Prevalence and clinical significance of antineutrophil cytoplasmic antibodies in Churg‐Strauss syndrome

Abstract

Objective. Churg-Strauss syndrome (CSS) is classified among the so-called antineutrophil cytoplasmic antibody-associated systemic vasculitides (AASVs) because of its clinicopathologic features that overlap with the other AASVs. However, while antineutrophil cytoplasmic antibodies (ANCAs) are consistently found in 75-95% of patients with Wegener's granulomatosis or microscopic polyangiitis, their prevalence in CSS varies widely and their clinical significance remains uncertain. We undertook this study to examine the prevalence and antigen specificity of ANCAs in a large cohort of patients with CSS. Moreover, we evaluated the relationship between ANCA positivity and clinicopathologic features.Methods. Immunofluorescence and enzymelinked immunosorbent assay were used to determine the presence or absence of ANCAs in 93 consecutive patients at the time of diagnosis. The main clinical and pathologic data, obtained by retrospective analysis, were correlated with ANCA status.Results. ANCAs were present by immunofluorescence in 35 of 93 patients (37.6%). A perinuclear ANCA (pANCA) pattern was found in 26 of 35 patients (74.3%), with specificity for myeloperoxidase (MPO) in 24 patients, while a cytoplasmic ANCA pattern, with specificity for proteinase 3, was found in 3 of 35 patients (8.6%). Atypical patterns were found in 6 of 30 patients with anti-MPO antibodies (20.0%). ANCA positivity was associated with higher prevalences of renal disease (51.4% versus 12.1%; P < 0.001) and pulmonary hemorrhage (20.0% versus 0.0%; P ‫؍‬ 0.001) and, to a lesser extent, with other organ system manifestations (purpura and mononeuritis multiplex), but with lower frequencies of lung disease (34.3% versus 60.3%; P ‫؍‬ 0.019) and heart disease (5.7% versus 22.4%; P ‫؍‬ 0.042).Conclusion. ANCAs are present in ϳ40% of patients with CSS. A pANCA pattern with specificity for MPO is found in most ANCA-positive patients. ANCA positivity is mainly associated with glomerular and alveolar capillaritis.

Churg–Strauss syndrome: retrospective study in Burgundian population in France in past 10 years

“…The majority of the demographic data as well as the proportion of each clinical manifestations are similar to those of the studies published to date (Figure4) [2,4,[16][17][18][19][20].…”

“…In our series, we have found a positivity of the ANCA (generally anti-MPO pANCA) in 26% of cases which is slightly lower than in the other series [5,19,20]. The difference between clinical manifestations of o ur study and the others series like more heart manifestation can maybe explain this low rate of ANCA positivity [22].…”

How I treat hypereosinophilic syndromes

“…42 This is particularly true for the 30% to 40% of EGPA patients who do not have detectable levels of anti-neutrophil cytoplasmic antibodies. 43 Whereas glucocorticoid therapy is recommended for the initial treatment of both EGPA and PDGFRA-negative HES, the recommended dose and duration of glucocorticoids and choices of second-line therapies differ. 44 Associated HE/HES (eosinophilia >1.5 3 10 9 /L in the setting of a distinct diagnosis, in which eosinophilia has been described in a subset of affected patients) HE/HES can complicate a wide variety of clinical diagnoses.…”

Clinical characterization and successful treatment of 6 patients with Churg–Strauss syndrome-associated neuropathy

“…We suppose that the pathogenic mechanisms in ANCA-positive CSS patients might differ from those in ANCA-negative ones [11,12]. These auto-antibodies might enhance focal inflammation and detection of them could be a poor prognostic factor in CSS-associated neuropathy.…”

“…Although two reports presented that ANCA-positivity in CSS patients reflects a more active vasculitic process and is correlated with the frequency of neuropathy [11,12], there are no previous reports clearly indicating the association between serum ANCA and the clinical features of neuropathy. We encountered 6 patients with CSS-associated neuropathy within 24 months.…”