Diagnosis

Lupus is a multisystem disease and patients can present in vastly different ways. Classification criteria have been developed. Published by The American College of Rheumatology, the criteria combine clinical signs and symptoms with abnormalities detected in blood tests (see Table 1).

There are several kinds of lupus.

Systemic lupus erythematosus (SLE) is the form of lupus that most people are referring to when they say "lupus." It is designated as systemic because of the involvement of multiple organs. The diagnostic criteria in the preceding chart assume systemic involvement.

Cutaneous lupus involves primarily the skin. It may be characterized by the discoid rash or photosensitivity.

Drug-induced lupus is caused by medications. Symptoms are similar to SLE, and resolve when the drug is stopped.

Neonatal lupus is a rare disease in newborn babies of women with SLE. Symptoms include skin rash, liver problems, and low blood count. These symptoms go away gradually over several months. In rare cases, the neonates may have congenital heart block.

Diagnosis of lupus can be difficult, because lupus has many symptoms and biomarkers in common with other diseases, and the signs and symptoms evolve over time. Patients see an average of 3 physicians over 4 years before lupus is correctly diagnosed.
 
Fibromyalgia symptoms include joint and muscle aches, and fatigue. Patients are occasionally antinuclear antibody (ANA) positive.

Sjögren syndrome can also present with arthritis, fatigue, and a positive ANA. A distinguishing feature are the classic symptoms of dry eyes and dry mouth.

Dermatomyositis causes rashes that may be mistaken for lupus, even with a skin biopsy.

Idiopathic or thrombotic thrombocytopenic purpura, primary antiphospholipid syndrome, and hematologic neoplasms are hematologic problems that can cause serologic changes, a positive ANA, and other manifestations seen in lupus.

Treatment

Successful therapy of lupus depends upon treating both symptoms and the underlying inflammation, which will most likely require pharmacological and nonpharmacological therapies. Below are some suggested treatment guidelines.

The pharmacological management of lupus generally revolves around four main classes of drugs:

• Nonsteroidal anti-inflammatory drugs (NSAIDS)
• Antimalarials
• Corticosteroids
• Cytotoxic drugs

Often used in combination, the broad indication for the use of these drugs is summarized in Table 2.
 

Methotrexate, a disease-modifying antirheumatic drug, may be useful in some patients. Other treatments may include hormonal therapies such as dehydroepiandrosterone (DHEA) and intravenous immunoglobulin, which may be useful for controlling lupus when other treatments haven’t worked.

Guidelines for Referral and Management of Systemic Lupus Erythematosus in Adults