Clinical features

As the name implies, systemic lupus is characterized by a very broad range of clinical manifestations. It is principally a disease affecting women during the child-bearing years. Invariably arthralgia or arthritis is involved (90%) but myalgia (50%) and tenosyov-itis (20%) are also common features in the musculoskeletal system. Involvement of the heart and lungs is also common with shortness of breath, pleurisy and pleural effusions being present in at least a quarter of the patients. Current pulmonary function tests will detect abnormalities in up to 85%. of lupus patients. Skin involvement is common in systemic lupus and up to 30% of patients have the so-called butterfly rash (Figure 1), essentially a vasculitis over the malar bones and bridge of the nose. A wide variety of other dermatological manifestations may be observed including alopecia, livedo reticularis, an erythematous masculopapular eruption and discoid lupus.

Involvement of the central nervous system (CNS)

Figure 1 The butterfly rash' seen in up to 40% of SLE patients. (See also color Plate 10.)

can manifest as an enormous range of features from single migraines or depression to frank psychosis, hemiplegia, or seizures. Recently a variety of CNS manifestations, together with a predisposition for venous or arterial thrombosis and spontaneous abortions has been recognized. These features are often linked to the presence of antiphospholipid antibodies.

Kidney involvement is the most important feature of lupus, especially as significant kidney damage is the major threat to long-term survival. Whilst proteinuria is found in some 60% of patients overall, probably less than one-third of patients actually have serious renal disease. This may take a variety of forms ranging from minimal or mesangial involvement to severe diffuse proliferative glomerulonephritis. Gastrointestinal involvement is relatively uncommon. Anorexia occurs at some time in up to 40% of the patients and vague abdominal pains occur in approximately one-third but these often settle spontaneously.

Nonspecific but very troublesome features of lupus include lymphadenopathy (which may on occasion be severe enough to warrant biopsy to exclude an accompanying malignancy), weight loss and fatigue. Fatigue is frequently a major problem for patients with lupus. It is important to recognize that with time the cumulative clinical features in adult and childhood onset cases varies, as is shown in Table 1. There are also differences amongst different ethnic-groups; for example, black patients are more likely to have renal and central nervous system problems.

Investigations Hematological abnormalities

A normochromic normocytic anemia is a frequent feature of lupus. Hemolytic anemia, as evidenced b> a positive Coombs' test, is rarely found but iron-deficiency anemia may be induced by the nonsteroidal anti-inflammatory drugs sometimes prescribed. Thrombocytopenia occurs in three main forms: acute, chronic or antecedent. In this last case patients may initially be diagnosed as having idiopathic thrombocytopenia but within months or years other features evolve making it clear that the thrombocytopenia was merely the first sign of SLF. Persistent and sometimes profound leukopenia (<4.0 X 1091"'), often accompanied by lymphopenia (<1.5 X 1091~'), is frequently found.

Table 1 Cumulative clinical features in adult and childhood onset groups (as percentages)

By 2 years By 5 years By 10 years

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