PRESENTED BY:

DR. HIRDESH CHAWLA

JUNIOR RESIDENT III
 It is the prototype systemic autoimmune
disease characterised by diverse system
involvement with production of array of
antibodies
 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
atrophic area.

 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,
steroids

 Mucosal ulcers- gradual onset, unilateral and

asymmetric.more commonly on hard palate, buccal
mucosa and vermilion border. D/d-candidiasis and oral
lichen planus.

 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
patients.

 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,
fibromyalgia

 CPK normal in steroids and antimalarial

myopathy, fibromyalgia

 Characterstic histopathological finding

 Significant cause of morbidity and mortality

 Upto 90% have pathological evidence but only 50% have clinical
evidence

 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

renal outcome

 Vascular disease - lupus vasculopathy , TMA, vasculitis ,

non specific vascular sclerosis, arteriosclerosis

 Unrelated to SLE- FSGS, Thin BM disease, HTN

nephrosclerosis

 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
(A/C) nephritis
IV – G Active & chronic lesions - Diffuse global proliferative & sclerosing lupus nephritis
(A/C)
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

ESRD

 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
toxicity.

 Valvular Abnormalities-

 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

initial presentation

 Additional risk factors-Steroids use,

Hypertension
 ACR has classified them into 19 different syndromes encompassing CNS
and PNS.

 Headache in more than 50% and cognitive dysfunction in more than

80%.Intractable headaches not responding to narcotic analgesics are
most common

 Psychiatric disorders like psychosis, depression, anxiety. D/d

schizophrenia, steroid induced psychosis(within first 2 weeks) and
metabolic disorders.

 Demyelinating disorders such as optic neuritis, myelitis.D/d MS,NMO

and APLA

 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
anytime.

 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

involvement.
 Decreased peristalsis in upper 1/3 esophagus with sparing of LES(versus
scleroderma)

 SLE related causes of abdominal pain- peritonitis, pancreatitis, mesenteric

vasculitis, pseudo obstruction

 Signs of acute abdomen and rebound tenderness may be masked because of

steroids

 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,

Antithrombopoeitin Ab

 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
estrogen levels.

 Hyperprolactinemia in 20% of patients.

 Adrenal Failure, Hypothyroidism

 SIADH
 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

 Malignancy-particularly NHL

 Autoimmune disorders- RA, Dermatomyositis ,

Still’s disease

 MCTD

 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.

 SEROLOGICAL TESTS-
 ANA(byIF test) is gold standard and present in >95% of patients.
 Also positive in RA,scleroderma, polymyositis, autoimmune
thyroiditis, elderly(<1:80)
 Negative test rules out SLE
 If ANA positive, then determine which particular nuclear antigens
are target
 Anti ds DNA is highly specific for SLE but present in 60% of
patients
 Anti Sm is also highly specific for SLE but present in 30% of
patients.
 Complement consumption leads to hypocomplementemia(rare in
other diseases) and signify active disease
Homogenous-anti dsDNA, speckled-anti RNP, nucleolar-anti
RNA polymerase I/III,cytoplasmic-anti Jo-1
 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

disease.

 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
titers.

 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
nephritis.
 Accurate assessment of disease activity and
flares
 Stratification according to severity of organ
involvement
 Safe and effective drugs to induce remission
promptly
 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.
 Education.
 Physical and lifestyle measures-diet, smoking
cessation, exercise, sun protection.
 Emotional support.
 Adjunctive measures-colonoscopy, Bone
Mineral Denstiometry, eye examination,
atherogenesis screening.
 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

week.

 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
skin disease

 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
neuropsychiatric, vasculitis
 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.
 Rituximab
 Epratuzumab
 Ocrelizumab
 Belimumab(approved by FDA)
 Atacicept
 Abatacept
 Other therapies-IVIg,DHEA,Clofazimine
 Constitutional symptoms-moderate dose steroids.

 Cutaneous disorders-steroids,topical, HCQ/CQ. Oral ulcers by

cyclosporine and triamcinolone dental gels.

 Cutaneovascular- CCBs, PDEIs, PGs, cold preventive measures

 Musculoskeletal Lupus-NSAIDs, Steroids, HCQ, AZA, MTX,

Leflunomide(no MMF)

 Cardiopulmonary-NSAIDs, Steroids, HCQ. Pulmonary HTN by

vasodilators, anticoagulation.

 Hematological-Steroids, IVIg and Rituximab ( for ITP),apheresis

 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
abnormal) GFR

 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
immunosuppressive treatment.

 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
structural MRI.

 fMRI may be informative regarding dysfunction or

redistribution of functions as a result of SLE.
Establishment of NPSLE
diagnosis
Cerebrospinal fluid examination
primarily to exclude infection
■ Identification of aggravating
factors
Symptomatic therapy
Cognitive rehabilitation
■ Immunosuppression
Anticoagulation
Autoantibody profile
(antiphospholipid, antiribosomal
P)
Neuroimaging to assess brain
structure and function
Neuropsychological assessment
Hypertension, infection,
metabolic abnormalities
Anticonvulsants, psychotropics,
anxiolytics
Corticosteroids, azathioprine,
cyclophosphamide,
mycophenolate mofetil, B-
lymphocyte depletion
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
delivery.

 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, β 2-

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)

 Multiple pregnancies

 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
pregnancy.

 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
autoimmune process.

 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.