Severe CNS manifestations as the clinical hallmark in generalized Wegener's granulomatosis consistently negative for antineutrophil cytoplasmic antibodies (ANCA). A report of 3 cases and a review of the literature

Clinical and Experimental Rheumatology

Volumn 19, Issue 5, 2001, Pages 541-549

  Reinhold Keller, E ^a   De Groot, K ^a   Holl Ulrich, K ^a   Arlt, A C ^a   Heller, M ^a   Feller, A C ^a   Gross, W L ^a  

^a UNIVERSITY OF LÜBECK   (Germany)

Author keywords

Antineutrophil cytoplasmic antibodies (ANCA); CNS manifestation; Meningitis; Wegener's granulomatosis

Indexed keywords

AZATHIOPRINE; CYCLOPHOSPHAMIDE; IMMUNOGLOBULIN A; IMMUNOGLOBULIN G; IMMUNOGLOBULIN M; IMMUNOSUPPRESSIVE AGENT; METHOTREXATE; MYELOBLASTIN; NEUTROPHIL CYTOPLASMIC ANTIBODY; PREDNISONE;

ADULT; ANTIBODY TITER; ARTICLE; BIOPSY; BRAIN DISEASE; CASE REPORT; CENTRAL NERVOUS SYSTEM DISEASE; CLINICAL EXAMINATION; CLINICAL OBSERVATION; COHORT ANALYSIS; CONTROLLED STUDY; DISEASE ACTIVITY; DISEASE MARKER; DISEASE SEVERITY; ELECTROENCEPHALOGRAM; ENZYME LINKED IMMUNOSORBENT ASSAY; ENZYME MECHANISM; FATALITY; FEMALE; FLUORESCENCE; HUMAN; HUMAN TISSUE; IMMUNOSUPPRESSIVE TREATMENT; LABORATORY TEST; MALE; MEDICAL LITERATURE; MENINX DISORDER; NUCLEAR MAGNETIC RESONANCE IMAGING; PRIORITY JOURNAL; RETROSPECTIVE STUDY; SENSITIVITY AND SPECIFICITY; SEROLOGY; SKIN BIOPSY; SPINE DISEASE; STANDARDIZATION; TIME; WEGENER GRANULOMATOSIS;

ADULT; ANTIBODIES, ANTINEUTROPHIL CYTOPLASMIC; CHILD; CYCLOPHOSPHAMIDE; ENZYME-LINKED IMMUNOSORBENT ASSAY; FATAL OUTCOME; FEMALE; HUMANS; IMMUNOSUPPRESSIVE AGENTS; MALE; MENINGITIS; SEROLOGIC TESTS; TREATMENT OUTCOME; VASCULITIS, CENTRAL NERVOUS SYSTEM; WEGENER GRANULOMATOSIS;

EID: 0034821902     PISSN: 0392856X     EISSN: None     Source Type: Journal    
DOI: None     Document Type: Article

References (36)

1. Anticytoplasmic autoantibodies: Their immunodiagnostic value in Wegener's granulomatosis
2. The diagnostic value of standardized assays for antineutrophil cytoplasmic antibodies (ANCA) in systemic vasculitis. Results of an international collaborative study
3. Nomenclature of systemic vasculitides: Proposal of an International Consensus Conference
4. The American College of Rheumatology 1990 criteria for the classification of Wegener's granulomatosis
5. Association of a syndrome resembling Wegener's granulomatosis with low surface expression of HLA class-I molecules
6. Intracranial Wegener's granulomatosis
7. Neuro-ophthalmic manifestations of meningocerebral inflammation from the limited form of Wegener's granulomatosis
8. Pregnancy in granulomatosis vasculitis
9. Wegener granulomatosis: CT and MR findings
10. Neutrophil-related cytokines and neutrophil products in bronchoalveolar lavage fluid of a patient with ANCA negative Wegener's granulomatosis
11. Limited Wegener's granulomatosis with central nervous system involvement and fatal outcome
12. An autopsy case of Wegener's granulomatosis with Pachymeningitis
13. Wegener's granulomatosis with unusual cavernous sinus and sella turciva extension
14. Pial involvement in Wegener's granulomatosis shown on MRI
15. Wegener granulomatosis: An analysis of 158 patients
16. Interdisciplinary approach to the care of patients with Wegener's granulomatosis
17. ANCA in systemic vasculitides, collagen vascular diseases, rheumatic disorders and inflammatory bowel diseases. ANCA associated vasculitides
18. Use of native and recombinant bactericidal/permeability-increasing protein (BPI) as antigens for detection of BPI-ANCA
19. Screening for ANCA: Is indirect immunofluorescence the method of choice?
20. Clinical evaluation of a capture ELISA for PR3-ANCA
21. New perspectives in pulmonary angiitis. From pulmonary angiitis and granulomatosis to ANCA associated vasculitis
22. Interpretation of head and neck biopsies in Wegener's granulomatosis. A pathologic study of 126 biopsies in 70 patients
23. Neurological complications of Wegener's granulomatosis
24. Case records of the Massachusetts general hospital. Weekly clinicopathological exercises
25. Wegener granulomatosis with meningeal involvement
26. The spectrum of neurologic involvement in Wegener's granulomatosis
27. Chronic meningitis with cranial neuropathies in Wegener's granulomatosis
28. Meningeal involvement in Wegener's granulomatosis
29. Communicating hydrocephalus secondary to diffuse meningeal spread of Wegener's granulomatosis: Case report and literature review
30. Granulomatosis de Wegener con afectatcion meningea: Correlacion clinicorradiologica
31. Pachymeningitis in Wegener's granulomatosis
32. Granulomatous compressive thoracic myelopathy as the initial manifestation of Wegener's granulomatosis
33. Wegener granulomatosis: MR imaging findings in brain and meninges
34. Case records of the Massachusetts General Hospital. Case 9-99
35. Neurological involvement in Wegener's granulomatosis: An analysis of 324 consecutive patients at the Mayo Clinic
36. Standardized neurological follow-up as part of the interdisciplinary approach to the care of 128 patients with Wegener's granulomatosis