The evidence supporting the use of long-term IVIG therapy to induce remissions in SLE is unimpressive. The single extant clinical study used an open-label design with 12 patients, no placebo control, and questionable statistical methodology. The lack of definitive clinical studies, however, is tempered by case reports documenting significant improvement and apparent lack of toxicity in patients with SLE treated with IVIG. Standard first-line therapy of active SLE should consist of nonsteroidal antiinflammatory drugs, followed by low-dose corticosteroids and antimalarial compounds. Second-line therapeutic alternatives are the cytotoxic agents methotrexate, azathioprine, or cyclophosphamide. IVIG's primary advantage over these conventional therapies is that, unlike immunosuppressant and cytotoxic drugs, IVIG has not been reported to increase the risk of opportunistic infections. Additionally, IVIG obviates the ovarian/testicular toxicity, hemorrhagic cystitis, and carcinogenicity caused by cyclophosphamide. However, IVIG therapy is extremely expensive. (Approximate average wholesale price is $1800 per dose for a 70-kg patient). Thus, IVIG treatment consisting of 0.4 g/kg/d for 5 consecutive days on a monthly basis should be reserved for patients with active SLE resistant to the first- and second-line therapies. While IVIG-induced acute renal failure is considered rare, the serious nature of this adverse event warrants close monitoring of blood urea nitrogen and serum creatinine during and several days after treatment. Preexisting renal dysfunction should be considered a relative contradiction. Further double-blind multicenter trials are warranted to determine the long-term safety, efficacy, and cost/benefit ratio of using IVIG in SLE.

Systemic lupus erythematosus is a chronic, multisystem, inflammatory disorder of autoimmune etiology, occurring predominantly in young women. Common manifestations may include arthralgias and arthritis; malar and other skin rashes; pleuritis or pericarditis; renal or CNS involvement; and hematologic cytopenias. Diagnosis requires clinical and serologic criteria. Treatment of severe ongoing active disease requires corticosteroids, often hydroxychloroquine, and sometimes immunosuppressants.

Of all cases, 70 to 90% occur in women (usually of child-bearing age). SLE is more common among blacks and Asians than whites. It can affect patients of any age, including neonates. Increased awareness of mild forms has resulted in a worldwide rise in reported cases. In some countries, the prevalence of SLE rivals that of RA. SLE may be precipitated by currently unknown environmental triggers that cause autoimmune reactions in genetically predisposed people. Some drugs (eg, hydralazine Some Trade Names
APRESOLINE
 

Symptoms and Signs

Clinical findings vary greatly. SLE may develop abruptly with fever or insidiously over months or years with episodes of arthralgias and malaise. Vascular headaches, epilepsy, or psychoses may be initial findings. Manifestations referable to any organ system may appear. Periodic exacerbations (flares) may occur.

Joint manifestations: Joint symptoms, ranging from intermittent arthralgias to acute polyarthritis, occur in about 90% of patients and may precede other manifestations by years. Most lupus polyarthritis is nondestructive and non deforming. However, in long-standing disease, deformities without bone erosions may develop (eg, the metacarpophalangeal and interphalangeal joints may rarely develop ulnar drift or swan-neck deformities without bony or cartilaginous erosions [Jaccoud's arthritis]).

Skin and mucous membrane manifestations: Skin lesions include malar butterfly erythema (flat or raised) that generally spares the nasolabial folds. The absence of papules and pustules helps distinguish this from rosacea. A variety of other erythematous, firm, maculopapular lesions can occur elsewhere, including exposed areas of the face and neck, upper chest, and elbows. Skin blistering and ulceration are rare, although recurrent ulcers on mucous membranes (particularly the central portion of the hard palate near the junction of the hard and soft palate, the buccal and gum mucosa, and the anterior nasal septum) are common. Generalized or focal alopecia is common during active phases of SLE. Panniculitis can cause subcutaneous nodular lesions. Vasculitic skin lesions may include mottled erythema on the palms and fingers, periungual erythema, nail-fold infarcts, urticaria, and palpable purpura. Petechiae may develop secondary to thrombocytopenia. Photosensitivity occurs in most patients.

Cardiopulmonary manifestations: Cardiopulmonary symptoms commonly include recurrent pleurisy, with or without pleural effusion. Pneumonitis is rare, although minor impairments in pulmonary function are common. Severe pulmonary hemorrhage occasionally occurs. Prognosis has traditionally been poor but seems to be improving, possibly because of better early, aggressive, critical care. Other complications include pulmonary emboli, pulmonary hypertension, and shrinking lung syndrome. Cardiac complications include pericarditis (most commonly), pericardial effusion, and myocarditis. Serious, rare complications are coronary artery vasculitis, valvular involvement, and Libman-Sacks endocarditis. Accelerated atherosclerosis is an increasing cause of morbidity and mortality. Congenital heart block can develop in neonates.

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