Guideline for SLE Management

7/27/2019 Guideline for SLE Management

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Guideline for SLE

management


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Epidemiology of SLE

Prevalence of SLE

In U.S18-24/100000

black female7.9-10.5/100000

white female4/100000

More common in urban than in rural areas

Femalemale=1.4-5.81 (children)8-131 (adult)

21 (older)

Onset age=65% between 16-55 y/o

20% < 16 y/o

15% > 55 y/o

Identical twin30%

first degre relative5%

Annual incidence of new case6/100000 (low-risk group)

35/100000 (high risk group)


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Subacute Cutaneous Lupus

Erythematosus Widespread, non-scarring but often

photosensitive rash

Annular or papulosquamous morphology Mild systemic disease common but renal

involvement rare

Positive ANA in most patients, but anti-nDNAuncommon Anti-Ro in two thirds patients

HLA-DR3 present in the majority of patients


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Symptoms Percentage

Fatigue 80-100

Fever >80

Weight loss >60

Arthritis, arthralgia 95

SkinButterfly rashPhotosensitivityMucous membrane lesion

AlopeciaRaynauds phenomenonPurpuraUrticaria

>80>50


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Table 9-3. THE SPECTRUM OF ANAs

Chromatin

Anti-native DNA

Anti-single-stranded DNA

Anti-Z DNA

Anti-centromere

Anti-Ku

Anti-HMG proteinsAnti-topoisomerase I (Scl-70 antigen)

Anti-topoisomerase II

Anti-PBC 95K

Anti-lamins

Nucleolar Components

Anti-RNA polymerase I

Anti-Th

Anti-Us (fibrillarin)

Anti-Pm/Scl

Anti-NOR-90

Other Cellular ComponentsAnti-unclear pore complexes

Anti-centrosomes

Anti-midbody

Anti-spindle

Anti-Mi

Anti-Su

Nuclear Ribonucleoproteins

Anti-U1 RNP

Anti-Sm

Anti-Ro

Anti-La

Anti-U2 RNP

Anti-U4 U6 RNP

Anti-U5 RNP

Anti-5S rRNAprotein

Cytoplasmic Components

Anti-Jo-1 (tRNAhistidylsynthetase)

Anti-tRNAalanyl synthetase

Anti-tRNAthreonyl synthetase

Anti-tRNAglycyl synthetase

Anti-signal recognition particle (SRP)Anti-ribosomes


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Fig 1 Four steps in the indirect immunofluorescence assay (cross-sectional view).

Monolayer cells grown on a glass slide (A) are fixed and permeabilized (B) with chemicals

such as acetone, methanol, ethanol, or formaldehyde. After a first incubation with patients

serum containing autoantibodies , cells are washed to get rid of unbound antibodies, and

the second incubation takes place with fluorescent-labeled anti-buman antibody (D). The

slides are again washed, mounted with coverslips, and read on a fluorescence microscope.

Nu, nucleoplasm; Cy, cytoplasm.


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CAUSES OF POSITIVE ANTINUCLEAR ANTIBODIES

1. Rheumatic diseases

Systemic lupus erythematosus

Polymyositis

Sjogrens syndrome

Scleroderma

Vasculitis

Rheumatoid arthritis

2. Normal, healthy individuals

Females > males, prevalence increases with age

Relatives of patients with rheumatic diseases

? Pregnant females

3. Drug-induced

4. Hepatic diseases

Chronic active hepatitis

Primary biliary cirrhosis

Alcoholic liver disease

5. Pulmonary diseases

Idiopathic pulmonary fibrosis

Asbestos-induced fibrosis

Primary pulmonary hypertension

6. Chronic infections

7. Maliganncies

LymphomaLeukemiaMelanomaSolid tumors (ovary, breast, lung, kidney)

8. Hematologic disorders

Idiopathic thrombocytopenic purpuraAutoimmune hemolytic anemia

9. Miscellaneous

Endocrine disorders (type I diabetes mellitus, Graves disease)Neurologic diseases (multiple sclerosis)End-stage renal failureAfter organ transplantation


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Serological Tests to Aid

Diagnosis of SLETest % positive in SLE

ANA 95%

Anti-nDNA 60%

Anti-nRNP 80%

Anti-Sm 20%

Anti-Ro 30%

Anti-La 10%


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Main patterns of autoantibody

1. HomogenousAnti-histone

2. PeripheralAnti-dsDNA, Anti-lamine

3. SpeckledA large family of nonhiston antigens

-Coarse:Anti-Sm, Anti-U1-nRNP

-Fine:Anti-Ro, Anti-La

-Distinct speckles varying in number(PBC)

Anti-p80-coilin, Anti-p95

4. Nucleolar Scleroderma or overlap syndrome

DNA topoisomerase:nucleolar speckles

PM-Scl:homogenous decorating nucleoliFibrillarin (U3-RNP):clummy nucleolar

NOR-90:nucleolar speckles

5. CentromereCREST syndrome, and PBC

6. Cytoplasmic

ANCA,


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Positive

ANA

Nucleoli

Raynauds

phenomenon

Scleroderma

Diffuse (honogeneous)

Anti-nucleoprotein

SLERADrug LE

Histone

SLERADrug LE

Centromere

CRESTSdleroderma

Peripheral (rim)

Anti-dsDNA

SLE

Negative

No disease

Lab errorTreatmentRemissionAntigen XSNephrotic syndrome

No specificityUCTDSLERALiver diseaseMono

Any chronicinflammatory disease

RNP

SLEMCTDRASclerodermaUCTD

Sm

SLE

RO (SS-A)

SLE

Sogrenssyndrome

PcNA

SLE

Scl-70

Scleroderma

PM/Jo/Ku/Mi

PM/DM

La

SLE

Sogrenssyndrome

Speckled


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Tabel 1. CUTANEOUS CHANGES IN LUPUS ERYTHEMATOSUS

Speclfic

Discold

Subacute cutaneousPapulosquamous

Annular/polycyclic

Neonatal lupus erythematosus

Malar dermatitis

Nonspeclfic Lesions

Bullous

Lupus panniculitis

Alopecia

Vasculitis

Urticaria-like vasculitis

Livedo reticularisRaynauds phenomenon

Photosensitivity

Oral ulcerations

Nail changes

Cutaneous mucinosis

Rheumatoid nodules


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Table 61-2. MUSCULOSKELETAL MANIFESTATIONS IN SLE

SLE RA

Arthralgia Common Common

Arthritis Common Deforming

Symmetry Yes Yes

Joints involved PIP > MCP > wrist > knee MCp > wrist > knee

Synovial hypertrophy Rare Common

Synovial membrane abnormality Minimal Proliferative

Synovial fluid Transudate Exudate

Subcutaneous nodules Rare 35%

Erosions Very rare Common

Morning stiffness Minutes Hours

Myalgia Common Common

Myositis Rare UncommonOsteoporosis Variable Common

Avascular necrosis 5-50% Uncommon

Deforming arthritis

Swan neck

Ulnar deviation

Uncommon

10%

5%

Common

Common

Common

MCP, Metacarpophalangeal joint; PIP, proximal interphalangeal joint.


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Possible causes of leukopenia in SLE

Immune destruction

Marrow suppression

Hypersplenism

Drugs


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Possible causes of anemia in SLE

Anemia of chronic disease

Auto-immune hemolytic anemia

Hypoplastic anemia Blood loss due to thrombocytopenia or NSAID use

Hypersplenism

Anemia of renal failure


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AN APPROACH TO THE MANAGEMENT OF LUPUS THROMBOCYTOPENIA

Confirm diagnosis by examining peripheral smear and bone marrow examination, and determineseverity

Rule out drug effects and discontinue all but absolutely essential drugs

Rule out thrombotic throbocytopenic purpura (may be indicated by anemia with pronouncedreticulocytosis and fragmented erythrocytes in the peripheral smear)

Rule out infectionviral

HIV, HBV, CMVbacterialsubacute bacterial endocarditis, gram-negative sepsis

Look for evidence of lupus activity in other organs; beware of major organ involvement

Use prednisone 0.25-1.0mg/kg/day for 3-4 weeks if platelets < 50.000/mm3 (unless otherwiseindicated for other manifestations of lupus); taper after 3-4 weeks

The goal is a stable platelet count > 50.000/mm3

If prednisone fails or unable to tape, consider danazol (400-800mg/day),-globulin orsplenectomy

In patients refractory to these modalities or patients with major organ involvement, use monthlypulses of cyclophosphamide for at least 6 months

Fig. 7.14 An approach to the management of lupus thrombocytopenia.


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Lupus lymphadeenitis (I)

The prevalence of lymphadenopathy range from 12-59% of lupus patients The most common sites are cervical (43%), mesenteric

(21%), axillary (18%) and inguinal (17%). Unusual sites

such as hilar, mediastinal and retroperitoneal were also

reported

The pathognomonic pathologic feature of lupuslymphadenitis, the hematoxylin body, was described by

Ginzler and Fox in 1940, which stain with periodic acid-

Schiff and Feulgen methods, are coalescent amorphicaggregates of deeply basophilic material found within

areas of lymph node necrosis.

The hematoxylin bodies are highly specific for SLE, arealso found in glomeruli, endocardium and spleen


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Lupus lymphadeenitis (II)

Other lymph node features include paracortical focl ofnecrosis marked infiltration by histiocytes, lymphocytes andplasma cells and the preesence of imunoblasts. Neutrophils,

eosinophils and granulomata are conspiculously absent.

Both lupus lymphadenitis and KFD are characterized by ofhistiocytic and immunoblastic infiltrates. A prominent plasmacell component strongly suggests lupus lymphadenitis. When

present, hematoxylin bodies are virtually diagnostic of SLE.

The clinical feature, building on these pathologicresemblances, supports a link between KFD and SLE.

Perhaps KFD and SLE share a common inclting event, suchas exposure to an enviromental or infectious agent, that can

produce either disorder. Alternatively, KFD may be an

antoimmune-midiated necrotizing lymphadenitis that can

remain self-limited or develop into SLE


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Primary Respiratory System Involvement in Systemic Lupus Erythematosus

Upper airway disease

Epiglottitis

Subglottic stenosis

Vocal cord paralysis

Laryngeal edema or ulceration

Inflammatory mass lesions or nodules

Cricoarytenoid arthritis

Necrotizing vasculitis

Parenchymal disease

Acute lupus pneumonitis

Alveolar hemorrhage syndrome

Chronic lupus pneumonitis or interstitial lung disease

Lymphocytic interstitial pneumonia or pseudolymphoma

Bronchiolitis obliterans with or without organizing pneumonia

Respiratory muscle disease

Shrinking lung syndrome

Pleural disease

Pleuritis with or without effusion

Vascular diaease

Pulmonary hypertension

Pulmonary embolism

Acute reversible hypoxemia


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Pulmonary Involvement In SLE

Pleural disease

Acute lupus pneumonitis

Chronic interstitial lung disease

Pulmonary hemorrhage

Pulmonary embolism

Pulmonary vascular disease

Diaphragmatic dysfunction


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Cardic manifestation of SLE

Pericardium Pericarditis with or without

effusion

Cardiac tamponade (rare)

Constrictive perlcarditis

Myocardium Myocarditis

Endocardium Libman-Sacks endocarditis

Coronary artery Accelerated atherosclerosis

vasculitis


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Common Clinical and Laboratory Findings in Lupus Nephritis

Disordered fluid and electrolyte balanceNocturia, decreased urinary concentrating capacity, hyperkalemia,

renal tubular acidosis

Nephritic syndromeHematuria, cellular casts; variable bypertension, edema, proteinuria,

azotemia

Nephrotic syndromeFrothy urine, edema, proteinuria >3.5g per day, lipiduria (fatty casts,

oval fat bodies, doubly refractile fat bodies), hypoalbuminemia,

hyperlipidemia

Secondary complications of nephrotic syndrome includevolume depletion, prerenal azotemia, venous thrombosis, pulmonary embolism,

atherosclerosis, hypogammaglobulinemia

Renal insufficiency

Acute, rapidly progressive or chronic renal failure


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Characteristic Clinicopathologic Correlations in the Major Classes of Lupus Nephritis

Normal or minimal disease

Mesangial lupus nephritis

Clinicallow-grade hematuria, proteinuria, normal renal function

Pathologyincreased mesangial cells, matrix, and immune complexes; patent glomerular

capillary loops

Focal proliferative lupus nephritis

Clinicalnephritic urine sediment, variable but usually nonnephrotic proteinuriaPathologysegmental proliferation, necrosis, crescents compromising capillary loops in 50% of glomeruli; variable sclerosis, atrophy, andfibrosis; mesangial, subendothelial, and subepithelial immune complex deposits

Membranous nephropathy

Clinicalnephrotic syndrome

Pathologydiffuse capillary loop thickening; subepithelial immune deposits


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Morphological Classification of Lupus Nephritis

(modified WHO Classification)

Class Biopsy finding

I Normal glomerule

II Pure messngial alterationIII Focal proliferative glomerulonephritis

IV Diffuse proliferative glomerulonephritis

V Menbranous glomerulopathyVI Advanced glomerulosclerosis


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INDICES OF ACTIVITY AND CHRONICITY IN LUPUS NEPHRITIS*

Activity Index (Range 0 to 24)

Glomerular hypercillularity

Leukocyte exudation

Karyorrhexis/fibrinoid necrosis

Cellular crescents

Hyaline thrombi

Tubulointerstitial inflammationChronicity index (Range 0 to 12)

Glomerular lesions

Glomerular sclerosis

Fibrous crescents

Tubulointerstitial lesionsTubular atrophy

Interstitial fibrosis

*Individual lesions are scored 0 to 3+ (absent, mild, moderate, severe). Indices are composite scores for

individual lesions in each category of activity or chronicity. Necrosis/karyorrhexis and cellular crescents

are weighted by a factor of 2.


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INDICATIONS FOR RENAL BIOPSY IN LUPUS NEPHRITIS

Proteinuria of >1g/day

The threshold is conventionally 1-2g/dayLess proteinuria does not preclude biopsy if it occurs in the context of major serologicabnormalities, especially hypocomplementemiaAt the other extreme, the presence of full-bolwn nephrotic and nephritic syndromes may makerenal biopsy unnecessary

Progressive azotemia

Decreasing renal function in associtaion with active urinary sediment is an indication for biopsyin order to assess the extent of crescents and necrosis which would warrant very aggressivetherapy

Ambiguity or inconsistency of data

Lupus nephritis of indeterminate duration, severity and potential responsiveness warrants theestablishment of a fresh baseline database including determination of class,activity andchronicity indices

Overlapping clinical features

Situations where clinical and laboratory data are compatible with different classes of lupusnephritis, for which different approaches to management are warranted

Redirection of therapy

Partially treated or incompletely responsive lupus nephritis for which a change in therapeuticplan is deemed appropriate


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SLE and ESRD (1)

5-22% of SLE patients progress to ESRD requiring H/D

In USA, Iupus nephropathy accounting for 1.4% off allESRD

Decreased clinical and serological lupus activity

following ESRD. Some theories had

1.Depressed cellular and humoral immunity

2.Lack of mediators produced by the kidney

3.Removal of lupus factors by dialysis itself4.Nature end point in SLE

Survival of lupus patients on dialysis versus non-SLEdialysis patientsno significant


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SLE and ESRD (2)

Renal transplant graft survival of lupus versus non-lupuspatientsno difference

Lupus patients have slightly better outcome with LRrather than CAD grafts

The transplantation time following dialysis need at least3 months

Recurrence of transplanted allograft is often similar to

histologic or immunofluorescent type as in the originkidney, but it is a rare event.


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NEUROPSYCHIATRIC MANIFESTATIONS IN SYSTEMIC LUPUS ERYTHEMATOSUS

Central Nervous System

Diffuse manifestations (35%-60%) Seizures (15%-35%)

Organic brain syndromes

Organic amnestic/cognitive dysfunction

Dementia

Altered consciousness

Grand mal

Focal

Temporal lobe

Petit mal

Psychiatric

Psychosis

Organic mood/anxiety syndromes

Other

Headaches

Aseptic meningitis

Pseudotumor cerebri

Normal pressure hydrocephalus

Focal manifestations (10%-35%)

Cranial neuropathies

Cerebrovascular accidents/strokes

Transverse myelltis

Movement disorders

Peripheral Nervous System

Peripheral Neuropathies (10%-20%) Other

Sensory polyneuropathy

Mononeruitis multiplex

Chronic, relapsing polyneruopathy

Guillien-Barre syndrome

Autonomic noruopathy

Myasthenial gravis

Eaton-Lambert syndrome


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PATHOGENETIC MECHANISMS CAUSING NEUROPSYCHIATRIC

SYMPTOMS IN SYSTEMIC LUPUS ERYTHEMATOSUS

Primary Secondary

Autoantibody mediatedAntineuronal antibodies

Vascular occlusion

Immune complex-mediated vasculitis

Immune complex-mediated anaphylatoxin

release causing leukoagglutination

Antiphospholipid antibody-associated

hypercoagulability

Thrombosis

Emboli from cardiac source

Cytokine effects

Combination of mechanisms

InfectionHypertensionUremia

Electrolyte imbalances

Hypoxia

Fever

Thyroid disease

Thrombotic thrombocytopenic purpuraAtherosclerotic strokes

Emboli from valvular vegetations

Subdural hematoma

Cerebral lymphoma

Medications

Drug overdose

Corticosteroids

NSAIDs

Hydroxychloroquine

Azathioprine

Fibromyalgia

Reactive depression


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Pathogenesis of Neuropsychiatric Events in Patients with SLE

Primary events

Vascular occlusion from immune-complex-mediated or antibody (for example,antiphospholipid)mediated vasculopathy, vasculitis, leukoagglutination, or

thrombosis.

Cerebral dysfunction from antibodies to brain tissue (antineruonal,

antiribosomal P protein) or cytokines (interleukin-6, interferon-).

Secondary eventsInfection (meningitis, abscess, discitis)

Cerebrovascular accidents due to accelerated atherosclerosis

Hypertensive encephalopathy

Metabolic encephalopathy (uremia, electrolyte imbalance, fever, hypoxia)

Hypercoagulable state due to the nephrotic syndrome

Drugs (glucocorticoids, nonsteroidal anti-inflammatory agents, trimethoprim and

sulfamethoxazole, hydroxychloroquine, azathioprine).

Intrathecal production or entrance through a blood-brain barrier disturbed by

vascular injury.


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FREQUENCY OF ABNORMAL LABORATORY TESTS COMMONLY USED IN THE

EVALUATION OF NEUROPSYCHIATRIC LUPUS ERYTHEMATOSUS

Test Frequency of

Abnormal Test Result

Range (%)

Comment

Serologic

Antimeuronal antibodies

Antineurofilament antibodies

Antiribosomal-P antibodies

Antiphospholipid antibodies

30-92

58

45-90

45-80

Diffuse manifestations


Psychosis/depression

Focal manifestations, strokes

Cerebrospinal fluid

Routine

Pleocytosis

Increased protein]

Low glucose

6-34

22-50

3-8

Rule out infection and NSAID meningitis

Nonspecific

Rule out infection, transerse myelitis

SpecialAntineuronal antibodies (lgG)

Elevated Q albumin

Elevated lgG/lgM index

Oligoclonal bands (2 bands)

90

8-33

25-66

20-82

Diffuse manifestations, present in 40%

with focal manifestations

Break in blood-brain barrier




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Pathologic studies in NPLE

Several autopsy series agree on several important points

There is no pathognomonic lesion that NPLE causes in

the brain that is diagnostically specific like the wire loop

lesion observed in lupus nephritis.

Active vasculitis is rare, bland vasculopathy (vascularhyalinization, perivascular imflammation, and endothelial

proliferation associated with microinfarcts and

hemorrhage is the most common pathologic abnormalities

seen.)

Clinical manifestations may not be readily explained by

pathologic findings, some NPLE patients, particularly

those with diffuse neuropsychiatric manifestations may

have normal or unremarkable brain pathology.


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Proactive and preventive strategies in

addition to lupus therapies(1)

Patients education programs, eliminate patientnonadherence

Monitor vital signs, update physical examination, and

have laboratory work done Adhere to a general conditioning exercise program to

minimize osteoporosis and muscle atrophy

Cognitive therapy for lupus fog;biofeedback forRaynauds phenomenon

Counseling and stress management

Physical and occupational therapy, ergonomic workstation evaluation


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Proactive and preventive strategies in

addition to lupus therapies(2)

Aggressive proactive management of blood pressure,blood sugars, serum lipids, and weight. Smokingcessation.

Yearly bone densitometry and osteoporosisprevention measures.

Annual electrocardiogram and chest x-ray

Prompt evaluation of all fevers

Periodic screening with carotid duplex scanning,treadmill, or stress testing; screening for, andprophylactic management of, antiphospholipidantibodies.


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Therapies for lupus patients with

skin lesions(1)

General

Avoid sun: clothing, sunscreens, avoid hot part of

day with most UV-B light, camouflage cosmetics

Stop smoking (so antimalarials works better)

Thiazides and sulfonylureals may exacerbate skin

disease


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skin lesions(2)

Routine therapy

Topical steroids, intralesional steroids

Hydroxychloroquine

Oral corticosteroids

Dapsone for bullous lesions


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skin lesions(3)

Advanced therapy for resistant causes

Subacute cutaneous lupus: mycophenylate

mofetil, retinoids, or cyclosporine Discoid lesions: chloroquine, clofazimine,thalidomide, or cyclosporine

Lupus profundus: dapsone

Chronic lesions over 50% of body: topicalnitrogen mustard, BCNU, or tacrolimus

Vasculitis: may need immunosuppressives


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Therapy for lupus patients with arthritis

(no internal organ involvement)

First line: NSAIDs

Cyclooxygenase-2 specific inhibitors

(but may induce thrombotic risk in

patients with antiphospholipid antibodies)

Low dose hydroxychloroquine(200mg

twice a day)


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Indications of high dose corticosteroid

therapy in lupus patients

Severe lupus nephritis

CNS lupus with severe manifestations

Autoimmune thrombocytopenia withextremely low platelet counts(e.g.


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Life-Threatening Manifestations of SLE:

Responses to glucocorticoids(1)

Manifestations often responsive to glucocorticoids

Vasculitis

Severe dermatitis of subacute cutaneous lupus

erythematosus or SLE

Polyarthritis

Polyserositispericarditis, pleurisy, peritonitis

Myocarditis Lupus pneumonitis


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~(continue)

Glomerulonephritisproliferative forms

Hemolytic anemia

Thrombocytopenia Diffuse CNS syndromeacute confusional state,

demyelinating syndromes, intractable headache

Serious cognitive defects

Myelopathies Peripheral neuropathies

Lupus crisishigh fever and prostration


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Manifestations not often responsive toglucocorticoids Thrombosisincludes strokes

Glomerulonephritisscarred end-stage renaldisease, pure membranous glomerulonephritis

Resistant thrombocytopenia or hemolyticanemiaoccurs in a minority of patients; considersplenectomy, cytotoxics, danazol, orcyclosporine/neoral therapies

Psychosis related to conditions other than SLE,such as glucocorticoid therapy


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Therapy for patients with lupus nephritis

Previously untreated patients with active

lupus nephritis or severe manifestations

( decreased renal function and /or high-grade proteinuria)

First line: high doses of corticosteroids

(about 1mg/kg/day) Cytotoxic drugs or other

immunosuppressive drugs


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The indications of cytotoxic drugs use in

the treatment of lupus nephritis

Active and severe GN despite treatment withhigh dose prednisone

Responded to corticosteroids but require anunacceptably high dose to maintain aresponse.

Unacceptable side effects fromcorticosteroids.

Chronic damage on a renal biopsy and otherindicators of a poor prognosis.


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Systemic therapies for nonorgan-

threatening lupus

Nonsteroidal anti-inflammatory drugs

Antimalarials

Thalidomide Hormonal interventions:

dehydroepiandrosterone, testosterone

patches, bromocriptine, prolactin Immunosuppressive therapies:

azathioprine, methotrexate, leflunomide


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The management of organ-threatening lupus

Existing immunosuppressive therapies:

cyclophosphamide, mycophenolate mofetil,

cyclosporine A, fludarabine, cladribine (2-CDA)

Apheresis

Intravenous immunoglobulin

Various biologic agents: BlyS inhibitor, CTLA-4Ig,

LL2IgG

Stem cell transplantation


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Use of Cytotoxic Drugs in SLE :

Azathioprine

requires 612 months to work well

13 mg/kg/day(initial dose)

12 mg/kg/day(maintenance dose) Advantage:probably reduces flares, reduces

renalscarring, reduces glucocorticoid dose

requirement Side effects: Bone marrow suppression,

leukopenia, infection(herpes zoster), infertility,malignancy, early menopause, hepatic

damage, nausea


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Use of Cytotoxic Drugs in SLE:

Cyclophosphamide

requires 216 weeks to work well

Initial dose:1-3 mg/kg/day orally or 820mg/kg intravenously once a monthplus mesna

Maintenance dose:0.52 mg/kg/day orally or 820mg/kg intravenously every 412 wks plus mesna

Adverse effects:probably reduces flares, reducesrenal scarring, reduces glucocorticoid dose

requirement Adverse effects: marrow suppression, leukopenia,

infection, infertility, malignancy,menopause,cystitis,nausea


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The treatment in lupus patients with

autoimmune thrombocytopenia

Splenectomy

Danazol

Immunosuppressive or cytotoxic drugs:azathioprine, cyclophosphamide

Intravenous immunoglobulin(IVIG)


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Other management principles in the

treatment of lupus patients(1)

Thrombosis-Anticoagulation

Recurrent fetal loss with antiphospholipid

-Heparin in low dose or low-molecular-weightheparin with or without aspirin

-If heparin ineffective or not tolerated, use low-doseaspirin alone

-Glucocorticoids plus aspirin in moderate to highdose may be used but is controversial

Thrombocytopenia or hemolytic anemia

-Intravenous gamma globulin, splenectomy, danazol,

cyclosporine, cytotoxic drugs


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Seizures without other serious manifestations

-Anticonvulsants

Behavior disorders or psychosis without otherserious manifestations:

-Psychoactive drugs, neuroleptics

Pure membranous glomerulonephritis:

-Limited trials of immunosuppressives or no specific

treatment


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Avoid possible disease triggers-sulfaantibiotics, sun, high estrogen-containingbirth control pills,alfalfa sprouts

Prevent atherosclerosis Prevent osteoporosis

Prevent infection

Prevent progression of renal disease

Prevent clots in patients with antiphospholipidantibodies

Treat fatigue.

Guideline for SLE Management

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