Management &prognosis of systemic lupus erythematosus (SLE) in adults

Management &prognosis of systemic lupus erythematosus (SLE) in adults

Therapeutic goals for SLE ptns are to provide long-term survival, stabilize the lowest state of disease activity, prevent organ damage, limit drug toxicity, and improve quality of life, in addition to ptns education about their role in disease control. To determine the proper therapeutic regimen necessitate an accurate evaluation of disease activity & severity, with clear assessment of ptn's response to the current & previous therapeutic maneuvers. There’re 3 general patterns of SLE to consider during assessment disease activity including:

(1) Intermittent disease flares (or relapsing & remitting episodes),

(2) Chronically active disease &

(3) Quiescent disease.

Clinically-wise, disease activity & severity can be assessed via a combination of history taking, physical examination, lab & imaging profiles for systemic organs, in addition to serological testing. A number of lupus activity and damage indices can be also applied for research work-up.

Follow up checking may include lab tests to monitor disease activity in ptns with SLE: CBC, ESR, CRP, spot urine protein & Cr, SCr, eGFR, anti-dsDNA), in addition to C3 & C4. Frequency of lab tests monitoring could be tailored in an individual basis. Monitoring for a particular SLE-related organ affection usually requires the additional profile related to the organ system in question. Several non-pharmacological & preventive measures essential in the managing SLE may include: sun protection, diet & nutrition, exercise, smoking cessation, maintenance of appropriate immunizations, ttt of co-morbid conditions, avoiding of certain drugs, and pregnancy & contraception planning.

Therapeutic regimen of SLE is primarily individualized to each ptn and can be guided via the predominant organ affection. However, some general recommendations of drug therapy for ALL SLE ptns have been advised:

· SLE ptn with any degree & type of disease activity, hydroxychloroquine or chloroquine, unless there is contraindication.

· Additional therapeutics usually depending on the severity of flares as well as the constellation of manifestations:

(1) Mild lupus disease: (e.g., skin, joint, & mucosal involvement) > hydroxychloroquine or chloroquine, +/- NSAIDs, and/or short-course of low-dose steroids (e.g., ≤7.5 mg prednisone / d).

(2) Moderate lupus: defined as significant but non-organ-threatening involvement (e.g., constitutional, cutaneous, musculoskeletal, or hematologic). Ptns usually respond to hydroxychloroquine or chloroquine + short-term of 5-15 mg/d. prednisone. The latter can be tapered once hydroxychloroquine or chloroquine has commencing its effect. A steroid-sparing im/m. agent (e.g., azathioprine or methotrexate) is may be indicated to control Sms.

(3) Severe or life-threatening disease that’s related to major organ affection (e.g., kidney, CNS) generally in need to initial period of intensive im/m. agents (as induction) to control manifestations & prevent organ damage. Ptns can be ttt with short period of high doses GC (e.g., 1-2 mg/kg/d prednisone or intermittent IV "pulses" of methylprednisolone) alone or combined with other im/m. agents (e.g., MMF, cyclophosphamide, or rituximab). Initial therapy is then followed by longer period of less intensive, ideally, less toxic maintenance therapy to stabilize remissions & halt flares. Current dose of prednisone can be reduced with monitoring clinical & lab profiles of disease activity.

SLE can experience a variable clinical course ranging from a relative benign illness to a rapidly progressive disease and fulminant organ failure & death. Clinical remission after proper therapy is uncommon, and, when it‘s achieved, it is usually not sustained. Poor prognostic factors for survival in SLE include:

(1) Renal disease (esp. diffuse proliferative GN),

(2) Male sex,

(3) Black race,

(4) Hypertension,

(5) Low socioeconomic status,

(6) High overall disease activity.

(7) Younger or older age at presentation,

(8) The presence of antiphospholipid AB.

SLE ptns have MR ranging from 2-5 times higher than general population. The major causes of death in the 1^st few ys of illness are active disease (e.g., CNS & renal) or infection due to im/m therapy, while causes of late death include: SLE sequelae (e.g., ESRD), ttt complications, & CVS disease. Ptns are also at risk for significant morbidity due to both active flares and the SE of drugs e.g. steroids & cytotoxic medications. Despite the overall risk of death related to malignancy is not currently observed to be elevated in SLE ptns, the risk of death due to particular malignancy, especially non-Hodgkin lymphoma, is significantly raised among SLE ptns as compared to the general population.