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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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646

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