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

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare form of systemic vasculitis in which cardiac involvement is frequent and severe, and accounts for half of EGPA-related deaths. ANCA-positive EGPA differs from ANCA-negative EGPA in that the former is significantly associated with renal involvement, peripheral neuropathy and biopsy proven vasculitis, whereas the latter is associated with cardiac involvement. Herein, we report a case of EGPA with myocarditis in a woman, who was successfully treated with steroids and cyclophosphamide. This report highlights the importance of diagnosing cardiac involvement in EGPA early, especially in ANCA-negative patients.

Idiopathic Hypereosinophilic Syndrome With Cutaneous Manifestations and Flame Figures

“…There were no signs or symptoms of intoxication or withdrawal throughout her hospitalization to suggest illegal drug-induced eosinophilia. Although cardiac involvement can be associated with antineutrophilic cytoplasmic antibody-negative CSS, 13 our patient did not demonstrate upper respiratory manifestations and did not respond to corticosteroids, which are characteristic features of CSS. In addition, presence of mild dyspoietic changes in the myeloid elements are features that support a primary hematologic disorder rather than an autoimmune disease.…”

A case report and literature review of Churg–Strauss syndrome presenting with myocarditis

“…The habits of males, such as smoking, likely have a synergistic effect with eosinophils on the heart [5] . As well, estrogen probably protects the female cardiovascular system against inflammation [9] . Further research is needed to verify and illuminate the protective effect of sex on CSS.…”

“…ANCAs are reported in ∼40% of CSS cases, [2,9] the presence associated with increased risk of glomerular disorders, biopsy-proven vasculitis, and peripheral neuropathy and the absence related to heart disease. From our case and the literature, almost all cases with cardiac involvement are usually negative for ANCAs (14 of 16), and 1 ANCA-positive case involves the kidney.…”

“…To conclude, our CSS patient with a predominant cardiac manifestation and without renal impairment lacked a characteristic ANCA pattern, which agrees with reported findings. [2,9] Only 3 of 22 CSS cases were complicated by renal involvement, [10–12] so myocarditis and renal involvement may be rarely present at the same time, which may be due to the ANCAs.…”