Autoimmune Diseases

Autoimmune inflammation of the inner ear may occur in isolation as an organ-specific process or may be part of a systemic autoimmune syndrome (20). Evidence is mounting to support the existence of an organ-specific cochlear autoimmune disease. The presence of antibodies to cochlear antigens, such as the 68 kD inner-ear antigen, as well as an impressive response to immunosuppressive medications, implicate autoimmunity as the underlying pathologic process in some patients with idiopathic...

Treatment

The treatment for AIED is high-dose oral or parenteral corticosteroids. Typically, treatment is initiated with oral prednisone at a dosing of 60 mg per day, as long as the patient does not have any contraindications to this therapy. A detailed list of possible side effects and complications of this therapy is given to the patient and includes hyperglycemia, insomnolence, hypertension, and mood disturbance, as well as sequelae of long-term usage such as moon facies, abdominal obesity, cataracts,...

Head and Neck Manifestations

Auricular chondritis is the most common presenting feature (40-50 ) and eventually occurs in 83 to 95 of patients. The inflammation of the external ear occurs suddenly and can be unilateral or bilateral. Pain and tenderness are frequently severe. The cartilaginous portion of the pinna becomes red violaceous and swollen while the noncartilaginous earlobe is spared. This helps to separate it from cellulitis. The auricular inflammation lasts a few days or can persist for weeks before subsiding...

Summary

GCA and PMR are common diseases in the elderly, particularly in patients of European descent. GCA has pleomorphic symptoms related to the systemic inflammation and compromise of arterial flow, by vasculitic lesions in particular, can lead to systemic as well as the characteristic head and neck manifestations. It must be distinguished from other systemic diseases including infections and cancer. With proper treatment, patients do well, although a substantial number of patients may suffer...

Systemic Lupus Erythematosus

Systemic lupus erythematosus is a chronic autoimmune inflammatory disorder caused by abnormal deposition of antigen-antibody complexes. Kaposi is credited with the first description of the disorder in 1872 and noted disturbed neurologic function in his report (92). Although any organ system in the body can be affected, patients most often have arthritis (88 ), butterfly rash (79 ), pericarditis (64 ), and kidney dysfunction (48 ). Neurologic sequelae of lupus are common and have been estimated...

Lyme Disease

Lyme disease, also termed Bannwarth's syndrome, is caused by an infection with the spirochete Borrelia burgdorferi transmitted by tick bites. It is a systemic disease affecting the skin, nervous system, heart, and joints. Symptoms may be mild and can include fever, fatigue, migratory arthritis, myalgia, headache, meningismus, lymphadenopathy, and skin lesions. Erythema migrans, a characteristic skin lesion found in Lyme disease, is defined as an enlarging, annular erythematous skin lesion seen...

TABLE 1 Diseases Associated with Relapsing Polychondritis

Autoimmune rheumatic diseases Rheumatoid arthritis Juvenile idiopathic arthritis Systemic lupus erythematosus Progressive systemic sclerosis Sj gren's syndrome Mixed connective tissue disease Ankylosing spondylitis Psoriatic arthritis Reiter's syndrome RS3PE Hematological disorders Myelodysplastic syndromes Hodgkin's disease MALT-type lymphoma Non-Hodgkin's lymphomas Acute lymphoblastic leukemia Pernicious anemia Endocrine diseases Diabetes mellitus type 1 Hashimoto's thyroiditis Graves'...

Rheumatoid Arthritis

RA, discussed at length in Chapter 1, is a symmetric autoimmune polyarticular arthritis. It also affects nonarticular structures. Patients may have vasculitis, pulmonary fibrosis, and inflammatory changes in ligaments, tendons, and fascia. Of patients with RA, 25 to 30 have cricoarytenoid arthritis, with symptoms of hoarseness, globus, odynophagia, and pain with speaking or coughing. If the joints become fixed in an adducted position, the airway can be severely obstructed. Cricoarytenoid

Juvenile Rheumatoid Arthritis

The classification system for juvenile inflammatory arthritis (JRA) divides this form of childhood arthritis into three categories systemic, pauciarticular (four or fewer affected joints), and polyarticular. Among children with JRA, the most important ocular disease is chronic, nongranulomatous anterior uveitis. It occurs in about 20 of children with pauciarticular JRA, 5 to 10 of children with polyarticular JRA, and rarely, if ever, in those with the systemic type. Young girls with the...

Disequilibrium

Any of the abovementioned disease processes may cause disequilibrium rather than vertigo if they do not involve the labyrinth itself but instead cause dysfunction with brainstem integration, cortical perception, or musculoskeletal function. In such cases, other cranial nerve or somatic deficits point toward the etiology of the problem. Common examples include loss of vibration sensation and joint proprioception in diabetic and hypertensive patients, poor muscle strength, and joint deformity in...

Complications And Prognosis

Ocular infection resulting from autoinoculation during acute herpetic gingivostomatosis or asymptomatic oropharyngeal infection is not an uncommon complication of HSV-l infection in children. The condition manifests as unilateral follicular conjunctivitis or as an acute herpetic keratoconjunctivitis with dendritic corneal ulcers, and can recur in as many as 25 of patients. HSV-1 infection can be associated with progressive scarring of the cornea and has been a leading infectious cause of...