The online version of this article (doi:10.1186/1752-1947-8-190) contains supplementary material, which is available to authorized users.
The authors declare that they have no competing interests.
GT contributed to the clinical management of the patient, was responsible for acquisition of data, and edited the manuscript; GA performed the coronary angiography, and helped write the manuscript; MAB performed the radiology analysis and helped in writing the manuscript; EJC-G contributed to the review of the literature and the writing of the manuscript; AB contributed to the review of the literature and the writing of the manuscript; SB was responsible for the clinical management of the patient, and coordinated and edited the manuscript. All authors have read and approved the final manuscript.
Eosinophilic 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.
A 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.
A 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.
Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, Flores-Suarez LF, Gross WL, Guillevin L, Hagen EC, Hoffman GS, Jayne DR, Kallenberg CG, Lamprecht P, Langford CA, Luqmani RA, Mahr AD, Matteson EL, Merkel PA, Ozen S, Pusey CD, Rasmussen N, Rees AJ, Scott DG, Specks U, Stone JH, Takahashi K, Watts RA: 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2012, 65: 1-11. CrossRef
Lanham JG, Elkon KB, Pusey CD, Hughes GR: Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss syndrome. Medicine (Baltimore). 1984, 63: 65-81. CrossRef
Guillevin L, Cohen P, Gayraud M, Lhote F, Jarrousse B, Casassus P: Churg-Strauss syndrome. Clinical study and long-term follow-up of 96 patients. Medicine (Baltimore). 1999, 78: 26-37. 10.1097/00005792-199901000-00003. CrossRef
Sablé-Fourtassou R, Cohen P, Mahr A, Pagnoux C, Mouthon L, Jayne D, Blockmans D, Cordier JF, Delaval P, Puechal X, Lauque D, Viallard JF, Zoulim A, Guillevin L: Antineutrophil cytoplasmic antibodies and the Churg-Strauss syndrome. Ann Intern Med. 2005, 143: 632-638. 10.7326/0003-4819-143-9-200511010-00006. CrossRefPubMed
Association of the European Self-Medication Industry: Self-Medication Market. http://www.aesgp.eu/facts-figures/market-data/self-medication-market/ (accessed on 16th October 2013)
Luqmani RA, Bacon PA, Moots RJ, Janssen BA, Pall A, Emery P, Savage C, Adu D: Birmingham Vasculitis Activity Score (BVAS) in systemic necrotizing vasculitis. QJM. 1994, 87: 671-678. PubMed
Guillevin L, Pagnoux C, Seror R, Mahr A, Mouthon L, Le Toumelin P: The Five-Factor Score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort. Medicine (Baltimore). 2011, 90: 19-27. 10.1097/MD.0b013e318205a4c6. CrossRef
Mukhtyar C, Guillevin L, Cid MC, Dasgupta B, de Groot K, Gross W, Hauser T, Hellmich B, Jayne D, Kallenberg CG, Merkel PA, Raspe H, Salvarani C, Scott DG, Stegeman C, Watts R, Westman K, Witter J, Yazici H, Luqmani R: EULAR recommendations for the management of primary small and medium vessel vasculitis. Ann Rheum Dis. 2009, 68: 310-317. 10.1136/ard.2008.088096. CrossRefPubMed
Bosch X, Guilabert A, Espinosa G, Mirapeix E: Treatment of antineutrophil cytoplasmic antibody associated vasculitis: a systematic review. J Am Med Ass. 2007, 298: 655-669. 10.1001/jama.298.6.655. CrossRef
- An uncommon presentation of eosinophilic granulomatosis with polyangiitis: a case report
Maria Aurelia Banco
Edy Julia Costanza-Gaglio
- BioMed Central