eMedicine Specialties > Neurology > Inflammatory and Demyelinating Diseases

Wegener Granulomatosis: Differential Diagnoses & Workup

Author: Thomas F Scott, MD, Professor, Program Director, Department of Neurology, Drexel University College of Medicine; Director, Allegheny MS Treatment Center
Contributor Information and Disclosures

Updated: Mar 17, 2010

Differential Diagnoses

Acute Disseminated Encephalomyelitis
Confusional States and Acute Memory Disorders
Acute Inflammatory Demyelinating Polyradiculoneuropathy
Diabetic Neuropathy
Amyotrophic Lateral Sclerosis
Frontal Lobe Epilepsy
Aseptic Meningitis
HIV-1 Associated CNS Complications (Overview)
Basilar Artery Thrombosis
HIV-1 Associated Neuromuscular Complications (Overview)
Brainstem Gliomas
Leptomeningeal Carcinomatosis
Cardioembolic Stroke
Metastatic Disease to the Brain
Cauda Equina and Conus Medullaris Syndromes
Metastatic Disease to the Spine and Related Structures
Cavernous Sinus Syndromes
Multiple Sclerosis
Cerebellar Hemorrhage
Neurosarcoidosis
Cerebral Aneurysms
Neurosyphilis
Cerebral Venous Thrombosis
Paraneoplastic Encephalomyelitis
Chronic Inflammatory Demyelinating Polyradiculoneuropathy
Rosai-Dorfman Disease
Complex Partial Seizures
Sarcoidosis and Neuropathy

Other Problems to Be Considered

Lymphoma and other multifocal (metastatic) neoplasms
Lymphomatoid granulomatosis (lung and cerebral involvement)
Other disorders causing inflammation or infection of various organs, including sarcoidosis
Carotid disease and stroke

Workup

Laboratory Studies

  • Laboratory diagnosis of Wegener's granulomatosis has been assisted greatly by emergence of testing for c-antineutrophil cytoplasmic antibody (ANCA) levels2,3 , which if elevated, are 97% specific for Wegener's granulomatosis. Testing for c-ANCA is 90% sensitive for the diagnosis when the presentation is classic, involving both upper and lower respiratory system and kidneys; sensitivity drops to 40% in limited Wegener's granulomatosis (ie, limited to only kidneys or respiratory system).
  • Along with other markers of inflammation such as C-reactive protein and erythrocyte sedimentation rate, c-ANCA can be used to monitor disease and response to therapy, but cannot reliably predict potential for disease relapse.
  • Other laboratory abnormalities often seen include anemias, thrombocytosis, hypergammaglobulinemia, and rarely cryoglobulins or circulating immune complexes; rheumatoid factor is found frequently.
  • In cases with myopathy, creatine kinase may be elevated.

Imaging Studies

  • Neuroimaging findings are nonspecific.
  • Areas of cerebritis may appear as supratentorial, irregularly enhancing, edematous lesions of any size, seen on both MRI and CT scan.
  • Meningeal involvement, seen as enhancement, is less common but is reported. Ischemic or hemorrhagic infarcts also may be suggested by neuroimaging.

Other Tests

Cerebrospinal fluid (CSF) analysis: Nonspecific CSF abnormalities are found commonly (eg, mild to moderate pleocytosis, mostly lymphocytes; elevated protein; elevated immunoglobulin G production) and may provide clues toward ruling in an inflammatory process (ie, Wegener's granulomatosis).

Procedures

In patients with neurologic involvement, electromyography (EMG), nerve conduction studies, CSF analysis, and MRI are the primary investigations used to localize the lesions. EMG and nerve conduction studies may reveal acute and/or chronic denervation in involved muscles, slowed nerve conductions, decreased amplitude of action potentials, and myopathy. A typical picture would involve few to several nerves in an asymmetrical pattern (ie, mononeuritis multiplex).

Histologic Findings

Definitive diagnosis is based on biopsy.4

  • A characteristic chest radiograph showing pulmonary infiltrates and nodules may suggest a high yield for positive confirmation by transbronchial biopsy or open lung biopsy (yield is highest with open biopsy).
  • Renal biopsy is rarely definitive, since the histopathology is generally less specific than that of the lungs.
  • Biopsy of the nasal mucosa shows more distinctive features of Wegener's granulomatosis and has a low rate of associated morbidity.
  • Biopsy of sural nerve may demonstrate vasculitis with noncaseating granulomas, affecting small arteries. Lesions may contain acute and chronic inflammatory features of vasculitis with focal areas of demyelination.
  • Autopsy studies in neurologic Wegener's granulomatosis are sparse and, although cerebral vasculitis sometimes has been assumed clinically in patients with infarctions (and seizures), tissues usually appear bland. Similarly, angiography may miss small-vessel vasculitis.

More on Wegener Granulomatosis

Overview: Wegener Granulomatosis
Differential Diagnoses & Workup: Wegener Granulomatosis
Treatment & Medication: Wegener Granulomatosis
Follow-up: Wegener Granulomatosis
References
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References

  1. Nishino H, Rubino FA, DeRemee RA, Swanson JW, Parisi JE. Neurological involvement in Wegener's granulomatosis: an analysis of 324 consecutive patients at the Mayo Clinic. Ann Neurol. Jan 1993;33(1):4-9. [Medline].

  2. Tervaert JW, Huitema MG, Hené RJ, Sluiter WJ, The TH, van der Hem GK, et al. Prevention of relapses in Wegener's granulomatosis by treatment based on antineutrophil cytoplasmic antibody titre. Lancet. Sep 22 1990;336(8717):709-11. [Medline].

  3. Spranger M, Schwab S, Meinck HM, Tischendorf M, Sis J, Breitbart A, et al. Meningeal involvement in Wegener's granulomatosis confirmed and monitored by positive circulating antineutrophil cytoplasm in cerebrospinal fluid. Neurology. Jan 1997;48(1):263-5. [Medline].

  4. Travis WD, Hoffman GS, Leavitt RY, Pass HI, Fauci AS. Surgical pathology of the lung in Wegener's granulomatosis. Review of 87 open lung biopsies from 67 patients. Am J Surg Pathol. Apr 1991;15(4):315-33. [Medline].

  5. Sharma A, Kumar S, Wanchu A, Lal V, Singh R, Gupta V. Successful treatment of hypertrophic pachymeningitis in refractory Wegener's granulomatosis with rituximab. Clin Rheumatol. Jan 2010;29(1):107-10. [Medline].

  6. Bachmeyer C, Cadranel JF, Demontis R. Rituximab is an alternative in a case of contra-indication of cyclophosphamide in Wegener's granulomatosis. Nephrol Dial Transplant. Jun 2005;20(6):1274. [Medline].

  7. Bellisai F, Morozzi G, Marcolongo R, Galeazzi M. Pregnancy in Wegener's granulomatosis: successful treatment with intravenous immunoglobulin. Clin Rheumatol. Dec 2004;23(6):533-5. [Medline].

  8. Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 9-1999. A 74-year-old woman with hydrocephalus and pleocytosis. N Engl J Med. Mar 25 1999;340(12):945-53. [Medline].

  9. Drachman DD. Neurological complications of Wegener's granulomatosis. Arch Neurol. 1963;8:144-55.

  10. Fauci AS, Haynes BF, Katz P, Wolff SM. Wegener's granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years. Ann Intern Med. Jan 1983;98(1):76-85. [Medline].

  11. Howell SB, Epstein WV. Circulating immunoglobulin complexes in Wegener's granulomatosis. Am J Med. Feb 1976;60(2):259-68. [Medline].

  12. Kelley WN, Harris ED, Ruddy S, Sledge CB. Textbook of Rheumotology. Vol 2. 5th ed. Philadelphia, Pa: WB Saunders; 1997.

  13. Kerr GS, Fleisher TA, Hallahan CW, Leavitt RY, Fauci AS, Hoffman GS. Limited prognostic value of changes in antineutrophil cytoplasmic antibody titer in patients with Wegener's granulomatosis. Arthritis Rheum. Mar 1993;36(3):365-71. [Medline].

  14. Moore PM. Immune-mediated vasculopathies of the central nervous system. In: Gilchrist J, ed. Prognosis in Neurology. Boston, Mass: Butterworth-Heinemann; 1998.

  15. Scott DG, Watts RA. Classification and epidemiology of systemic vasculitis. Br J Rheumatol. Oct 1994;33(10):897-9. [Medline].

  16. Wegener F. [Uber eine eigenartige rhinogene Granulomatose mit besonderer Beteilgung des Arteriensytems und der Nieren]. Beitrage der Pathologischen Anatomie. 1939;102:30-68.

  17. Zhang W, Zhou G, Shi Q, Zhang X, Zeng XF, Zhang FC. Clinical analysis of nervous system involvement in ANCA-associated systemic vasculitides. Clin Exp Rheumatol. Jan-Feb 2009;27(1 Suppl 52):S65-9. [Medline].

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Further Reading

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Keywords

Wegener's granulomatosis, granuloma, lymphomatoid granulomatosis, necrotizing inflammation, autoimmune disease, autoantibodies, neutrophil cytoplasmic antibodies, ANCA, c-ANCA, vasculitis, vasculitides, arteritis

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Contributor Information and Disclosures

Author

Thomas F Scott, MD, Professor, Program Director, Department of Neurology, Drexel University College of Medicine; Director, Allegheny MS Treatment Center
Thomas F Scott, MD is a member of the following medical societies: American Neurological Association, Consortium of Multiple Sclerosis Centers, and National Multiple Sclerosis Society Advisory Board, Allegheny Chapter
Disclosure: Nothing to disclose.

Medical Editor

Carmel Armon, MD, MSc, MHS, Professor of Neurology, Tufts University School of Medicine; Chief, Division of Neurology, Baystate Medical Center
Carmel Armon, MD, MSc, MHS is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Association of Neuromuscular and Electrodiagnostic Medicine, American Clinical Neurophysiology Society, American College of Physicians, American Epilepsy Society, American Medical Association, American Neurological Association, American Stroke Association, Massachusetts Medical Society, Movement Disorders Society, and Sigma Xi
Disclosure: Avanir Pharmaceuticals Consulting fee Consulting

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Florian P Thomas, MD, MA, PhD, Drmed, Director, Spinal Cord Injury Unit, Saint Louis Veterans Affairs Medical Center; Director, National MS Society Multiple Sclerosis Center; Director, Neuropathy Association Center of Excellence, Professor, Department of Neurology and Psychiatry, Associate Professor, Institute for Molecular Virology, and Department of Molecular Microbiology and Immunology, Saint Louis University
Florian P Thomas, MD, MA, PhD, Drmed is a member of the following medical societies: American Academy of Neurology, American Neurological Association, American Paraplegia Society, Consortium of Multiple Sclerosis Centers, and National Multiple Sclerosis Society
Disclosure: Nothing to disclose.

Chief Editor

B Mark Keegan, MD, FRCPC, Assistant Professor of Neurology, College of Medicine, Mayo Clinic; Master's Faculty, Mayo Graduate School; Consultant, Department of Neurology, Mayo Clinic, Rochester
B Mark Keegan, MD, FRCPC is a member of the following medical societies: American Academy of Neurology, American Medical Association, and Minnesota Medical Association
Disclosure: Neurology (Journal of the American Academy of Neurology)  Honoraria Section Editor

 
 
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