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Appendix 1 0-A:
Disorders of Altered Immunity
Systemic Lupus Erythematosus
Systemic lupus erythematosus (SLE) is a chronic, multisystem autoimmune disease with strong genetic predisposition. There is also evidence suggesting risk factors that can trigger the onset of this disease, slIch as physical or emotional stress, pregnancy, sulfa antibiotics, and
environmental factors, such as sun exposure. Women who are black,
Asian, and Native American, ages 20-40 years, are more susceptible
than men in acquiring this disease. SLE is characterized by a systemic,
remitting and relapsing clinical presentarion.I--4
Diagnosis of SLE is confirmed if a patient has four of the following
1 1 manifestations of SLE: malar rash, discoid rash (individual round
lesions), photosensitivity, oral ulcers, arthricis, serositis, renal disorder, neurologic disorder, hematologic disorder, immunologic disorder, and the presence of antinuclear antibodies.3
Prognosis for l O-year survival after diagnosis is 90%. The most
common cause of death in SLE is renal failure, and the second most
common is CNS dysfunction.'-3
Clinical presentation of SLE may include the following' .... :
• Stiffness and pain in hands, feet, and large joints
• Red, warm, and tender joints
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ACtrrE CARE HANDBOOK FOR I'HYSICAL TI-IERAI'ISTS
• Butterfly (malar) rash on face
• Fever, fatigue, anorexia, and weight loss
• Raynaud's phenomenon
• Headache, seizures, organic brain syndrome
• Hemolytic anemia, thrombocytopenia, leukopenia
• Renal disease or failure
Management of SLE may consist of nonsteroidal ami-inflammatory drugs, hydroxychloroquine and other amimalarial agems, glucocorticoids, immunosuppressive agents (cyclophosphamide), dialysis, and renal transplantation in severe cases,I.2.4.5
Sarcoidosis
Sarcoidosis is a systemic disorder that primarily affects women and
nonwhite adults in the third decade of their life. The definitive etiology is unknown, although an autoimmune process that is environmemally triggered is the generally agreed on hypothesis. Sarcoidosis may have periods of progression and remission. [,5-7
The lungs are the primary organs affected by sarcoidosis, with dyspnea, dry cough, and chest pain being common symptoms. Pulmonary involvement can be staged according to radiographic evidence"" :
Stage O-no radiographic abnormalities
Stage I-bilateral hilar lymphadenopathy
Stage II-bilateral hilar adenopathy and parenchymal infiltration
Stage Ill-parenchymal infiltration without hilar adenopathy
Stage IV-advanced fibrosis with evidence of honey-combing, hilar
retraction, bullae, cysts, and emphysema
Orher systems of the body can be affected as well, including the
following:
• Eye and skin lesions
• Fever, fatigue, and weight loss
• Hepatosplenomegaly
INFEcnouS DISEASES API)ENDIX IO-A
647
• Hypercalcemia, anemia, and leukopenia
• Arthralgia, arthritis
Management of sarcoidosis usually consists of corticosteroid therapy, ranging from ropical ro oral administration. Additionally, cyrotoxic agents (methotrexate and azathioprine), antimalarial agents (chloroquine and hydroxychloroquine), and nonsteroidal anti-inflammatory drugs may also be used. In severe cases of pulmonary disease, single and double lung transplantation may be performed. 1,5-7
Amyloidosis
Amyloidosis is a very rare metabolic disorder characterized by deposition of amyloid ( a rype of protein) in various tissues and organs.
Amyloidosis is classified according ro protein rype and tissue distribution. The etiology of amyloidosis is not fully understood; however, relarion ro a disordered reticuloendothelial system and abnormal
immunoglobulin synthesis has been shown.
Clinical signs and symproms are representative of the affected