Wegener's Granulomatosis with Nervous System Involvement: A Hospital-Based Study

Huang, Yuhua; Ro, Long‐Sun; Lyu, Rong‐Kuo; Chang, Hsun-Yun; Wu, Yih-Ru; Chang, Kuo-Hsuan; Kuo, H.P.

doi:10.1159/000375492

Cited by 13 publications

(15 citation statements)

References 22 publications

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“…Headache was again the predominant symptom (66% of cases) . Additionally, a similar incidence of HP (44%) was reported in a series of patients who developed such severe CNS complications that a hospitalization in a neurological department was eventually required . Following similar observations, the diagnostic criteria for GPA have been revised to include a limited form of GPA …”

Section: Discussionmentioning

confidence: 71%

“…18 Additionally, a similar incidence of HP (44%) was reported in a series of patients who developed such severe CNS complications that a hospitalization in a neurological department was eventually required. 7 Following similar observations, the diagnostic criteria for GPA have been revised to include a limited form of GPA. 2,15 A more recent study 15 confirmed the above observations, reporting a 36% incidence of HP in patients with GPA and CNS involvement (9 of 25 patients).…”

Section: Discussionmentioning

confidence: 99%

“…GPA has a wide spectrum of clinical manifestations, including symptoms from the upper and lower respiratory system (hearing loss, epistaxis, inflammation, etc), cardiovascular symptoms (vasculitis, occlusive vascular disease, pericarditis, cardiomyopathy, ischemic heart disease, etc), gastrointestinal symptoms (mesenteric vasculitis, peritonitis), cutaneous and mucocutaneous symptoms. Renal participation may even lead to end‐stage renal failure …”

Section: Introductionmentioning

confidence: 99%

“…Renal participation may even lead to end-stage renal failure. 6,7 Involvement of the nervous system, even as the initial manifestation, is not uncommon, with reported frequencies ranging from 22 to 54% of patients. In older studies, peripheral nervous system (PNS) involvement seemed to dominate, predominantly as polyneuropathy or mononeuritis multiplex due to vasculitis of the vasa nervorum, whereas lesions of the central nervous system (CNS) were considered rather rare, with reported frequencies ranging from 7 to 11% (modern authors exclude cranial nerve palsies from CNS manifestations).…”

Section: Introductionmentioning

confidence: 99%

“…[10][11][12][13] However, in the modern era of neuroimaging, thanks to the increased availability of MRI scans, reports are focusing more and more on granulomatous manifestations of GPA and the impact of their CNS sequelae, 14 especially on the increased frequency of patients with hypertrophic pachymeningitis (HP) (as high as 36% of patients with GPA and CNS involvement or even 44% of more severe cases). 7,15 In light of the above development, we report the case of a patient with headache linked to intracranial hypertension and hypertrophic pachymeningitis as the initial and dominant presentation of GPA and we review the recent literature.…”

Section: Introductionmentioning

confidence: 99%

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Headache Linked to Intracranial Hypertension and Hypertrophic Pachymeningitis as the Initial and Dominant Presentation of Granulomatosis With Polyangiitis. Case Report and Review of the Recent Literature

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Section: Discussionmentioning

confidence: 71%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Headache Linked to Intracranial Hypertension and Hypertrophic Pachymeningitis as the Initial and Dominant Presentation of Granulomatosis With Polyangiitis. Case Report and Review of the Recent Literature

et al. 2018

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A 55‐Year‐Old Male Presenting With Altered Mental Status: A Complicated Case of Intracranial Aneurysm

Garg

Khosroshahi

2017

Arthritis Care & Research

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A 55-year-old man was transferred to our hospital for further evaluation of altered mental status and fusiform cerebral aneurysms. History of present illnessA 55-year-old African American male was in good health 5 months prior when he underwent a colonoscopy as part of a routine colon cancer screening. After the procedure, he developed colitis and was treated with antibiotics for 5 days with resolution of his symptoms. One month after this episode he was admitted at an outside hospital for constellation of fever, bilateral ankle swelling, and petechial pruritic rash on his lower extremities. The patient did not experience other symptoms such as weight loss, cough, hemoptysis, painful vision loss, oral ulcers, neurologic deficits, or gastrointestinal bleeding. During this hospitalization, the patient was found to have an elevated creatinine level of 2.65 mg/dl. Additional laboratory values were hemoglobin (9.8 gm/dl), white blood cells (5,000/ll), platelets (200,000/ll), normal liver enzymes, and low albumin (2.8 mg/dl). The serologic studies revealed elevated rheumatoid factor (RF; 114 IU/ml, normal value <20 IU/ml) and low complements C3 (49 mg/dl, normal value 80-252 mg/dl) and C4 (9 mg/dl, normal value 12-17 mg/dl), while other serologic markers, antineutrophil cytoplasmic antibody (ANCA), proteinase 3 (PR3) antibody, myeloperoxidase (MPO) antibody, antinuclear antibody (ANA), and cryoglobulins, were reported to be negative. His urinalysis revealed red blood cell (RBC) casts, dysmorphic RBCs, and 2+ protein. Given his presentation with constitutional symptoms, rash, and elevated creatinine with subnephrotic range proteinuria and casts, he underwent a kidney biopsy, the findings of which revealed "necrotizing glomerulonephritis with crescents, pauci-immune staining pattern with glomerular C3 dense deposit, which could be seen in infectious process-like endocarditis/abscesses." The patient was diagnosed with ANCA-associated vasculitis (AAV) based on a pauciimmune staining pattern and cresentric glomerulonephritis on his kidney biopsy results, and he was started on 60 mg of daily oral prednisone and 100 mg of daily oral cyclophosphamide. The patient continued these doses of prednisone and cyclophosphamide for 3 months without significant improvement in his proteinuria (persistently 2+ on urine dipstick, 1.5 gm on quantification) and creatinine levels (remained elevated in range 1.6-2.2 mg/dl).The patient was admitted again at an outside hospital for evaluation of shortness of breath and chest tightness. He was found to have mild elevation in cardiac enzymes and underwent cardiac catheterization as part of cardiac assessment. Soon after cardiac catheterization, he became delirious and was found to have an intracranial hemorrhage. An angiogram at the outside hospital highlighted an intracranial aneurysm; he was transferred to our hospital for embolization of the aneurysm. Upon arrival, he was intubated for airway protection and underwent repeat angiogram with demonstration of 2 fusiform aneurysms. Medical and famil...

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Characteristics of hypertrophic pachymeningitis in patients with granulomatosis with polyangiitis

2017

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Hypertrophic pachymeningitis (HP) is an important neurologic complication of granulomatosis with polyangiitis (GPA, formerly Wegener's granulomatosis). The aim of this study is to investigate the clinical features, radiological findings, and diagnostic pitfalls of GPA-related HP. A retrospective chart review was performed to screen patients diagnosed with GPA at Samsung Medical Center between 1997 and 2016. Neurologic manifestation, laboratory findings, neuroimaging data, and clinical course were evaluated in all patients. Characteristics of patients with HP were compared to those of patients without HP. Sixty-five patients with GPA were identified. Twenty-five of these patients had central nervous system involvement. HP (N = 9, 36%) was the second most common radiologic finding. Other neurologic findings included stroke (N = 7, 28%) and granulomatous disease (N = 10, 40%). Patients with HP had lower incidences of systemic manifestations (N = 2, 22.2% vs. N = 38, 67.9%, p = 0.013 in the lung and N = 1, 11.1% vs. N = 28, 50.0%, p = 0.030 in the kidney) than those without HP. Six patients with GPA-related HP were MPO-ANCA positive (66.7%) and two had PR3-ANCA (22.2%). Most of the patients with HP presented with headache (N = 8, 88.9%) at a rate that is similar to those of primary headache disorders (migraine, tension-type, and stabbing) and other secondary headache disorders (postural type and meningitis). Patients with HP rarely had neurologic deficits (N = 3, 37.5%). Different clinical or radiologic features may be observed in GPA-related HP. Early recognition and accurate diagnosis of GPA-related HP are needed in addition to neuroimaging findings.

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Wegener's Granulomatosis with Nervous System Involvement: A Hospital-Based Study

Cited by 13 publications

References 22 publications

Headache Linked to Intracranial Hypertension and Hypertrophic Pachymeningitis as the Initial and Dominant Presentation of Granulomatosis With Polyangiitis. Case Report and Review of the Recent Literature

Headache Linked to Intracranial Hypertension and Hypertrophic Pachymeningitis as the Initial and Dominant Presentation of Granulomatosis With Polyangiitis. Case Report and Review of the Recent Literature

A 55‐Year‐Old Male Presenting With Altered Mental Status: A Complicated Case of Intracranial Aneurysm

Characteristics of hypertrophic pachymeningitis in patients with granulomatosis with polyangiitis

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