Principles of Therapy Therapeutic choices depend on :
• Patients require individualized therapy ( 1 ) whether disease manifestations depending on disease manifestations, are life-threatening or likely to cause activity & severity organ damage • Control disease manifestations (2) whether manifestations are • Allow the patient to have a good quality potentially reversible of life w/o major exacerbations (3) the best approaches to • Prevent serious organ damage that preventing complications of disease adversely affect function or life span and its treatments. • Prevent adverse effects of the drugs used systemic glucocorticoids (0.5-1mg/kg per day PO)/ 500-1000 mg of methyl prednisolone sodium succinate IV daily for 3 days followed by 0.5- 1 mg/kg of daily prednisone or equivalent). + Immunosuppressive agent
low doses of cyclophosphamide (500 mg every 2 weeks for six total
doses, followed by azathioprine or mycophenolate maintenance) are as effective as standard high doses, with less toxicity. High-dose cyclophosphamide (500-1000 mg/m2 body surface area • Analgesic+A/malarial given monthly IV for 6 months, followed by azathioprine or +/- belimumab. mycophenolate maintenance)
The number of SLE flares is reduced by maintenance therapy
with myιophenolate mofetil ( 1 .5-2 g daily) or azathioprine (1- Activity Index [SLEDAI] score of >10, positive 2.5 mg/kg per day). anti-DNA, and low serum complment. SLEDAI is a widely used measure of SLE disease activity; scores >3 reflect clinically Cyclophosphamide and mycophenolate responses begin active disease. 3-16 weeks after treatment is initiated, whereas glucocorticoid responses may begin within 24 h. • Inhibit inflammatory reactions & pain by • Used for skin & joint manifestations decreasing prostaglandin synthesis • Useful for patients with skin disease that are unresponsive to topical corticosteroids & in • These drugs provide symptomatic relief of patients with arthritis that does not respond fever, arthritis & mild serositis to NSAID • SLE patients have a high incidence of NSAID- • Recommended as background treatment for Class induced hepatotoxicity III/IV SLE patients with nephritis • Patients with continuing treatment with Hydroxychloroquine showed less renal Non-Pharmacological Therapy damage as compared to those on placebo • Also used for preventing flares, reducing CV risk & • Patients w/ systemic lupus recurrent infection, & other constitutional erythematosus (SLE) should apply sunscreen symptoms w/ at least an sun protection factor (SPF) of 30 to prevent dermal or systemic disease flares upon exposure to ultraviolet light Topical corticosteroids Oral corticosteroids • Patients w/ mild (SLE) do not normally require use of • for discoid lesions especially on the scalp systemic corticosteroids but there are patients who has low quality of life if not given low-dose corticosteroids • Used as initial therapy for severe discoid lupus Parenteral erythematosus or lupus vasculitis Corticosteroids • Low-dose corticosteroids may be added to • Pulse therapy w/ IV Hydroxychloroquine for fatigue & fever corticosteroids in combination w/ • High-dose corticosteroids are necessary for refractory immunosuppressive manifestations of SLE & for severe organ involvement therapy is especially CNS, renal & hematologic manifestations recommended for Class • Corticosteroid use should be tapered as soon as desired III/IV SLE patients w/ response is observed (control of inflammatory confirmed manifestations) to avoid toxicity glomerulonephritis Choice of immunosuppressant will depend on nature & severity of disease manifestation • These agents act as immunosuppressive, cytotoxic & anti-inflammatory agents • In the treatment of severe CNS & severe glomerulonephritis, thrombocytopenia & hemolytic anemia, high-dose glucocorticoids & immunosuppressants are used • Concomitant use w/ corticosteroids allows lower doses of immunosuppressants Lifelong monitoring is required for systemic lupus erythematosus (SLE) patients. Frequency of visits depends on disease activity, severity & extent; response to treatment, type of treatment & monitoring of toxicity The most important tool in the management of SLE is careful, frequent clinical & laboratory evaluation to detect disease flares, appearance of infections & to tailor management based on patient response
Patients w/ severe SLE, complications & toxicity
will require more frequent follow-up
• Results of laboratory tests that may precede a disease flare:
• Decrease in serum complement levels • Increase in anti-dsDNA • Increase in erythrocyte sedimentation rate (ESR) • Decrease in hemoglobin level, leukocyte or platelet counts • Increase in creatine phosphokinase (CPK) levels • Appearance of microscopic hematuria or proteinuria • A rheumatologist is an integral part of the medical team managing an SLE patient • Referral to a rheumatologist is indicated for: • life-threatening or serious organ system • Confirmation of diagnosis involvement • Management plan for patient • requiring high-dose corticosteroid • Periodic evaluation of disease activity & severity • needing chemotherapy • Management of uncontrolled disease • with severe hypertension • Management of organ involvement or life-threatening • with unexplained fever to rule out sepsis disease • Prevention &/or management of treatment toxicities • Special situations (eg pregnancy, antiphospholipid syndrome, life-threatening disease, concomitant infection & surgery) • SLE patients should be referred to a psychologist, psychiatrist, physical &/or occupational therapist, ophthalmologist, dermatologist, nephrologist, cardiologist, orthopedic surgeon & other specialists when necessary • If there are signs of renal involvement, patient should be referred to a nephrologist for management • Glucocorticoids are Category A • Cyclosporin, Tacrolimus Rituximab in • Lupus does not affects fertility. Category C • Rate of fetal loss increased. • AZA, HCQs, MMF, CPM as category D • Demise is higher in mothers with (benefits outweighs risk) – high disease activity , APLA, • MTX is Cat X (risk outweighs benefits) active nephritis • Mx : HCQ + prednisone (lowest dose • Women with AntiRo need • for short time) additional monitoring, high risk • AZA may be added. for neonatal Lupus. • Breast feeding should be avoided • APLA with SLE treated with ( glucocorticoids and heparin and low dose Asprin. immunosuppressants get into breast milk).