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Disseminated Lupus Erythematosus)
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 , procainamide , isoniazid ) cause a reversible lupus-like syndrome.
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 nondeforming. 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: (see also Autoimmune Rheumatic Disorders: Variant Forms of Lupus) 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.
Adenopathy and splenic manifestations: Generalized adenopathy is common, particularly among children, young adults, and blacks. Splenomegaly occurs in 10% of patients. The spleen may develop periarterial fibrosis.
Neurologic manifestations: Neurologic symptoms can result from involvement of any part of the central or peripheral nervous system or meninges. Mild cognitive impairment is common. There may also be headaches, personality changes, ischemic stroke, subarachnoid hemorrhage, seizures, psychoses, organic brain syndrome, aseptic meningitis, peripheral neuropathies, transverse myelitis, or cerebellar dysfunction.
Renal manifestations: Renal involvement can develop at any time and may be the only manifestation of SLE. It may be benign and asymptomatic or progressive and fatal. Renal lesions can range in severity from a focal, usually benign, glomerulitis to a diffuse, potentially fatal, membranoproliferative glomerulonephritis. Common manifestations include proteinuria (most often), an abnormal urinary sediment manifested by RBC casts and leukocytes, hypertension, and edema.
Obstetric manifestations: Obstetric manifestations include early and late fetal loss. In patients with antiphospholipid antibodies, the risk of recurrent miscarriages is increased. Pregnancy can be successful (see Pregnancy Complicated by Disease: Systemic lupus erythematosus), particularly after 6 to 12 mo of remission, but SLE flares are common during pregnancy. Pregnancy should be timed when disease is in remission. During pregnancy, the patient should be monitored closely for any disease flare or thrombotic events by a multidisciplinary team that includes a rheumatologist, an obstetrician who specializes in high-risk pregnancies, and a hematologist.
Hematologic manifestations: Hematologic manifestations include anemia (often autoimmune hemolytic), leukopenia (usually lymphopenia, with < 1500 cells/μL,), and thrombocytopenia (sometimes life-threatening autoimmune thrombocytopenia). Recurrent arterial or venous thrombosis, thrombocytopenia, and a high probability of obstetric complications occur in patients with antiphospholipid antibodies. Thromboses probably account for many of the complications of SLE, including obstetric complications.
GI manifestations: GI manifestations can result from bowel vasculitis or impaired bowel motility. In addition, pancreatitis can result from SLE or from its treatment with corticosteroids or azathioprine . Manifestations may include abdominal pain from serositis, nausea, vomiting, manifestations of bowel perforation, and pseudo-obstruction. SLE rarely causes parenchymal liver disease.
Diagnosis |
