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 1st 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.
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