Complications
Typically secondary to chronic disease and improves with control of disease activity.
Typically due to lymphopenia, and to a lesser extent neutropenia.
Frequently seen in SLE, but other causes should be excluded.
Long-term corticosteroid therapy is a recognised cause of posterior subcapsular cataract.
However, there is no evidence to suggest that patients with SLE are at higher risk.
Guidelines for prevention and treatment of corticosteroid-induced osteoporosis should be followed.
High-dose corticosteroid therapy can result in insulin resistance and type 2 diabetes mellitus.
This is well documented. A male patient requiring cyclophosphamide should be counselled prior to starting therapy and discussions raised regarding sperm storage prior to treatment commencing.
SLE itself is associated with an increased frequency of sperm abnormality and reduced testicular volume. Post-pubertal cyclophosphamide is a major contributor to reduced fertility in male patients with SLE.[167]
May occur as part of serositis. Treated as per patients with serositis, with additional cardiovascular specialist input.
May occur as part of cardiopulmonary manifestations. Requires cardiovascular specialist input. Other causes need to be excluded.
May occur as part of cardiopulmonary manifestations. Requires cardiovascular specialist input. Other causes need to be excluded.
Can be either unilateral or bilateral. It is more common than pericarditis.
Pleural effusions in SLE are usually unilateral and generally exudative. Other causes of a pleural effusion should be excluded.
Immunosuppressives such as azathioprine and cyclophosphamide can increase the risk of malignancy in patients with SLE.[160]
Patients should be counselled on the risk before commencing this therapy and specialist oncology input sought if concerns for malignancy exist after therapy.
Corticosteroid therapy is associated with an increased risk of avascular necrosis of bone, most commonly in the femoral head but described at other sites.
An association with antiphospholipid antibody syndrome is also recognised.
Although not a common complication, can be very severe.
A rare viral brain disease that is a potential adverse effect of some disease modifying drugs, particularly rituximab.
A literature review suggests an increased risk of PML in patients with SLE compared with the general population, potentially due to immunosuppression, underlying disease, treatments to manage disease, or some combination of these factors.[172]
Standard treatment consists of trigger avoidance and lifestyle changes. Alternatives for refractory disease include oral or topical vasodilators (e.g., calcium-channel blockers, topical nitroglycerin). Severe disease may require intravascular prostacyclin or sympathectomy.
The phosphodiesterase-5 inhibitor sildenafil may be used for digital necrosis in scleroderma overlap syndrome.
Corticosteroids, methotrexate, cyclophosphamide, and azathioprine can all increase the risk of infection via myelosuppression.
Includes common bacterial infections as well as opportunistic infections: fungi, parasites, mycobacteria, and protozoa.
Infection should be treated with therapy as per local guidelines and the immunosuppressant withheld at that time.
Treatment with either non-selective NSAIDs or cyclo-oxygenase-2 (COX-2) inhibitors increases the risk of renal failure.[161] This therapy should not be commenced in patients with pre-existing renal impairment.
If renal failure develops, the therapy should be discontinued and appropriate additional treatment for the renal impairment undertaken.
Patients who require an anti-inflammatory and who are at high risk of gastrointestinal ulceration should be given a cyclo-oxygenase-2 (COX-2) inhibitor (e.g., celecoxib) if they are at low cardiovascular risk.
Depression is more commonly reported in patients with SLE than in healthy controls.[170]
Treatment approaches should be similar to those offered to individuals with depression. For depression with psychomotor symptoms, referral for specialist advice should be sought.
Results in heart failure and eventually death. There may be underlying parenchymal involvement due to interstitial lung disease.
Lupus pneumonitis may present with shortness of breath, cough, and fever.
Atypical infections such as Salmonella can occur. Consideration should be given to this complication in a patient with a single swollen joint; systemic symptoms may be masked by corticosteroid therapy.
Synovial fluid culture may be negative, especially if prior antibiotics have been prescribed.
Patients on cyclophosphamide are at risk of developing uro-epithelial toxicity as well as bladder tumours. The risk can be reduced by concomitant administration of mesna (uroprotective agent) and a fluid load.
Has been associated with SLE.
Abdominal pain, vomiting, and diarrhoea may be caused by lupus peritonitis, but other causes of an acute abdomen should be excluded. Although rare, lupus peritonitis may mimic appendicitis.
SLE-associated pancreatitis is rare, with an estimated annual incidence of <1 per 1000 lupus patients.[162] The mortality is thought to be higher in lupus than non-lupus patients. Standard assessment of the patient and treatment should be followed.
Azathioprine is a recognised cause of pancreatitis. In the majority of cases in patients with SLE, pancreatitis does not seem to be related to steroid or azathioprine therapy, but rather to the disease itself.[163]
Patients with SLE and antiphospholipid antibody syndrome are at higher risk of arterial thrombosis. Guidelines for treatment should be followed.[164]
There is accumulating evidence that patients with established disease are more likely to develop accelerated atherosclerotic vascular disease; it is unclear whether this is disease-related or secondary to corticosteroid therapy.
In a patient with venous thrombosis, tests for antiphospholipid antibody syndrome should be sought and treatment guidelines should be followed.[164]
This is a rare but potentially disabling complication. Possible predisposing factors include trauma, corticosteroid therapy, and local inflammation.
Patients with antiphospholipid syndrome are more likely to have valvular heart disease.
Requires emergency treatment, usually intravenous cyclophosphamide plus corticosteroids. Warfarin is used in the presence of antiphospholipid antibodies and plasmapheresis may be used in this setting as well.
Typically, presents with haemoptysis and anaemia. Chest x-ray demonstrates diffuse or focal patchy alveolar infiltrates. Requires specialist management.
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