ANCA (PR3-ANCA) has not been as strongly demonstrated. Moreover, some MPO-ANCA subsets, which are directed against a few specific MPO epitopes, have recently been found to be better associated with disease activity, but a different method than the one presently used in routine detection is required to detect them. B cells possibly play a major role in the pathogenesis because they produce ANCAs, as well as neutrophil abnormalities and imbalances in different T-cell subtypes [T helper (Th)1, Th2, Th17, regulatory cluster of differentiation (CD)4+ CD25+ forkhead box P3 (FoxP3)+ T cells] and/or cytokine-chemokine networks. The alternative complement pathway is also involved, and its blockade has been shown to prevent renal disease in an MPO-ANCA murine model. Other recent studies suggested strongest genetic associations by ANCA type rather than by clinical diagnosis. The induction treatment for severe granulomatosis with polyangiitis and microscopic polyangiitis is relatively well codified but does not (yet) really differ by precise diagnosis or ANCA type. It comprises glucocorticoids combined with another immunosuppressant, cyclophosphamide or rituximab. The choice between the two immunosuppressants must consider the comorbidities, past exposure to cyclophosphamide for relapsers, plans for pregnancy, and also the cost of rituximab. Once remission is achieved, maintenance strategy following cyclophosphamide-based induction relies on less toxic agents such as azathioprine or methotrexate. The optimal maintenance strategy following rituximab-based induction therapy remains to be determined. Preliminary results on rituximab for maintenance therapy appear promising. Efforts are still under way to determine the optimal duration of maintenance therapy, ideally tailored according to the characteristics of each patient and the previous treatment received.Keywords: Antineutrophil cytoplasmic antibody-associated vasculitis, granulomatosis with polyangiitis (Wegener's granulomatosis), microscopic polyangiitis, Churg-Strauss syndrome (eosinophilic granulomatosis with polyangiitis), cyclophosphamide, rituximab IntroductionAntineutrophil cytoplasm antibody (ANCA)-associated vasculitides are small-vessel vasculitides that include granulomatosis with polyangiitis (GPA; formerly Wegener's granulomatosis), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA; al...

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Section: Clinical and Biological Findings Diagnosismentioning

confidence: 99%

Updates in ANCA-associated vasculitis

2016

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“…The differential diagnosis of PAN is broad and includes systemic rheumatic diseases, such as systemic lupus erythematosus (SLE), atherosclerotic disease, infections and malignancies 11 12. While it is beyond the scope of this case report to provide a comprehensive list of all possible alternative diagnoses, we need to mention that our patient did not meet the criteria for other diagnoses such as SLE, an infectious or malignant process.…”

Section: Discussionmentioning

confidence: 97%

A case of isolated renal involvement of polyarteritis nodosa successfully treated with steroid monotherapy

Pourafshar¹,

Sobel

Segal

2016

BMJ Case Reports

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Polyarteritis nodosa (PAN) is a systemic necrotising vasculitis that affects medium-sized and small-sized arteries. The spectrum of disease ranges from involving a single organ to polyvisceral failure. We report a case of a 75-year-old male presented with solely hypertension and mild renal function impairment as a consequence of diffuse renal necrotising vasculitis with occlusive but non-stenotic lesions associated with PAN. The overall prognosis of PAN has been improved in recent decades, primarily reflecting early diagnosis and more effective treatments. Therefore, early diagnosis is critical and it warrants full investigations even in those patients without obvious multiorgan manifestations. In those instances, with mild disease, steroid monotherapy has been shown to be effective with excellent response. Our patient responded well to steroid monotherapy and we were able to gauge his response by improvement in his blood pressure.

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“…First, systemic symptoms may provide clues for the diagnosis of CNS angiitis. Secondary CNS angiitis related to systemic vasculitis usually presents with constitutional symptoms,5 and can therefore be diagnosed by careful history-taking and physical examination (e.g., oral and genital ulcers for Behçet's disease, and rash and photosensitivity for systemic lupus erythematosus). However, CNS angiitis presents with a variable clinical spectrum, and the constitutional symptoms lack sensitivity and specificity.…”

Section: Discussionmentioning

confidence: 99%

Role of High-Resolution Magnetic Resonance Imaging in the Diagnosis of Primary Angiitis of the Central Nervous System

et al. 2014

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BackgroundPrimary angiitis of the central nervous system (PACNS) is a rare disorder and is often difficult to diagnose due to the lack of a confirmatory test. PACNS can generally be diagnosed based on typical angiographic findings. We describe herein a patient diagnosed with PACNS despite the presence of normal findings on conventional angiography.Case ReportA 44-year-old man with a recent history of ischemic stroke in the right posterior cerebral artery territory developed acute-onset vertigo. Diffusion-weighted imaging revealed an acute infarction within the left posterior inferior cerebellar artery. His medical history was unremarkable except for hyperlipidemia; the initial examination revealed mild gait imbalance. During the 10 days of hospital admission, the patient experienced four recurrent ischemic strokes within the posterior circulation territory (occipital lobe, pons, and cerebellum). He was diagnosed with recurrent cerebral infarctions due to PACNS. The basilar artery exhibited no demonstrable luminal stenosis, but there were direct imaging signs of central nervous system angiitis including wall thickening and contrast enhancement. High-dose intravenous steroid therapy followed by oral prednisolone was administered. There was no further stroke recurrence and follow-up imaging of the arterial walls showed normalization of their characteristics.ConclusionsThe present case emphasizes the importance of wall imaging in the diagnosis and treatment of PACNS.

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Vasculitis mimics

Cited by 67 publications

References 30 publications

Updates in ANCA-associated vasculitis

Updates in ANCA-associated vasculitis

A case of isolated renal involvement of polyarteritis nodosa successfully treated with steroid monotherapy

Role of High-Resolution Magnetic Resonance Imaging in the Diagnosis of Primary Angiitis of the Central Nervous System

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