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Granulomatous Diseases

Neurosarcoidosis

  • Neurosarcoidosis is an inflammatory granulomatous disease that affects both the central and peripheral nervous system.
  • Neurosarcoidosis accounts for approximately 5 per cent of cases of sarcoidosis[1].
  • The etiology of sarcoidosis remains uncertain[1].
  • Sarcoidosis is characterized by the development and presence of non-caseating/non-necrotizing granulomas in the affected organ or tissue[1].
  • Because neurosarcoidosis manifests in many different ways, it is sometimes difficult to properly diagnose.
  • Diagnosis is also difficult since the gold standard is by tissue-proven brain biopsy, which is difficult to obtain[1].
  • Weakness of the facial muscles on one side of the face (Bell’s palsy) is a common symptom.  The optic and auditory nerves can also become inflamed, leading to vision and auditory deficits. Headaches, seizures, memory loss, hallucinations, and neuropsychiatric symptoms are also common[2].
  • Treatment strategies have not been rigorously evaluated but corticosteroids are considered the drug of choice. Other effective immunosuppressant agents include cyclophosphamide, mycophenolate mofetil, and infliximab[1].

References:

  1. Vargas DL, Stern BJ. Neurosarcoidosis: diagnosis and management. Semin Respir Crit Care Med, 2010. 31(4): p. 419-27.
  2. "Neurosarcoidosis Information Page." National Institute of Neurological Disorders and Stroke (NINDS). N.p., n.d. Web. 26 June 2012. <http://www.ninds.nihgov/disorders/neurosarcoidosis/neurosarcoidosis.htm>.

ANCA-Associated Vasculitis

  • Anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis is a common primary small-vessel vasculitis, characterized by necrotizing inflammation of small vessels and the presence in the patient's serum of ANCA antibodies[2].
  • ANCA antibodies are a group of antibodies that are directed against antigens in the cytoplasm of neutrophil granulocytes (the most common type of white blood cell) and monocytes. The two main target antigens for ANCA are proteinase 3 (PR3) and myeloperoxidase (MPO)[2].
  • These antibodies are known to be associated with microscopic polyangiitis, granulomatosis with polyangiitis (Wegener's), and Eosinophilic Granulomatosis with Polyangiitis (Churg–Strauss syndrome)[3].
  • The contributions of genetic, epigenetic and environmental factors to etiology and pathogenesis are still being investigated[1].
  • When the ANCAs attack the neutrophils, they cause the immune system to target the walls of small blood vessels in different tissues and organs of the body, causing vasculitis and inflammation.
  • Symptoms differ depending on which tissue is inflamed.
  • Diagnosis of small vessel vasculitis should rely on both clinical findings and histopathological examination of the organ involved[3].
  • Cyclophosphamide or rituximab combined with glucocorticoids is the standard therapy for remission induction in generalized ANCA-associated vasculitis[3].

References:

  1. Savage CO. Pathogenesis of anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis. Clin Exp Immunol, 2011. 164(Suppl 1): p. 23-6.
  2. Kallenberg CG. Pathogenesis of ANCA-associated vasculitides. Ann Rheum Dis, 2011. 70 (Suppl 1): p. 59-63.
  3. Pettersson T, Karjalainen A. Diagnosis and management of small vessel vasculitides. Duodecim, 2010. 126(12): p. 1496-507.

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