Serum biomarkers are similar in Churg–Strauss syndrome and hypereosinophilic syndrome

Abstract

Rationale Churg–Strauss syndrome (CSS) and hypereosinophilic syndrome (HES) overlap considerably in clinical presentation. A reliable means of distinguishing between these groups of patients is needed, especially in the setting of glucocorticoid therapy. Methods A retrospective chart review of 276 adult subjects referred for evaluation of eosinophilia > 1500/μl was performed, and subjects with a documented secondary cause of eosinophilia or a PDGFR ‐positive myeloproliferative neoplasm were excluded. The remaining subjects were assessed for the presence of American College of Rheumatology (ACR) criteria. Laboratory and clinical parameters were compared between subjects with biopsy‐proven vasculitis (CSS; n = 8), ≥4 ACR criteria (probable CSS; n = 21), HES with asthma and/or sinusitis without other CSS‐defining criteria (HESwAS; n = 20), HES without asthma or sinusitis (HES; n = 18), and normal controls (n = 8). Serum biomarkers reported to be associated with CSS were measured using standard techniques. Results There were no differences between the subjects with definite or probable CSS or HES with respect to age, gender, or maintenance steroid dose. Serum CCL17, IL‐8, and eotaxin levels were significantly increased in eosinophilic subjects as compared to normal controls, but were similar between the eosinophilic groups. Serum CCL17 correlated with eosinophil count (P < 0.0001, r = 0.73), but not with prednisone dose. Conclusions In patients with a history of asthma and sinusitis, distinguishing between ANCA‐negative CSS and PDGFR ‐ne‐gative HES is difficult because of significant overlap in clinical presentation and biomarker profiles.

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.…”

Primary eosinophilic lung diseases

“…Analysis of serum biomarkers between patients with CSS or HES has not revealed any useful marker to distinguish between the two diseases. 57 In a large multicenter HES cohort, the most common initial presenting manifestations were dermatologic (37%), pulmonary (25%), and GI (14%). 51 Respiratory involvement was also the second most common subsequent clinical manifestation of HES.…”

Acute heart failure with left ventricular thrombus in the course of eosinophilic granulomatosis with polyangiitis

“…Z danych literaturowych wynika, iż astma oskrzelowa może wyprzedzać rozpoznanie EGPA nawet o 30 lat. W początkowym okresie choroby różnicowanie zespołu hipereozynofilowego i EGPA może być bardzo trudne [10]. O ile objawy ze strony serca i płuc mogą być bardzo podobne w obu schorzeniach, o tyle zmiany skórne, zajęcie układu nerwowego (najczęściej obwodowego), a także nerek, związane z kwasochłonnym zapaleniem naczyń, występują w zasadzie tylko w EGPA, stanowiąc charakterystyczny komponent tego zespołu [5,11].…”