An uncommon presentation of eosinophilic granulomatosis with polyangiitis: a case report

Abstract

IntroductionEosinophilic granulomatosis with polyangiitis is a rare and potentially fatal disease if not readily diagnosed. Cerebral involvement is extremely rare and clinical presentation as hemorrhagic stroke is even rarer.Case presentationA 58-year-old Caucasian man was admitted to our medical unit because of a computed tomography-diagnosed hemorrhagic stroke with right-sided hemiparesis and fever. A chest computed tomography scan also revealed multiple bilateral pulmonary infiltrates; coronary artery, and carotid and left vertebral artery calcifications were also observed. Empiric antimicrobial therapy with cephalosporins was promptly undertaken; low-molecular-weight heparin was introduced as prophylaxis for venous thromboembolism. Over the following days, magnetic resonance imaging scans showed a regression of the hemorrhagic framework, also revealing hypoxic areas consistent with acute ischemic lesions. With a computed tomography scan showing a worsening of his pulmonary framework, antimicrobial therapy was modified and corticosteroids were introduced. A new blood cell count revealed further increased leukocytosis (17.49×103μL), characterized by a surprising rise of eosinophilic cells (32.8%). Angiography of the coronary arteries found diffuse dilatations with severe signs of endothelial damage. Such an unexpected framework induced a strong suspicion that the stroke was the expression of a systemic vasculitis, which had triggered his cerebral, coronary, and pulmonary frameworks. The search for antineutrophil cytoplasmic antibody was positive for perinuclear antineutrophil cytoplasmic antibody, and eosinophilic granulomatosis with polyangiitis was diagnosed. Explaining to the patient the rarity of his disease, and what the most typical presentations of eosinophilic granulomatosis with polyangiitis were, he revealed that before admission he had had scalp injuries, in the nuchal region, and had taken corticosteroids as self-medication, with subsequent disappearance of the lesions. Therefore, high-dose corticosteroid treatment was started, and at discharge he was in good clinical condition with a slight right-sided hyposthenia.ConclusionsA diagnosis of eosinophilic granulomatosis with polyangiitis is often difficult, but we are convinced that intake of corticosteroids on a self-prescribed basis may have obscured the clinical presentation. Therefore, this case also suggests how the growing phenomenon of self-medication can be harmful, and that a careful investigation of clinical history is still an act of paramount importance.

Demographic and Clinical Characteristics Associated with Central Nervous System Hemorrhage in Patients with Eosinophilic Granulomatosis with Polyangiitis: A Case Report and Review of the Literature

“…In these cases, cerebral ischemic infarction (~80%) and diffuse encephalopathy (~10%) have been by far the findings most frequently reported. Published data on EGPA with CNS hemorrhage are limited to anecdotal case reports [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20]21,22,23,24,25,26 . We described a case of EGPA presenting with intracerebral hemorrhage (ICH) and did a comprehensive review of the literature.…”

Familial eosinophilic granulomatosis with polyangiitis in a mother and daughter

“…Pulmonary involvement consists of irregular, migratory and bilateral infiltrates with areas of ground-glass appearance, as in our case. Extrapulmonary findings have been commonly reported among patients with EGPA, such as constitutional symptoms, musculoskeletal symptoms, cutaneous, peripheral nervous system, cardiac and gastrointestinal involvement, mainly related to the vasculitic phase 1 6. The patient had weight loss and arthralgia; her mother had weight loss, paralysis and cutaneous involvement.…”

Eosinophilic granulomatosis with polyangiitis: myocardial thickening reversed by corticosteroids

“…Notably, he had a cerebral hemorrhage, likely due to the presence of ischemic lesions in combination with antiplatelet therapy. Vasculitis affecting the central nervous system and leading to hemorrhagic stroke is uncommon in EGPA, but has been reported [ 9 – 11 ]. One case report also described concomitant vertebral artery dissection [ 11 ].…”