Lupus is an autoimmune disease that varies greatly in severity, from mild cases requiring minimal intervention to those in which significant and potentially severe and permanent damage occurs to vital organs such as the lungs, heart, kidney, and brain.

The disease is characterized by "flares" of activity interspersed with periods of improvement or remission.

While lupus can affect anyone, it is more common among women. African-American women are three to four times more at risk than Caucasian women, and Latina and Asian women also develop lupus more frequently.

Timely and accurate diagnosis of SLE is important, as controlling the initial acute phase of the disease through treatment improves long-term prognosis.

Lupus can be categorized into three groups:

  • Discoid lupus erythematosus (DLE) is characterized by a skin rash only. It occurs in about 20% of patients with systemic lupus erythematosus.
    • The lesions are patchy, crusty, sharply defined skin plaques that may scar. Biopsy of a lesion will usually confirm the diagnosis.
    • Topical and intralesional corticosteroids are usually effective for localized lesions; antimalarial drugs may be needed for more generalized lesions.
    • DLE only rarely progresses to systemic lupus erythematosus.
  • Systemic lupus erythematosus (SLE, or lupus) is a chronic, inflammatory, multisystem disorder of the immune system. The course of the disease is unpredictable and individualized; no two patients are affected alike.
    • It usually develops in young women of childbearing years, but many men and children also develop lupus. African-Americans and Hispanics have a higher frequency of this disease than do Caucasians.
    • African-American women also tend to develop the disease at a younger age, develop more serious complications, and have a higher mortality rate from the disease than do Caucasian women.
    • SLE appears in the first-degree relatives of lupus patients more often than it does in the general population.
  • Drug-induced SLE develops after the use of certain drugs and has symptoms similar to those of SLE.
    • The characteristics of this syndrome are pleuropericardial inflammation, fever, rash, and arthritis.
    • Serologic changes also occur.
    • The clinical and serologic signs usually subside gradually after the offending drug is discontinued.
    • See the list of implicated drugs on side

"African-American women are three to four times more at risk than Caucasian women, and Latina and Asian women also develop lupus more frequently."

Drugs Implicated as Activators of SLE

While the causes of lupus are not known, some drugs have been associated with lupus. Drugs with proven association include:

  • Chlorpromazine
  • Hydralazine
  • Isoniazid
  • Methyldopa
  • Procainamide

Drugs with possible association include:

  • Beta blockers (e.g., acebutolol, atenolol, labetalol, metoprotolol, oxprenolol, pindolol, practolol, and propranolol)
  • Captopril
  • Carbamazine
  • Cimetidine
  • Diphenylhydantoin (phenytoin)
  • Ethosuximide
  • Methimazole
  • Penicillamine
  • Phenazine
  • Quinidine

Symptomatology

The general manifestations of SLŒE include fatigue, fever, and psychological and emotional effects. Specific manifestations include:

  • Dermatologic: Butterfly rash, photosensitivity, DLE, subcutaneous LE, mucosal ulcers, alopecia, pain and discomfort, pruritus, bruising.
  • Musculoskeletal: Arthralgias, arthritis, other joint complications.
  • Hematologic: Anemia, decreased WBC count, thrombocytopenia, lupus anticoagulants, false-positive VDRL, elevated ESR.
  • Cardiopulmonary: Pericarditis, myocarditis, myocardial infarction, vasculitis, pleurisy, valvular heart disease.
  • Renal: Asymptomatic microscopic renal involvement, renal failure, fluid and electrolyte imbalance, urinary tract infection.
  • Central Nervous System (CNS): General CNS symptomology, cranial neuropathies, cognitive impairment, mental changes, seizures.
  • Gastrointestinal: Anorexia, ascites, pancreatitis, mesenteric or intestinal vasculitis.
  • Ophthalmologic: Eyelid problems, conjunctivitis, cytoid bodies, dry eyes, glaucoma, cataracts, retinal pigmentation.

Adapted primarily from Lupus: A Patient Care Guide for Nurses and Other Health Professionals (NationŒal Institute of Arthritis and Musculoskeletal and Skin Diseases, NIH)

Generally, four of the eleven criteria below proposed by the American College of Rheumatology should be experienced by a patient before a classification of SLE is made, though these criteria should not be relied upon solely for diagnosis or for the treatment of specific lupus symptoms:

  • Characteristic rash across the cheek
  • Discoid lesion rash
  • Photosensitivity
  • Oral ulcers
  • Arthritis
  • Inflammation of membranes in the lungs, the heart or the abdomen
  • Evidence of kidney disease
  • Evidence of severe neurologic disease
  • Blood disorders, including low red and white blood cell and platelet counts
  • Immunologic abnormalities
  • Positive antinuclear antibody (ANA)

This information is provided by the Environmental Health within the Department of Public Health.