A Case of Eosinophilic Granulomatosis with Polyangiitis Presenting with Subarachnoid Hemorrhage and Mononeuritis Multiplex

Se Hwan Oh; Won Park; Seong Ryul Kwon; Mie Jin Lim; Ko Woon Joo; Oh Hyun Lee; Ha Young Lee; Se Yang Oh; Kyong-Hee Jung

doi:10.4078/jrd.2015.22.3.190

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Oh, Park, Kwon, Lim, Joo, Lee, Lee, Oh, and Jung: A Case of Eosinophilic Granulomatosis with Polyangiitis Presenting with Subarachnoid Hemorrhage and Mononeuritis Multiplex

Case Report

J Rheum Dis 2015;22(3):190-194.

Published online: 31 October 2015

DOI: https://doi.org/10.4078/jrd.2015.22.3.190

A Case of Eosinophilic Granulomatosis with Polyangiitis Presenting with Subarachnoid Hemorrhage and Mononeuritis Multiplex

Se Hwan Oh¹, Won Park¹, Seong Ryul Kwon¹, Mie Jin Lim¹, Ko Woon Joo¹, Oh Hyun Lee¹, Ha Young Lee², Se Yang Oh³, Kyong-Hee Jung¹

¹Division of Rheumatology, Department of Internal Medicine, Inha University School of Medicine, Incheon, Korea

²Department of Radiology, Inha University School of Medicine, Incheon, Korea

³Department of Neurosurgery, Inha University School of Medicine, Incheon, Korea

Corresponding to: Kyong-Hee Jung, Division of Rheumatology, Department of Internal Medicine, Inha University Hospital, 27 Inhang-ro, Jung-gu, Incheon 400-711, Korea. E-mail: khjung@inha.ac.kr

Received 6 June 2014 Revised 1 August 2014 Accepted 1 August 2014

This is a Free Access article, which permits unrestricted non-commerical use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA), previously called Churg-Strauss syndrome, is an antineutrophil cytoplasmic antibody associated vasculitis, accompanied by asthma, hypereosinophilia, nonfixed pulmonary infiltrates, and sinusitis. Peripheral neuropathy is common in patients with EGPA; however, a few cases of EGPA with central nervous system (CNS) involvement have been reported. A 45-year-old female referred for right side weakness and posterior neck pain was diagnosed as EGPA with subarachnoid hemorrhage and mononeuritis multiplex. She was effectively treated with a high dose glucocorticoid, cyclophosphamide, and intravenous immunoglobulin. EGPA with CNS involvement is uncommon and causes significant morbidity and mortality. Therefore more rapid and accurate diagnostic evaluation may be required. EGPA should be considered in patients with neurological symptoms and hypereosinophilia.

Keywords: Churg-Strauss syndrome, Subarachnoid hemorrhage, Mononeuropathies

REFERENCES

1. Noth I, Strek ME, Leff AR. Churg-Strauss syndrome. Lancet. 2003; 361:587–94.

2. Masi AT, Hunder GG, Lie JT, Michel BA, Bloch DA, Arend WP, et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum. 1990; 33:1094–100.

3. Uhm WS. ANCA associated vasculitis. J Korean Rheum Assoc. 2010; 17:108–32.

4. Baldini C, Talarico R, Della Rossa A, Bombardieri S. Clinical manifestations and treatment of Churg-Strauss syndrome. Rheum Dis Clin North Am. 2010; 36:527–43.

5. Cheng MJ, Huang PH, Liao PW, Chen JT, Chiang TR. Multiple cerebral and cerebellar infarcts as the first clinical manifestation in a patient with Churg-Strauss syndrome: case report and literature review. Acta Neurol Taiwan. 2012; 21:169–75.

6. Nam TS, Jung HJ, Kim JT, Park MS, Kim BC, Kim MK, et al. Churg-Strauss syndrome complicated with intracerebral hemorrhage. J Korean Neurol Assoc. 2009; 27:257–9.

7. Go MH, Park JU, Kang JG, Lim YC. Subarachnoid and intracerebral hemorrhage in patients with Churg-Strauss syndrome: two case reports. J Cerebrovasc Endovasc Neurosurg. 2012; 14:255–61.

8. Mouthon L, Dunogue B, Guillevin L. Diagnosis and classification of eosinophilic granulomatosis with polyangiitis (formerly named Churg-Strauss syndrome). J Autoimmun. 2014; 48-49:99–103.

9. Vaglio A, Buzio C, Zwerina J. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): state of the art. Allergy. 2013; 68:261–73.

10. Calvo-Romero JM, del Carmen Bonilla-Gracia M, Bureo-Dacal P. Churg-Strauss syndrome presenting as spontaneous subarachnoid haemorrhage. Clin Rheumatol. 2002; 21:261–3.

11. Sakamoto S, Ohba S, Eguchi K, Shibukawa M, Kiura Y, Okazaki T, et al. Churg-Strauss syndrome presenting with subarachnoid hemorrhage from ruptured dissecting aneurysm of the intracranial vertebral artery. Clin Neurol Neurosurg. 2005; 107:428–31.

12. Sheerin UM, Barreto J, Brown MM, Brew S, Losseff NA. Subarachnoid haemorrhage as the first clinical manifestation of Churg-Strauss syndrome. J Neurol. 2008; 255:607–8.

13. Menditto VG, Di Rienzo A, De Nicola M, Balzano L, Polonara S. Subarachnoid haemorrhage from PICA aneurysm rupture in a Churg-Strauss patient: a case report and a review of the literature. Clin Neurol Neurosurg. 2013; 115:197–9.

14. van Gijn J, van Dongen KJ, Vermeulen M, Hijdra A. Perimesencephalic hemorrhage: a nonaneurysmal and benign form of subarachnoid hemorrhage. Neurology. 1985; 35:493–7.

15. Abril A. Churg-Strauss syndrome: an update. Curr Rheumatol Rep. 2011; 13:489–95.

Figure 1.

(A) Non-contrast-enhanced brain computed tomography image demonstrated subarachnoid hemorrhage in basal cistern, suprasellar cistern, perimesencephalic cistern and pre-pontine cistern. (B) Volume-ren-dered cerebral angiography image shows mild segmental stenosis at the P2 segment of left posterior cerebral artery (arrow).

Figure 2.

This chest computed tomography shows diffuse ground glass opacity, interlobular septal thickening. Small nodular lesion was seen in peripheral left upper lobe and right middle lobe (arrow).

Figure 3.

The progress report of this patient. Eosinophil count, antineutrophil cytoplasmic antibody (ANCA) titer and peripheral neuropathy are improved gradually after administration of high dose methylprednisolone, cylclophosphamide and intravenous immunoglobulin (IVIG).

Table 1.

Summary of reports of subarachnoid hemorrhage in eosinophilic granulomatosis with polyangiitis patients

Case	Age (yr)	Sex	Initial manifestation	Eosinophil count (%)	ANCA	Angiography	Treatment	Outcome
Case 1 [7]	39	M	Tonic clonic seizures	19	P-ANCA: positive	Left vertebral artery dissection	MP (1 mg/kg/d), CYC, followed by daily PDN 1 mg /kg	Death
Case 2 [10]	47	F	Severe headache	46.6	C-ANCA: positive	Vasculitis in the basilar artery without aneurysm or arteriovenous malformation	PDN (1 mg/kg/d) and CYC (2 mg/kg/d)	Remission
Case 3 [11]	36	F	Severe headache, vomiting	38.4	ANCA: negative	Vasculitis in the basilar artery and intracranial dissecting aneurysm	Coil embolization and PDN	Remission
Case 4 [12]	37	F	Severe headache	17.4	P-ANCA: positive	Vasculitis	MP	Not descriptive
Case 5 [13]	64	F	Headache, nuchal pain, vomiting	10.9	P-ANCA: positive	A left PICA aneurysm associated with a focal narrowing of the ipsilateral proximal vertebral artery	CYC (2 mg/kg/d)	Remission
Our case	45	F	Right side weakness	62.7	P-ANCA: positive	Mild segmental stenosis at the P2 segment of left PCA	MP (1 g/d), CYC (15 mg/kg), IVIG (400 mg/kg/d), followed by PDN (5 mg/d) and AZA	Remission

ANCA: antineutrophil cytoplasmic antibody, AZA: azathioprine, CYC: cyclophosphamide, F: famale, IVIG: intravenous immunoglobulin, M: male, MP: methylprednisolone PCA: posterior cerebral artery, PDN: prednisolone, PICA: posterior inferior cerebellar artery.

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