Does One Size Fit All?

Kemna, Michael J.; Tervaert, Jan Willem Cohen

doi:10.3899/jrheum.130805

Cited by 5 publications

(9 citation statements)

References 19 publications

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“…Finally, 5 of 62 patients who were initially diagnosed with nonrenal disease developed a renal relapse, suggesting that we should probably focus on markers of the biologic behavior of the disease in patients that initially present with nonrenal disease in order to predict the value of serial ANCA measurements in this patient subgroup. 28 In summary, we have demonstrated in the largest published cohort with longitudinal ANCA measurements that an ANCA rise is related to a relapse in patients with renal involvement, but less in patients with nonrenal disease.…”

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confidence: 63%

ANCA as a Predictor of Relapse

Kemna

Damoiseaux

Austen

et al. 2015

Journal of the American Society of Nephrology

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The value of measuring ANCA during follow-up to predict a relapse is controversial. On the basis of recently obtained pathophysiologic insights, we postulated that measuring ANCA is useful in patients with renal involvement but is less valuable in patients with nonrenal disease. One hundred sixty-six consecutive patients with ANCA-associated vasculitis, positive for either proteinase 3 (PR3)-ANCA or myeloperoxidase (MPO)-ANCA, were included in our study, followed at regular intervals, and tested for PR3-ANCA and MPO-ANCA. In this cohort, 104 patients had renal involvement (72 with PR3-ANCA, 32 with MPO-ANCA) and 62 patients had nonrenal disease (36 with PR3-ANCA, 26 with MPO-ANCA). During an average (6SD) follow-up of 49633 months and 18614 ANCA measurements, 89 ANCA rises and 74 relapses were recorded. ANCA rises correlated with relapses in patients who presented with renal involvement (hazard ratio [HR], 11.09; 95% confidence interval [95% CI], 5.01 to 24.55), but in comparison, associated only weakly with relapses in patients who presented with nonrenal disease (HR, 2.79; 95% CI, 1.30 to 5.98). In conclusion, longitudinal ANCA measurements may be useful in patients with renal involvement but is less valuable in patients with nonrenal disease.

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confidence: 63%

ANCA as a Predictor of Relapse

Kemna

Damoiseaux

Austen

et al. 2015

Journal of the American Society of Nephrology

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191

180

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“…Future collaborations may increase the amount of included patients and will enable us to analyze the predictive value of a panel of biomarkers to better predict relapses. As previously stated, these results may subsequently lead to a classification system that is focused on the biologic behavior of the disease and may in the future be used to guide maintenance therapy in patients with AAV [16].…”

Section: Expert Opinionmentioning

confidence: 85%

“…In addition, patients within clinical entities such as GPA and MPA have been categorized based on the severity of disease activity [16]. Organ involvement at the time of active disease and the severity thereof has been summarized in the Birmingham vasculitis activity score (BVAS) [17], while the extent of the disease is summarized by the disease extent index (DEI) [18].…”

Section: Patient Classifications -The Old-fashioned Waymentioning

confidence: 99%

“…The purpose of these classification systems is to provide a framework to tailor induction therapy based on the severity of the disease at the initial presentation [19,21]. During the last decade, it became clear that these systems are of limited prognostic value since they only reflect the severity of disease at initial presentation and do not take the clinical follow up into account [16]. Therefore, these classification systems are limited in the guidance of maintenance therapy [22].…”

Section: Patient Classifications -The Old-fashioned Waymentioning

confidence: 99%

“…Therefore, DPB4 homozygous patients are prone to relapse also when they are MPO-ANCA positive. The incorporation of these findings into a classification system may lead to a classification that is focused on the biologic behavior of the disease and may in the future be used to guide maintenance therapy in patients with AAV [16].…”

Section: Patient Classifications -Based On the Chance To Relapsementioning

confidence: 99%

See 2 more Smart Citations

Maintaining remission in patients with granulomatosis with polyangiitis or microscopic polyangiitis: the role of ANCA

Kemna¹,

Paassen²,

Damoiseaux³

et al. 2017

Expert Opinion on Orphan Drugs

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Introduction: Granulomatosis with Polyangiitis (GPA; formerly Wegener's) and Microscopic Polyangiitis (MPA) are inflammatory disease entities affecting small to medium vessels. They are characterized by the presence of anti-neutrophil cytoplasmic antibodies (ANCA) and are frequently grouped together with Eosinophilic Granulomatosis with Polyangiitis (EGPA; formerly Churg Strauss Syndrome) under the term ANCA-associated Vasculitis (AAV). Due to effective immunosuppressive therapy that was introduced more than 45 years ago, AAV has become a chronic disease in which relapses occur frequently during maintenance treatment or later on after all immunosuppressive therapy is stopped. Since relapses are associated with morbidity and mortality, early detection and prevention of relapses is of great importance. Areas covered: This review focuses on the potential strategies to guide maintenance therapy in patients with GPA or MPA, with an emphasis on patient classifications and the role of serial ANCA measurements. Expert opinion: Risk factors for relapses, e.g., genetic background of the patient, have been studied during the last three decades. Only few of these risk factors directly influence the management of patients. One potentially important factor that may influence therapeutic decisions in AAV patients is serial measurement of ANCA. Recently, it became clear that serial ANCA measurement is an attractive approach in AAV patients with (a history of) capillaritis, e.g., renal involvement or alveolar haemorrhage, but not in patients with more limited disease. Whether ANCA rises can be used to guide therapy andhence-to prevent relapses has been a great debate during the last three decades. At present, we conclude that small studies do support such an approach but that these findings require further validation in larger randomized clinical trials. ARTICLE HISTORY

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Positron Emission Tomography scanning in Anti-Neutrophil Cytoplasmic Antibodies-Associated Vasculitis

et al. 2015

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Tools for evaluation of disease activity in patients with anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) include scoring clinical manifestations, determination of biochemical parameters of inflammation, and obtaining tissue biopsies. These tools, however, are sometimes inconclusive. 2-deoxy-2-[18F]-fluoro-D-glucose (FDG) positron emission tomography (PET) scans are commonly used to detect inflammatory or malignant lesions. Our objective is to explore the ability of PET scanning to assess the extent of disease activity in patients with AAV.Consecutive PET scans made between December 2006 and March 2014 in Maastricht (MUMC) and between July 2008 and June 2013 in Brussels (EUH) to assess disease activity in patients with AAV were retrospectively included. Scans were re-examined and quantitatively scored using maximum standard uptake values (SUVmax). PET findings were compared with C-reactive protein (CRP) and ANCA positivity at the time of scanning.Forty-four scans were performed in 33 patients during a period of suspected active disease. All but 2 scans showed PET-positive sites, most commonly the nasopharynx (n = 22) and the lung (n = 22). Forty-one clinically occult lesions were found, including the thyroid gland (n = 4 patients), aorta (n = 8), and bone marrow (n = 7). The amount of hotspots, but not the highest observed SUVmax value, was higher if CRP levels were elevated. Seventeen follow-up scans were made in 13 patients and showed decreased SUVmax values.FDG PET scans in AAV patients with active disease show positive findings in multiple sites of the body even when biochemical parameters are inconclusive, including sites clinically unsuspected and difficult to assess otherwise.

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Does One Size Fit All?

Cited by 5 publications

References 19 publications

ANCA as a Predictor of Relapse

ANCA as a Predictor of Relapse

Maintaining remission in patients with granulomatosis with polyangiitis or microscopic polyangiitis: the role of ANCA

Positron Emission Tomography scanning in Anti-Neutrophil Cytoplasmic Antibodies-Associated Vasculitis

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