DR. HIRDESH CHAWLA
JUNIOR RESIDENT III
It is the prototype systemic autoimmune
disease characterised by diverse system
involvement with production of array of
Clinical features may be quite variable
ranging from mild joint involvement to severe
life threatening disease.
Diagnostic criteria developed by ACR in 1971
revised in 1982 and then in 1997
A person must have to fulfill 4 out of 11 criteria to be
classified as SLE but it can extend over a period of
weeks to years.
Fulfillment of these criteria is not absolute
requirement rather diagnosis rests on constellation of
symptoms with supportive serological studies.
Most common presenting complaints are
constitutional symptoms, cutaneous manifestations
and articular manifestations(present in atleast 50% at
time of diagnosis).These manifestations appear over
time as disease evolves.
Fatigue occurs frequently and is sometimes
the most disabling symptom
About 42% of patients have fever as
manifestation of active lupus. SLE is cause of
PUO in less than 5% of patients.
Fever can also be because of medications,
malignancies and infections.
ACLE - Localised to malar region , confluent,
macular or papular erythema, symmetrical on
cheeks and bridge of nose sparing nasolabial
folds with or without induration and scaling.
can be generalised in photosensitive
distribution. Pruritic and painful.
D/d- acne, rosacea, seborrhoeic Dermatitis,
erysipelas, atopic dermatitis
Biopsy required in uncertain cases
SCLE-non scarring, photosensitive lesions that
can be papulosquamous resembling psoriasis or
annular polycyclic with central clearing.
Predilection for back, neck, shoulders and spares
face. Heal without scarring
Annular type associated with anti-SSA/Ro
antibody.(seen in Sjogren,RA)
Drugs- ACEIs,CCBs ,Hydrochlorthiazide
CCLE-can lead to skin atrophy and scarring and
persist for months
Discoid LE is most common. Sharply demarcated,
disk shaped, raised, erythematosus plaques with
adhered scales and central hypopigmented
Follicular plugging is characterstic finding
Permanent alopecia, SCC can occur if untreated
Photosensitivity-abnormal skin reaction in 90% of patients
on provocative testing. D/d is PMLE (treatment by UV light)
Alopecia-scarring secondary to DLE. Non scarring-telogen
effluvium, lupus hair, alopecia areata,cytotoxic drugs,
Mucosal ulcers- gradual onset, unilateral and
asymmetric.more commonly on hard palate, buccal
mucosa and vermilion border. D/d-candidiasis and oral
Lobar panniculitis presenting as subcutaneous nodules
called as lupus profundus
Arthritis and arthralgia-present in 90%.Earliest and most
common initial symptom. Symmetric, inflammatory, non
erosive(mostly) affecting knees, wrists and small joints.
Hand deformity similar to Jaccoud’s arthropathy because
of ligament and joint capsule laxity.
Avascular necrosis- femoral heads, tibial plateaus, femoral
condyles most common. Acute onset of pain. can also be
because of high dose of steroids.MRI is diagnostic.10% of
Deforming arthritis can be either mild deforming
polyarthritis or rhupus (symmetric,resembling RA)
Salmonella septic arthritis is more common than
non salmonella species
Myositis-usually involves proximal upper and
lower extremities. D/d myopathy of steroids,
antimalarials, statins, MCTD, infectious myositis,
CPK normal in steroids and antimalarial
Characterstic histopathological finding
Significant cause of morbidity and mortality
Upto 90% have pathological evidence but only 50% have clinical
Typically develops within first 36 months of disease and
frequency decreases after 5 yrs.
Features include proteinuria, hematuria, hypertension,pyuria,
urinary casts, deranged KFT.
Presentation can be variable ranging from hematuria to frankly
nephrotic to RPGN
Routine screening for polyuria, hematuria, hypertension,
proteinuria, LE edema, change in creatinine is important.
Immune complex mediated GN-most common
Tubulointerstitial disease-predictor of poor long term
Vascular disease - lupus vasculopathy , TMA, vasculitis ,
non specific vascular sclerosis, arteriosclerosis
Unrelated to SLE- FSGS, Thin BM disease, HTN
Biopsy is diagnostic
Finding of TMA should suggest APLA nephropathy
Class I Minimal mesangial lupus nephritis (Light microscopy : Normal glomeruli)
Class II Mesangial proliferative LN (few isolated subepithelial/endothelial deposits visible by
IF or electron microscopy)
Class III Focal lupus nephritis (<50% glomerulli, sub endothelial deposits)
III A Active lesions
III A/C Active and Chronic Lesions
III C Chronic Lesions
Class IV Diffuse lupus nephritis( >50% glomeruli involved, Segmental and Global lesions)
IV – S (A) Active lesions - Diffuse segmental proliferative lupus nephritis
IV – G (A) Active lesions - Diffuse global proliferative lupus nephritis
IV – S
Active & chronic lesions - Diffuse segmental proliferative & sclerosing lupus
IV – G
Active & chronic lesions - Diffuse global proliferative & sclerosing lupus nephritis
IV – S (C) Chronic inactive lesions with scars - Diffuse segmental sclerosing lupus nephritis
IV – G (C) Chronic inactive lesions with scars - Diffuse segmental global lupus nephritis
Class V Membranous lupus nephritis
Class VI Advanced sclerosing lupus nephritis
Mesangial nephritis is most common amongst
the silent nephritis cases.
Class IV patients tend to relapse most frequently.
Class V-anti C1q Ab has best negative predictive
value for predicting flares.
Despite treatment, 10% patient will progress to
Type I and Type IV RTA also is reported.
Pleuritis- upto 50%. Small, bilateral , exudative.
Massive effusion is uncommon. D/d-infection, HF,
malignancy, drug induced
Lupus pneumonitis- rare. severe, acute respiratory
illness. fever, cough, pulmonary infiltrates,
hypoxemia.High mortality rate.
Chronic Interstitial Lung disease-NSIP and UIP are
most common patterns
DAH-DLCO is increased, Fall in Hb level. Hemosiderin
laden macrophages on BAL.High mortality rate.
PAH-0.5 to 14% of patients. Should exclude
other secondary causes
Shrinking lung syndrome-Dyspnea, low lung
volumes, elevation of hemi diaphragm in
absence of parenchymal involvement
Pericarditis-most common seen in 50%.Fibrinous and exudative.rarely tamponade
and purulent effusions.
Myocarditis-in 10% of patients clinically. unexplained HF, unexplained tachycardia,
ECG abnormalities. Endomyocardial biopsy helps in distinguishing from HCQ
Valvular thickening with mitral and aortic valve involvement is most common in
60% of patients.
Libman Sacks endocarditis – 43% of patients. pea sized, flat or raised, bland,
granular lesion on ventricular aspect of mitral valve posterior leaflet and vessel
side of aortic valve. Can be active or healed lesions.
Valvular regurgitation 25%
Valvular stenosis in 4%
CAD-Atherosclerosis, coronary artery
vasculitis, coronary artery thrombosis
Young women with SLE have 50 fold higher
risk of MI.
Rule out APLA because thrombosis can be the
Additional risk factors-Steroids use,
ACR has classified them into 19 different syndromes encompassing CNS
Headache in more than 50% and cognitive dysfunction in more than
80%.Intractable headaches not responding to narcotic analgesics are
Psychiatric disorders like psychosis, depression, anxiety. D/d
schizophrenia, steroid induced psychosis(within first 2 weeks) and
Demyelinating disorders such as optic neuritis, myelitis.D/d MS,NMO
Ischemic stroke is more common than IC hemorrhage
Peripheral neuropathy- symmetric, length dependent sensory(glove and
stocking) or sensorimotor.(vasculitis and demyelination)
GTCS is more common than other types of epilepsy and
indicated active lupus whereas focal seizures can occur
Risk of seizure is more with presence of anti Smith and
anti cardiolipin Ab.
Major risk of stroke is in first 5 years of diagnosis and
younger SLE have higher risk.
Antibodies to ribosomal P protein is associated with
depression and psychosis.
Anti NMDA receptor Abs are associated with diffuse CNS
Decreased peristalsis in upper 1/3 esophagus with sparing of LES(versus
SLE related causes of abdominal pain- peritonitis, pancreatitis, mesenteric
vasculitis, pseudo obstruction
Signs of acute abdomen and rebound tenderness may be masked because of
Liver abnormalities in 60% of patients but rarely significant. Presence of liver
disease in SLE should prompt non SLE causes like Drugs(azathioprine,MTx,
NSAIDs), obesity, Diabetes, Steroid treatment, EBV and CMV
Lupus hepatitis is different from autoimmune hepatitis(no lymphoid infiltrate,anti
SMA and anti LKM negative)
Veno occlusive disorders, Budd Chiari syndrome and hepatic infarction
Lupus enteritis affects jejunum and ileum. Ascites is seen in 11% of patients
though peritoneal inflammation is seen in 60% of patients.
50% of SLE have anemia.(ACD>IDA>AIHA)
Always consider myelosuppression because of azathioprine, MTx,MPF and cyclophophamide.
Steroid causes lymphopenia and leucocytosis
AIHA is mediated by warm reacting IgG anti erythrocyte Ab.(spherocytosis)
MAHA should prompt consideration for TTP and HUS(schistocytes).rule out CAPS by APLA
Leucopenia in approx 50% of patients.(BM suppression ,drugs,antilymphocytotoxic Ab)
Neutropenia because of immune mediated destruction or marrow suppression
Thrombocytopenia because of immune mediated destruction, TTP, Splenomegaly,
AIHA and isolated thrombocytopenia may precede years before SLE develops
Periarterial fibrosis or ‘onion skin’ lesion in
spleen is pathognomonic of SLE.
Kikuri Fujimoto Disease is a self limited form
of SLE.Presenting as cervical Lnpathy with
tenderness and night sweats, fever.
Lower androgen levels with increased
Hyperprolactinemia in 20% of patients.
Adrenal Failure, Hypothyroidism
Most common is keratoconjunctivitis sicca
Retinal abnormalities-hemorrhages, vasculitic
lesions, cotton wool spots and hard exudates
Uveitis is very rare
Posterior subcapsular cataracts and increased IOT
because of steroids
Maculopathy because of HCQ therapy
Risk of retinal toxicity is low if CQ is<3mg/kg daily
and HCQ is <6.5mg/kg
Viral infections-Parvo B19,Hep B and Hep C,CMV
and EBV, HIV
Autoimmune disorders- RA, Dermatomyositis ,
Drug induced lupus-Antihistone Ab are present
in >95% of cases except those by minocycline (ds
DNA and p ANCA)
SLE is diagnosed on the basis of constellation of symptoms,
signs and lab findings in appropriate clinical text.
ANA(byIF test) is gold standard and present in >95% of patients.
Also positive in RA,scleroderma, polymyositis, autoimmune
Negative test rules out SLE
If ANA positive, then determine which particular nuclear antigens
Anti ds DNA is highly specific for SLE but present in 60% of
Anti Sm is also highly specific for SLE but present in 30% of
Complement consumption leads to hypocomplementemia(rare in
other diseases) and signify active disease
Urinalysis is an effective method to detect and monitor disease
activity in lupus nephritis.
Hematuria (usually microscopic) with dysmorphic cells indicates
inflammatory glomerular or tubulointerstitial disease.
In severe proliferative nephritis, urine sediment may contain the
full range of cells and casts.
Granular and fatty casts reflect proteinuric disease.
Erythrocyte, leukocyte, and mixed cellular casts reflect nephritic
Broad and waxy casts are found in chronic renal failure.
Urine sediment abnormalities in active lupus nephritis are
typically accompanied by significant proteinuria (more than 0.5 g
of protein per 24 hours)
Mesangial nephritis- low-grade proteinuria
with hematuria but typically no cellular casts.
Membranous glomerulopathy - proteinuria,
often at nephrotic range ,but otherwise blunt
urine sediments. Serum C3 levels are normal and
anti-DNA antibodies are usually found in low
Proliferative nephritis- hypertension, nephritic
urine sediment with various degrees of
proteinuria, low C3 levels, and typically high
titers of anti-DNA antibodies.
Any sign of renal involvement—in particular, reproducible
proteinuria of 0.5 g of protein or more per 24 hours,
especially with glomerular hematuria—should be an
indication for biopsy.
Although biopsy can also be considered in cases of
persisting isolated glomerular hematuria, isolated
leukocyturia (after other causes, such as infection or
drugs, are excluded), or unexplained renal insufficiency
with normal urinary findings.
In several studies of lupus nephritis, class IV nephritis has
been found to be the most common (approximately 40% of
cases), followed by class III and V with approximate
frequencies of 25% and 15%, respectively.
Changes in SCr concentration (20% to 30% increase or
more) or GFR (10% or more deterioration from baseline)
are of concern because they indicate significant ongoing
loss of renal function.
Persistently increased SCr levels (2.0 mg/dL or higher) at
the time of response to immunosuppressive treatment is
associated with subsequent development of ESRD.
Conversely, reduction of SCr concentration (even within
the “normal” range of values) at 6 months after induction
therapy is associated with increased likelihood of a
favorable long-term renal outcome
Spot urine protein-creatinine ratio (UPCR) measured is a
conveniently repeatable measure for detecting within-
patient changes in proteinuria and is currently
recommended for monitoring patients with lupus
Accurate assessment of disease activity and
Stratification according to severity of organ
Safe and effective drugs to induce remission
Prevention and management of disease and
treatment related comorbidities
SLEDAI and its modifications measure disease activity
based on 24 questions assessing the clinical
manifestations of SLE, including physical findings and
laboratory values weighted across organ systems.
Although the maximum score achievable is 105, even
with very active disease, scores rarely exceed 20.
Preferential weighting is based on the manifestations
of vasculitis, central nervous system (CNS)
involvement and active renal disease .
Score manifestations over the past 28 to 30 days
A flare is a measurable increase in disease activity in one
or more organ systems involving new or worse clinical
signs and symptoms and/or laboratory measurements. It
must be considered clinically significant by the assessor
and usually there would be at least consideration of a
change or increase in treatment.
Specific criteria to distinguish between mild, moderate,
and severe flares remain to be established.
Mild/moderate flares are defined as new or worsened
clinical features resulting in increases in the SELENA-
SLEDAI score of 3 or more points, prednisone use less than
0.5 mg/kg/day, PGA score of 1.0 to 2.5 on a 0 to 3 visual
analog scale (VAS), or any combination of these measures.
Severe flares are defined as changes in SELENA-SLEDAI
scores to higher than 12 or clinical manifestations
requiring doubling of the prednisone dose or an increase
to 0.5 mg/kg/day or greater.
Physical and lifestyle measures-diet, smoking
cessation, exercise, sun protection.
Adjunctive measures-colonoscopy, Bone
Mineral Denstiometry, eye examination,
For cutaneous manifestations of lupus
Nonfluorinated steroid creams are weak but safe for long-term
use, especially for facial lesions.
Fluorinated steroids are quite effective but should never be used
on a facial lesion for longer than 2 weeks (otherwise, they will
lead to cutaneous atrophy and telangiectasias).
Ninety percent of all lupus prescriptions are written for one of
three preparations: desonide (mild potency), triamcinolone
(moderate potency), or clobetasol (high potency).
Topical calcineurins can be applied with or without
corticosteroids for cutaneous disease. These include
pimecrolimus creams and tacrolimus ointments.
Fever, headache, myalgias, arthralgias,
arthritis, and/or serositis.
NSAIDs should be used in pregnancy only
occasionally and with great caution.
Topical NSAIDs (diclofenac or ketoprofen gel,
salicylates) are safer and should be
considered as a first-line treatment for local
or regional inflammation.
High-dose daily corticosteroids are efficacious for life or organ-threatening AIHA,
ITP, bone marrow hypoplasia, acute myocarditis, acute lupus pneumonitis,
mesenteric vasculitis, autoimmune pancreatitis, optic neuritis, and retinal vasculitis.
Treatment is usually prescribed at a dose of 1 mg/kg/day of prednisone equivalent
for 2 to 4 weeks.
A dramatic response is often followed by a slow tapering of approximately 10% per
If the manifestation is refractory to treatment or if tapering to 10
mg/day(0.25mg/kg alternate day) of prednisone equivalent cannot be achieved,
steroid-sparing immunosuppressive regimens of targeted therapies can be added
In severe rapidly progressing disease requiring >0.6mg/kg/day prednisone, pulse
therapy with 250 to 1000mg of MPS for 1 to 3 consecutive days.
Non-CNS or nonrenal manifestations of lupus warrant consideration of pulse
corticosteroids as induction therapy followed by high-dose daily
corticosteroids, including any of the aforementioned complications as well as
thrombotic thrombocytopenic purpura, Stevens-Johnson syndrome–like skin
reactions, severe acute cutaneous disease, or alveolar hemorrhage.
Moderate-dose daily corticosteroids (considered to be 0.25 to 0.5
mg/kg/day of prednisone equivalent) may be given for 2 to 4 weeks followed
by tapering for treatment of acute pleurisy or pericarditis, flares of
thrombocytopenia, active rashes, or lupus myositis.
Low-dose daily corticosteroids (less than 10 mg of prednisone equivalent
daily) are the mainstay for persistent, chronic, active disease, especially with
musculoskeletal, constitutional (fatigue, fever, adenopathy), cutaneous, and
serous membrane manifestations or combinations of these
HCQ and CQ are commonly prescribed to SLE patients with skin and joint
involvement and as adjuvant in severe lupus. CQ is given in refractory skin lesions.
HCQ delays the onset of SLE and reduces the number and severity of clinical flares.
They protect against thrombosis, renal damage, and major infections in SLE.
HCQ is associated with an 80% clinical response rate at 3 months when used in
doses of 5 mg/kg/day for non–organ-threatening disease.
Because HCQ can induce corneal edema and retinal pigmentation (the latter occurs
in 3% of patients who have used the drug for 10 years or longer), it is
recommended that baseline screening should be performed during the first few
months of therapy and again at 5 years followed by annual examinations.
Chloroquine is usually given at a dosage of 500 mg daily for the first 1 to 2
months, after which treatment is transitioned to HCQ or dosing is gradually
tapered to 250 mg as 1 to 2 tablets a week.
MTX is used as steroid sparing in articular,serosal and cutaneous manifestations in
mild to moderate cases.
Leflunomide is used in lupus nephritis refractory or intolerant to standard therapy.
Cyclophosphamide-equivalent efficacy with monthly IV(0.5 to 1
g/m2) versus oral regimens(25-100 mg).
7 monthly pulses of IV followed by quarterly pulses for atleast 1
yr beyond remission in lupus nephritis.
Also used in extrarenal disease like severe thrombocytopenia,
neurologic disease, abdominal vasculitis,alveolar h’ge and severe
Combination of IV-MP with IV-CYC is treatment of choice for
severe inflammatory neurologic SLE.
Chlorambucil-orally (0.1-0.2 mg/kg/day). In combination with
IV-MP for remission of nephrotic syndrome. Also in
Azathioprine(2 mg/kg/day)-safe and efficacious for maintenance
of remission in SLE including moderately severe Proliferative
Lupus nephritis. Also in thrombocytopenia(20000-50000/mm3)
and serositis .
Measurement of TMPT activity before initiation of therapy may
identify patients at greatest risk of bone marrow suppression .
Although it is classified as a pregnancy category D drug, AZA has
a well-established safety record as an option for lupus patients
who wish to conceive.
Mycophenolate Mofetil(2gm/day)-MMF is as effective as CYC for
complete remission in PLN and can be used as induction therapy.
Used as maintenance therapy in PLN.
May be effective in refractory skin and blood manifestations.
Cyclosporin A-Maintenance therapy in mild to
moderate proteinuric PLN with preserved
renal function.treatment of membranous
lupus nephropathy. Can be used as an
alternative steroid sparing agent to AZA
Tacrolimus-10-100 times more potent than
CsA.Beneficial effects in refractory to
conventional therapy PLN and LMN.
Plasmapheresis is used in SLE for TTP ,
cryoglobulinemia , NMO, pulmonary
hemorrhage , and hyperviscosity syndrome.
Laser therapy is occasionally used for
telangiectasias and refractory discoid lesions.
Photopheresis is occasionally used for
refractory skin manifestations.
Belimumab(approved by FDA)
Demographic: Black race,males, children
Clinical: Hypertension; severe extrarenal disease affecting major organ; failure
to achieve or marked delay (>2 years) in achieving renal remission; multiple
flares of lupus nephritis; pregnancy
Laboratory: Nephritic urinary sediment; azotemia; anemia; thrombocytopenia;
antiphospholipid antibodies; thrombotic microangiopathy;
hypocomplementemia (especially falling levels); high anti-DNA titer
(especially rising titers); persistent severe nephrotic syndrome
Histologic: Proliferative glomerulonephritis ; mixed membranous (class V) and
proliferative (class III-IV) glomerulonephritis; cellular crescents; fibrinoid
necrosis; very high activity index (>12); moderate to high chronicity index
(>3); combinations of active (cellular crescents) and chronic (interstitial
fibrosis) histologic features; extensive subendothelial deposits
The EULAR recommendations defined partial renal response as at least 50%
reduction in UPCR and normal or near-normal (within 10% if initially
abnormal) GFR. Partial response should be achieved preferably 6 months and
no later than 12 months after initiation of treatment.
The ultimate goal of treatment should be complete renal response, defined as a
UPCR of less than 0.5 and normal or near-normal (within 10% if initially
Refractory disease as failure to achieve (1) any reproducible reduction in
UPCR during the first 3 to 4 months, or (2) partial renal response after 6 to 12
months, or (3) complete renal response after 1 to 2 years of
Nephritic flares consist of a reproducible increase in SCr concentration of 30%
or more (or a reduction in GFR by 10% or more) and active urine sediment
with an increase in glomerular hematuria by 10 or more red blood cells per
HPF, irrespective of changes in UPCR.
Proteinuric flares consist of a reproducible doubling of UPCR to more than 1.0
after complete renal response or a reproducible doubling of UPCR to more
than 2.0 after partial response
Decreased whole-brain magnetization transfer on MTI
has been reported in SLE patients even in the absence
of other structural MRI changes.
MRS- Decreased NAA, believed to reflect neuronal/
axonal loss or dysfunction, has been reported in SLE
patients, even in the absence of visible damage on
fMRI may be informative regarding dysfunction or
redistribution of functions as a result of SLE.
Establishment of NPSLE
Cerebrospinal fluid examination
primarily to exclude infection
Neuroimaging to assess brain
structure and function
■ Identification of aggravating
Symptomatic therapy Anticonvulsants, psychotropics,
■ Immunosuppression Corticosteroids, azathioprine,
mycophenolate mofetil, B-
Anticoagulation Heparin, warfarin
Women with lupus tend to have fewer live births,
however, vast majority of pregnancies in the
setting of SLE are uneventful.
Disease quiescence at the time of, and 6 months
previous to, conception is an important
prognostic factor for pregnancy loss and preterm
The presence of active lupus nephritis is a
predictor of increased lupus activity during
pregnancy and poor pregnancy outcomes
History of pregnancy complications and a poor obstetric outcome
Presence of high-titer antiphospholipid antibodies (anticardiolipin antibodies, β2-
glycoprotein, and/or lupus anticoagulant)
Presence of anti-Ro and anti-La antibodies
Current or previous lupus nephritis or ongoing severe renal impairment
Advanced maternal age (>40 years)
Use of cytotoxic medications at the time of conception, including high-dose prednisone
Active flare at the time of, or 6 months before, conception
Hydroxychloroquine-safe to use in pregnancy , associated with decreased SLE flares
when used continuously.
NSAIDs can be used sporadically in the first and second trimesters of pregnancy, but
they are associated with an increased risk of miscarriage and oligohydramnios . NSAIDs
should be avoided in the last trimester of pregnancy due to concerns of premature
closure of the ductus arteriosus and worsening of hypertension.
Corticosteroids are sometimes indicated in the setting of a lupus flare. Dexamethasone
and betamethasone cross the placenta and should not be used unless there is an intent to
expose the fetus to corticosteroids.
Prednisone and prednisolone are deactivated by placental hydroxylases but should be
used with caution given the increased risk of maternal diabetes, hypertension,
preeclampsia, and premature rupture of membranes.
Antihypertensives - ACEIs are contraindicated in the setting of pregnancy. methyldopa,
nifedipine, and labetalol are generally thought to be safe options
Rituximab and belimumab are not recommended in
Azathioprine is probably the safest
immunosuppressive medication in pregnancy.
Azathioprine is generally regarded to be safe when
used at dosages of 2 mg/kg or less.
Cyclophosphamide, methotrexate, mycophenolate,
leflunomide, and warfarin, is contraindicated in
pregnancy, because of their teratogenicity. If
possible, these medications should be discontinued
at least 3 months before conception.
Palmar erythema because of pregnancy, and mild alopecia should be distinguished from
a true lupus-related rash.
Arthralgias, myalgias, and backaches are normal symptoms of pregnancy and can
usually be managed conservatively with acetaminophen and physical therapy. True
synovitis does not occur in normal pregnancy and can be related to a lupus flare.
Mild anemia and thrombocytopenia are normal variants of pregnancy, but severe anemia
and thrombocytopenia may be an indication of an immune-mediated process. The
presence of leukopenia and lymphopenia should raise the suspicion of a systemic
A rise in C3 and C4 levels is a normal variation of pregnancy and this can make
identifying an SLE flare difficult, so normal complement levels should not automatically
be interpreted as an indication of disease quiescence.
Double-stranded DNA, however is not affected by pregnancy and can increase when
SLE is uncontrolled.
Asymptomatic carriers of antiphospholipid antibodies with no history of pregnancy loss
or thrombosis, treatment is generally not needed.
In women with a history of recurrent pregnancy loss or thrombosis, treatment options
include low-dose aspirin combined with either unfractionated heparin or enoxaparin
For women in whom combination therapy has been ineffective, intravenous
immunoglobulin, corticosteroids, and plasmapheresis are treatment options, although the
evidence for their effectiveness is questionable.
Treatment of high-risk patients is continued for at least 6 to 8 weeks after delivery
because of a continuing thrombosis risk in the postpartum period.
Women with a history of recurrent thrombosis may need lifelong treatment. Despite the
risks associated with the presence of antiphospholipid antibodies, live births have been
reported in over 50% to 74% of such patients when treatment was initiated.