Drug-Induced Lupus Erythematosus Clinical Presentation

Updated: Jul 10, 2018
  • Author: Catharine Lisa Kauffman, MD, FACP; Chief Editor: Dirk M Elston, MD  more...
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Presentation

History

Most patients with drug-induced lupus erythematosus (DILE) have 1 or more clinical symptoms of systemic lupus erythematous (SLE), such as arthralgias, lymphadenopathy, rash, and fever, and have no prior history of autoimmune disease. If a rash is present, it often manifests as a polycyclic, scaling, erythematous rash in sun-exposed areas.

Approximately 50% of patients have constitutional symptoms of fever, weight loss, and fatigue. As many as 90% of patients with DILE have severe but usually noninflammatory joint pain; however, synovitis may be present. Arthralgia is often the only clinical manifestation of DILE. As many as 50% of patients with DILE experience muscle pain (myalgia).

The drug will have been taken anywhere from 3 weeks to 2 years before the appearance of symptoms. Note that drug-associated exacerbations of SLE and typical drug hypersensitivities can also be temporally related to drug exposure. Clinical improvement is usually rapid when the drug is discontinued, whereas antinuclear antibodies and other serologic markers slowly decrease toward more normal levels.

Generally, the absence of central nervous system (CNS) and renal involvement is more suggestive of DILE than of SLE. High rates (ie, 5-10%) of glomerulonephritis, however, occur in hydralazine-induced DILE, and rare cases of death from renal involvement in DILE have been reported.

DISCLE differs from idiopathic SCLE by virtue of distinctive cutaneous features, particularly the common widespread presentation (82% vs 6%) and the frequent occurrence of malar rash (45% vs 6%), vasculitic manifestations (45% vs 3%), and bullous and erythema multiforme–like lesions (45% vs 1%). Visceral manifestations are not seen in DISCLE. [47]

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Physical Examination

Extracutaneous physical findings in DILE can include the following:

  • Splenomegaly

  • Hepatomegaly

  • Inflammation of the serous membranes that surround the lungs and pleural cavity walls (pleurisy)

  • Fever

  • Inflammation of the fibroserous membranes that cover the heart and the initial part of the great vessels (ie, pericarditis)

  • Cerebritis (rarely)

  • Episcleritis (rarely) [48]

  • Nephritis (rarely)

Skin findings are apparent in approximately 25% of all patients diagnosed with DILE. Note, however, that certain manifestations typical in persons with SLE are not usually observed in persons with DILE. Patients with DILE (unlike patients with SLE) typically do not have the following:

  • Mucosal ulcers

  • Hair loss (alopecia)

  • Circular (discoid) plaques

  • Photosensitivity (with the exception of thiazide-induced subacute lupuslike syndrome)

Compared with patients who have SLE, patients with DILE present with a higher prevalence of the following:

  • Purpura

  • Erythema nodosum (painful nodules, usually on the extremities)

  • Erythematous papules (typically on sun-exposed areas; see the image below)

    Erythematous macules and papules are seen on face, Erythematous macules and papules are seen on face, upper chest, and arms in photodistribution.

Lymphadenopathy or Raynaud phenomenon is present in approximately 35-50% of patients with SLE but in fewer than 25% of those with DILE.

More than 75% of patients with SLE also have cutaneous findings, versus an average of less than 25% in patients with DILE. However, in both SLE and DILE, approximately 75% of patients have arthritis or arthralgia.

Patients with DILE less commonly present as drug-induced SCLE, exhibiting skin findings that are analogous to those manifested in patients with SCLE, such as erythematous annular polycyclic or papulosquamous lesions, but malar rash, vasculitic manifestations, blisters, or targetoid lesions mimicking erythema multiforme may also be observed. [49]

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Complications

Although both SLE and DILE can affect multiple organ systems, including the skin, joints, kidneys, eyes, and CNS, complications of DILE that affect the kidneys and CNS are generally considered rare. In DILE induced by certain drugs, however, the rate of kidney involvement can be significant. For example, the rate of glomerulonephritis in hydralazine-induced DILE is 5-10%. In rare instances, patients may die of renal involvement.

Penicillamine is also more likely to be associated with renal disease. Rare cases of death associated with DILE have been reported as a direct result of renal complications. Thus, a renal biopsy may be necessary to rule out membranous proliferative and necrotizing glomerulonephritis. Hepatic necrosis is another potential serious complication of DILE and has been documented in cases of minocycline-induced DILE.

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