HSC Section 3 - Trauma, Critical Care and Sleep Medicine

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Granulomatosis with polyangiitis (GPA), Wegener granulomatosis 42 is a multisystem inflammatory disease, characterized by necrotizing vasculitis of vessels (small and me- diumsize) withmultinucleatedgiant cells andmicroabscess formation. Itmost commonly affects the lungs and kidneys. FP is thought to be secondary to vasculitis of the vasa nerv- osum or a temporal bone abscess adjacent to the facial nerve. Antimyeloperoxidase (MPO) and proteinase 3 antineutrophil cytoplasmic antibodies (PR3 ANCA) testing aids the diagnosis. Treatment includes high-dose steroids and/or Rituxan. Melkersson-Rosenthal syndrome (MRS) is a rare neuromucocutaneous granuloma- tous disorder characterized by the triad of orofacial edema (most common), facial palsy, and lingua plicata (fissured tongue). 43–45 Typically, the symptoms present at different times making the diagnosis more difficult. The etiology is unknown. Cerebro- spinal fluid (CSF) examination may show elevated protein levels. 45 A biopsy of involved tissue may reveal fibrosis with nonnecrotizing perivascular and perilymphatic granulomatous infiltrates with intralymphatic involvement. Steroids are a mainstay of medical therapy. Also, prophylactic FN decompression for patients with appropriate electrophysiological changes may be considered to prevent recurrence. Systemic Lupus Erythematosus Systemic lupus erythematosus (SLE) is a chronic autoimmune disorder that can affect any organ system. A classic presentation includes fever, malar rash, and joint pain in a young woman. Although rare, SLE and FP has been described in the literature. 46 The- ories regarding the pathophysiology of FP include immune dysregulation with activation of latent virus, presence of autoimmune antibodies to nerve antigen, or thrombotic vas- culopathy. 47 The diagnosis is based on clinical and laboratory studies, such as antinu- clear antibodies (ANA). Medical therapy includes nonsteroidal anti-inflammatory drugs and immune suppressants, such as corticosteroids, azathioprine, cyclophosphamide, and cyclosporine. Given the unknown etiology of FP, antivirals are also given. Behc¸et Disease Behc¸ et disease is a relapsing autoimmune vasculitis of unknown etiology and involves any organ system: ocular inflammation, oral ulceration, genital ulceration, arthropathy, cutaneous lesions, vasculitis, and also neural tissues. 48 Although rare, patients may also present with acute or recurrent FP. 49 The diagnosis is clinical; however, diag- nostic testing may include ESR, leukocyte count, and immunoglobulin evaluation. Laboratory tests for vasculitis include ANCA and PR3 to predict relapse. Imaging may be determined by the clinical picture. Treatment involves high-dose steroids and other immunosuppressant medications. Sjo¨ gren Disease Sjo¨ gren syndrome is an autoimmune disease characterized by dry eyes, dry mouth, fatigue, and arthropathy. 50 It can be associated with unilateral or bilateral FP and also affect any organ system. 51,52 The diagnosis is based on abnormal immunologic laboratory tests, such as serum anti-SSA antibodies (anti-Sjo¨ gren’s-syndrome-related antigen A) or the presence of lymphocytic sialadenitis on biopsy of labial salivary glands. Rheumatoid factor may also be positive. 50 Treatment for FP includes typical BP management as well as immunomodulators per rheumatology recommendations.

METABOLIC

Diabetes mellitus, a metabolic disorder with dysregulation of glucose metabolism may result in vasculopathy and peripheral neuropathy. Diabetes is a risk factor for worse

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