SLE is a disease of acute and chronic inflammation. Symptoms of SLE often mimic other, less serious illnesses. Fever is one of the most common clinical manifestations of SLE. Disease activity accounts for more than 66% of febrile episodes in patients with SLE. Antibodies with elevated titers that are characteristic of lupus disease activity rather than infection include anti-dsDNA and anti–ribosomal P antibodies, as well as reduced levels of complement and leukopenia.

Many of the clinical manifestations of SLE are a consequence of tissue damage from vasculopathy mediated by immune complexes. Other conditions (e.g., thrombocytopenia, antiphospholipid syndrome) are the direct effects of antibodies to cell surface molecules or serum components.

Manifestations of the disease range from a typical mild ill ness limited to a photosensitive facial rash and transient diffuse arthritis to life-threatening involvement of the CNS or renal, cardiac, or respiratory system (Fig. 1). In the early phases, it is often difficult to distinguish SLE from other systemic rheumatic disorders, such as progressive systemic sclerosis (PSS), polymyositis, primary Sjögren’s syndrome, primary Raynaud’s phenomenon, and rheumatoid arthritis. Polyarthritis and dermatitis are the most common clinical manifestations.

Fig1. Signs and symptoms of systemic lupus erythematosus (SLE).

The course of the disease is highly variable. It usually follows a chronic and irregular course, with periods of exacerbations and remissions. Clinical signs and symptoms can include fever, weight loss, malaise, arthralgia (joint pain) and arthritis (inflammation of the joints), and the characteristic erythematous, maculopapular (“butterfly”) rash over the bridge of the nose (Table 1). In addition, there is a tendency toward increased susceptibility to common and opportunistic infections. Multiple organ systems may be affected simultaneously.

Table1. Systemic Lupus Erythematosus Symptoms

The onset of lupus can be caused by sun exposure, resulting in sudden development of a rash and then possibly other symptoms. In some patients, an infection, even a cold, does not improve, and complications then arise. These complications may be the first signs of lupus. In some women, the first symptoms develop during pregnancy or soon after delivery.

Infection

About 20% of episodes of fever are caused by infections in patients with SLE. Infections are the leading cause of death in hospitalized patients. Infections can be caused by bacterial, viral, fungal, or parasitic pathogens. Immunosuppression produced by treatment (e.g., steroids) can interfere with host defense against opportunistic infections (e.g., Mycobacterium tuberculosis, Histoplasma capsulatum, Listeria monocytogenes).

Cutaneous Features

Approximately 20% to 25% of patients with SLE develop der mal disorders as the initial manifestation of the disease. As many as 65% of patients will develop a cutaneous abnormality during the course of the disease. The characteristic erythematous, maculopapular butterfly rash across the nose and upper cheeks is the cutaneous feature for which the disease is named—lupus erythematosus, the “red wolf” (Fig. 2). This rash may also be observed on the arms and trunk. Exposure to UV light will worsen erythematous, as well as other types of, cutaneous lesions.

Fig2. Facial rash over bridge of nose in patient with active SLE. (From Behrman R, Kliegman R, Jenson HB: Nelson’s textbook of pediatrics, ed 17, Philadelphia, 2004, Saunders.)

The spectrum of cutaneous abnormalities includes urticaria, angioedema, nonthrombocytopenic purpura associated with the presence of cryoglobulins, scale formation, and ulcerations of oral and genital mucous membranes. Although neither the collection of immunoglobulins and complement at the dermal epidermal junction nor the presence of specific antibody nuclear ribonucleoprotein (RNP), Sm, native DNA, and single stranded DNA appears to play a direct role in the pathogenesis of cutaneous lupus lesion, Ro (SS-A) and perhaps La (SS-B) antibodies may be prominent factors.

Diffuse or patchy alopecia is also a common cutaneous manifestation. Hair loss is caused by pustular lesions of the scalp and is usually related to the stress of the disease process. Although the cause of pustular lesions is unknown, these inflammatory infiltrates are characterized by the presence of predominantly Ia-positive (activated) T lymphocytes with both CD4+ and CD8+ phenotypes.

Approximately 2% to 3% of SLE patients demonstrate lupus panniculitis. This condition is characterized by tender or nontender subcutaneous nodules that sometimes ulcerate and discharge a yellowish lipid material. In addition, various nonspecific skin changes are observable secondary to vascular insults. Raynaud’s phenomenon is demonstrated by approximately one third of patients with SLE and appears to be increased in those who have antibodies to nuclear RNP in their serum.

The presence of lesions does not distinguish between the limited cutaneous (discoid lupus erythematosus) and cutaneous manifestations of SLE. The term discoid lupus is used to differentiate the benign dermatitis of cutaneous lupus from the cutaneous involvement of SLE. In discoid lupus, the round lesion is an erythematous inflammatory dermatosis. These lesions are primarily located in light-exposed areas of the skin.

Renal Characteristics

Complement-mediated injury to the renal system is a usual con sequence of the high levels of immune complexes in the blood that are deposited in tissues such as the kidneys. Renal disease progression is highly unpredictable. It may be acute, but more typically it progresses slowly. As the kidneys degenerate, the urinary sediment is typical of acute glomerulonephritis and later of chronic glomerulonephritis. Acute glomerulonephritis is characterized by the presence of erythrocytes, leukocytes, and granular and red blood cell (RBC) casts in urinary sediment. The presence of proteinuria may lead to nephrotic syndrome. If end stage renal disease (renal failure) occurs, it can be managed by dialysis or allograft transplantation.

The systemic necrotizing vasculitis of SLE involves small blood vessels and leads to renal involvement. The most common method of classification of the renal involvement of SLE is the World Health Organization (WHO) system, which is based on histopathologic criteria. The stages of renal disease range from the earliest and least severe form, class II, characterized by mesangial deposits of immunoglobulin and C3, to class V, the most severe form of involvement.

Lymphadenopathy

Enlargement of peripheral and axial lymph nodes and splenomegaly both occur in patients with SLE, but these conditions are usually transient. Patients with SLE may be at greater risk of developing lymphoma than the general population, especially those with secondary Sjögren’s syndrome.

Serositis

Serositis is an inflammation of the membrane consisting of mesothelium, a thin layer of connective tissue that lines enclosed body cavities. Mesothelium, a type of epithelium, is originally derived from the mesoderm lining the primitive embryonic body cavity. It becomes the covering of the serous membranes of the body surfaces such as the peritoneum, pleura, and pericardium. Inflammation of these serosal surfaces leads to sterile peritonitis, pleuritis, or pericarditis and is frequently accompanied by severe pain. Serositis is associated with an increased frequency of thrombophlebitis, which may lead to pulmonary embolization.

Cardiopulmonary Characteristics

Inflammation of the myocardium in patients with SLE can produce persistent tachycardia and, occasionally, intractable congestive heart failure. Ischemic disease or, more often, atherosclerotic coronary disease may occur. Patients with severe nephrosis or those treated with corticosteroids for a prolonged period are at an increased risk for developing atherosclerosis.

Pulmonary function studies reveal occult diffusion and obstructive abnormalities in a high proportion of SLE patients, but clinical problems secondary to pulmonary involvement are unusual. Massive hemoptysis may result from acute alveolar hemorrhage. This particular complication occurs in the absence of any detectable bleeding diathesis and is associated with a high rate of mortality.

Gastrointestinal Manifestations

Nonspecific gastrointestinal symptoms are relatively common in patients with SLE, but acute abdominal crises caused by visceral and peritoneal vasculitis are less common. Infarction and perforation of the bowel and viscera are associated with a high rate of mortality. Acute and chronic pancreatitis may also develop as a secondary complication of acute lupus or as a complication during therapy.

Musculoskeletal Features

A characteristic arthritis of SLE is a transient and peripheral polyarthritis with symmetric involvement of small and large joints. Chronic arthritis can result in disability and deformity in SLE patients. Rheumatoid-like hand deformities develop in about 10% of patients. Osteonecrosis develops in 25% of all SLE patients. Arthropathy of osteonecrosis, or avascular necro sis, is often initially detected in weight-bearing joints such as the hips and knees.

Neuropsychiatric Features

 In SLE, various neuropsychiatric manifestations develop secondary to involvement of the central and peripheral nervous systems. CNS involvement in SLE includes inflammation of the brain or intracranial blood vessels (vasculitis) and ischemic complications of vasculitis.

The most common abnormalities are disturbances of mental function, ranging from mild confusion, with memory deficiency and impairment of orientation and perception, to psychiatric disturbances such as hypomania, delirium, and schizophrenia. The most common manifestations are cognitive dysfunction, headache, seizures, and psychiatric conditions. Aseptic meningitis, stroke, encephalopathy, movement disorders, and myelopathy can be observed.

Seizures of the grand mal type may be the initial manifestation of SLE and may be present long before the multisystem disease develops. In addition, some patients may have epilepsy and severe headaches.

Antiribosomal P antibodies have been detected in patients with lupus suffer from psychosis or depression.

Late-Onset Lupus

 Lupus can occur at any age, in either gender, and in any race. The average age of onset is 59 years; the average age at diagnosis is 62 years. Late-onset lupus affects women eight times more often than men. Late-onset lupus is found primarily in whites, but it occurs in all races.

Symptoms in most cases are relatively mild, but symptoms of lupus in older people can mimic those of other diseases (e.g., rheumatoid arthritis, Sjögren’s syndrome, polymyalgia rheumatica). Distinguishing among these disorders can be difficult and may result in a delayed or missed diagnosis. Drug-induced lupus occurs more often in older people because they are more likely to have conditions (e.g., high blood pressure, heart dis ease) that require treatment that may cause the symptoms of lupus. Symptoms generally fade when the medication is discontinued. Patients with late-onset lupus have a good survival rate and rarely die of the disease or complications of therapy when treated conservatively.

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مواضيع ذات صلة

Treatment of Rheumatoid Arthritis

Juvenile Idiopathic Arthritis

Diagnostic Evaluation of Rheumatoid Arthritis

Immunologic Manifestations of Rheumatoid Arthritis

Signs and Symptoms of Rheumatoid Arthritis

Treatment of Systemic Lupus Erythematosus

Diagnostic Evaluation of Systemic Lupus Erythematosus

Immunologic Manifestations of Systemic Lupus Erythematosus

Different Forms of Lupus

Renal Disorders

Neuromuscular Disorders

Autoimmune Hematologic Disorders