Systemic lupus erythematosus (SLE): Treatment شرح بالعربي : الذئبة الحمراءالعلاج

Systemic lupus erythematosus (SLE): treatment شرح بالعربي : الذئبة الحمراءالعلاج
• Systemic lupus erythem...
Lecture outline
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-investigations
-Diagnosis
-Management
-Prognosis
Investigations
Blood
CBC:
may show a leucopenia, lymphopenia and/or thrombocytopenia.
Anaemia of chronic disease or autoimmune
haemolytic anaemia also occurs.
The ESR is raised in proportion to the disease activity.
In contrast, the CRP is usually normal but
may be high when the patient has lupus pleuritis or peritonitis,arthritis or a coexistent infection.
Blood
Urea and creatinine only rise when renal disease is advanced.
Low serum albumin or high urine albumin/creatinine ratio are earlier indicators of lupus nephritis.
Blood
Autoantibodies the most significant are ANA, anti-dsDNA, anti-Ro, anti-Sm and anti-La .
Antiphospholipid antibodies are
present in 25-40% of cases but not all of these patients develop antiphospholipid syndrome .
Blood
Serum complement
C3 and C4 levels are often reduced during
active disease.
The combination of high ESR, high anti-dsDNA
and low C3 may herald a flare of disease.
All these markers tend to return towards normal as the flare improves,
but in some patients, anti-dsDNA levels will remain high even during clinical remission. 
Diagnosis
Active SLE
1- Serum levels of C3 may be low.
2- A raised ESR, leucopenia and lymphopenia are typical of active SLE, and this may be accompanied by anaemia, haemolytic anaemia and thrombocytopenia.
NB:CRP is often normal in active SLE and presence of an elevated CRP in SLE raises the possibility of either serositis, or
coexisting infection.
Management
The therapeutic goals
1-Educate the patient about the nature of the illness.
2- Control symptoms .
3-Prevent organ damage and maintain normal function.
4-Patients should be advised to avoid sun and ultraviolet light exposure and to employ sun blocks (sun protection factor 25-50)
Mild to moderate disease
1-Patients with mild disease restricted to skin and joints can sometimes be managed with analgesics, NSAIDs and hydroxylchloroquine (HCQ).
2-Glucocorticoids may be also necessary (prednisolone 5-20 mg/day), often in combination with immunosuppressants such as Methotrexate(MTX), azathioprine or mycophenolate mofetil (MMF).
3- The monoclonal antibody belimumab has been shown to be effective in patients with active SLE who have responded inadequately to standard therapy.
Severe and life-threatening disease
Cases of renal, CNS and cardiac involvement. 1-High-dose glucocorticoids as pulsed methylprednisolone (10 mg/kg IV) and immunosuppressants as cyclophosphamide (15 mg/kg IV), repeated at 2-3-weekly intervals for six cycles.
Side effects of Cyclophosphamide
Cyclophosphamide may cause haemorrhagic cystitis, but the risk can be minimised by good hydration and co-prescription of mesna (2-mercaptoethane sulfonate), which binds its urotoxic metabolites.
Because of the risk of azoospermia and premature menopause, sperm or oocyte collection and storage need to be considered prior to treatment with cyclophosphamide
Severe and life-threatening disease
2- Mycophenolate mofetil (MMF) has been used successfully with high-dose glucocorticoids for renal involvement with results similar to those of pulsed cyclophosphamide, but fewer adverse effects .
3-Belimumab in combination with standard therapy significantly decreases disease activity in SLE patients and is safe and well tolerated.
Maintenance therapy
Following control of acute disease ,the long-term aim is to try to maintain remission with the lowest dose of glucocorticoid and immunosuppressant.
1-Oral prednisolone can be commenced in a dose of 40-60 mg daily, gradually reducing to 10-15 mg/day or less by 3 months.
Azathioprine (2-2.5 mg/kg/day), MTX (10-25 mg/week) or MMF (2-3 g/day) may also be prescribed as steroid-sparing agents
Maintenance therapy
2-Cardiovascular risk factors, such as obesity, hypertension and hyperlipidaemia, should be controlled and patients should be advised to stop smoking and reduce excess alcohol intake.
3-Patients with SLE and the antiphospholipid antibody syndrome, who have had previous thrombosis, require life-long warfarin therapy.
4-Patients with SLE are at risk of osteoporosis and hypovitaminosis D, and accordingly should be screened with biochemistry and DXA scannin
Prognosis
The 10-year survival rate is about 90%, but this is lower if major organ-based complications are present.
Deaths early in the course of disease are mainly due to renal or cerebral disease or infection,
later, coronary artery disease and stroke become more prevalent.
People with SLE have an increased long-term risk of developing some cancers, especially lymphoma.