Diagnosing ANCA-associated vasculitis in ANCA positive patients

Houben, Eline; Bax, Willem A.; Dam, Bastiaan van; Slieker, W. A. T.; Verhave, Gideon; Frerichs, Fenneke C.P.; Eijk, I C van; Boersma, Wim; Kuyper, Guido T.M. de; Penne, E. Lars

doi:10.1097/md.0000000000005096

Cited by 56 publications

(24 citation statements)

References 34 publications

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“…Consider a diagnosis of AAV in the following clinical circumstances 4. Any of these features should prompt consideration of AAV; multiple features are strongly suggestive of AAV 5. Figures in parentheses are the approximate proportion of incident cases exhibiting each feature 56…”

Section: What Symptoms Do Patients Develop?mentioning

confidence: 99%

“…Rather, the key to diagnosis is prompt recognition of an inflammatory disease pattern when multiple symptoms emerge, especially if more than one organ system is implicated or in combination with chronic systemic symptoms. Therefore, when two or more of these symptoms develop concurrently, the index of suspicion should be high5 (box 1).…”

Section: How Is It Diagnosed?mentioning

confidence: 99%

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ANCA associated vasculitis

Hunter

Welsh

Farrah

et al. 2020

BMJ

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Section: What Symptoms Do Patients Develop?mentioning

confidence: 99%

Section: How Is It Diagnosed?mentioning

confidence: 99%

ANCA associated vasculitis

Hunter

Welsh

Farrah

et al. 2020

BMJ

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“…Despite the abovementioned trend of specificity for AAV, ANCAs can be positive in several other conditions (infections, CTD, inflammatory bowel disease, some of which are able to justify ILD). Moreover, EGPA with PR3-ANCA positivity, as well as GPA associated with MPO-ANCA are not uncommon [88]. For this reason, ANCAs are not currently included in any classification criteria, neither for AAV nor for IPAF [89].…”

Section: First-line Autoimmunity Examsmentioning

confidence: 99%

Patients with Interstitial Lung Disease Secondary to Autoimmune Diseases: How to Recognize Them?

Sambataro

Pignataro³

et al. 2020

Diagnostics

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The diagnostic assessment of patients with Interstitial Lung Disease (ILD) can be challenging due to the large number of possible causes. Moreover, the diagnostic approach can be limited by the severity of the disease, which may not allow invasive exams. To overcome this issue, the referral centers for ILD organized Multidisciplinary Teams (MDTs), including physicians and experts in complementary discipline, to discuss the management of doubtful cases of ILD. MDT is currently considered the gold standard for ILD diagnosis, but it is not often simple to organize and, furthermore, rheumatologists are still not always included. In fact, even if rheumatologic conditions represent a common cause of ILD, they are sometimes difficult to recognize, considering the variegated clinical features and their association with all possible radiographic patterns of ILD. The first objective of this review is to describe the clinical, laboratory, and instrumental tests that can drive a diagnosis toward a possible rheumatic disease. The secondary objective is to propose a set of first-line tests to perform in all patients in order to recognize any possible rheumatic conditions underlying ILD.

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“…Autoantibody titers did not show any increases, with the exception of PR3-ANCA. Increased ANCA titers are sometimes a false-positive result, especially if several organs are involved ( 32 ). For example, inflammatory bowel disease, infection, and malignancy occasionally result in an elevated ANCA titer ( 32 ).…”

Section: Discussionmentioning

confidence: 99%

Anti-neutrophil Cytoplasmic Antibody-associated Vasculitis (AAV) Restricted to the Limbs

Ojima¹,

Sawada

Fujii

et al. 2018

Intern. Med.

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A previously healthy 58-year-old man was admitted for muscle pain and weakness [manual muscle testing (MMT) of 4/4 for upper and lower limbs]. We detected elevated levels of inflammatory makers and PR3-anti-neutrophil cytoplasmic antibody (ANCA). Subsequently, the muscle weakness rapidly progressed to an MMT of 2 for all limbs. Magnetic resonance imaging indicated muscle edema, and the creatine kinase (CK) level increased to 29,998 U/L. Methylprednisolone (mPSL) and cyclophosphamide pulse therapy improved the patient symptoms. MMT recovered to 4 for all limbs. A muscle biopsy showed degenerated muscle fibers surrounded by neutrophil-predominant infiltration. In addition, lamina elastic breakdown and fibrinoid necrosis of arterioles were observed. A final diagnosis of microscopic polyangiitis (MPA) limited to the muscles was made.

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Diagnosing ANCA-associated vasculitis in ANCA positive patients

Abstract: Supplemental Digital Content is available in the text

Cited by 56 publications

References 34 publications

ANCA associated vasculitis

ANCA associated vasculitis

Patients with Interstitial Lung Disease Secondary to Autoimmune Diseases: How to Recognize Them?

Anti-neutrophil Cytoplasmic Antibody-associated Vasculitis (AAV) Restricted to the Limbs

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