Churg-Strauss Syndrome: Clinical Symptoms, Complementary Investigations, Prognosis and Outcome, and Treatment

Abstract

Churg-Strauss syndrome (CSS), first described in 1951, is a rare vasculitis of small- and medium-sized vessels. It is characterized by a constant association with asthma and eosinophilia, and by the presence of anti-myeloperoxidase (MPO) anti-neutrophil cytoplasmic antibodies (ANCA) in ~40% of the patients. Vasculitis typically develops in a previously asthmatic and eosinophilic middle-aged patient and most frequently involves the peripheral nerves and skin. Other organs, however, may be affected and must be screened for vasculitis, especially those associated with a poorer prognosis, such as the heart, kidney, and gastrointestinal tract, as assessed by the recently revised Five-Factor Score (FFS). Overall survival of CSS patients is excellent, but relapses are not uncommon and require maintenance or steroid-sparing therapies, depending on the original FFS-based prognosis at diagnosis. All patients require corticosteroids, often for prolonged periods, combined with immunosuppressants [e.g., induction (cyclophosphamide) and maintenance therapy (azathioprine)], for those with poorer prognoses. Recent insights, especially concerning clinical differences associated with ANCA status, showed that CSS patients might constitute a heterogeneous group, both clinically and pathogenically. Future therapies might reflect these differences more strongly.

How I treat hypereosinophilic syndromes

“…For example, imatinib mesylate is most appropriate if myeloproliferative disease is suspected, 10 but is unlikely to be effective in a patient with lymphocyte-driven HES. Conversely, cyclophosphamide is effective in eosinophilic vasculitis 13 but would not be the treatment of choice for a patient with PDGFRA-associated MPN. Additional agents that have been used to rapidly lower counts in steroid-refractory patients include high-dose hydroxyurea, vincristine, and mepolizumab (humanized anti-interleukin eosinophilic gastrointestinal disorders *Allergic disorders, including asthma and atopic dermatitis, can be associated with HE (AEC $1.5 3 10 9 /L), especially in children, although extremely high eosinophil counts (AEC $5.0 3 10 9 /L) should prompt consideration of another cause.…”

Severe cardiomyopathy revealing antineutrophil cytoplasmic antibodies‐negative eosinophilic granulomatosis with polyangiitis

“…7,[9][10][11] EGPA has been associated with different cardiac manifestations, including myocarditis, coronary vasculitis, valvular heart disease, pericarditis and/or rhythm disorders. 7,11,12 Histologically, myocardial damage is rarely caused by small-vessel vasculitis but rather by the in situ production of toxic mediators, thus supporting the phenotypic differences between ANCA-positive and ANCAnegative EGPA. 7 The ECG usually shows non-specific conductance disturbances or patterns of acute coronary Figure 1 (A,B) Normal coronary angiography.…”

Clinical Features And Prognostic Factors Of Churg-strauss Syndrome

“…The second stage is characterized by peripheral blood eosinophilia with eosinophil tissue infiltration. In the third stage, vasculitis prevails [4]. Statistical analyses were conducted using the MannWhitney U test and Fisher exact test for comparisons between groups: ANCA(+) versus ANCA(-) patients, and good versus poor responders.…”

“…Although the annual incidence is low in the general population, 2.4 to 13 per million persons, it is relatively high in asthma patients, 34.6 to 64.6 per million persons [4,5]. It is not easy to diagnose CSS because of its low incidence and the variety of clinical features at each the stage.…”