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THE EGYPTIAN SOCIETY OF PEDIATRIC ALLERGY AND IMMUNOLOGY

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 This booklet is distributed (free of charge) during ESPAI's congresses and scientific meetings.

 

 

 

Systemic Lupus Erythematosus

Systemic lupus erythematosus (SLE) is a chronic inflammatory multi-system disease of unknown etiology. It is a disease of altered immune regulation probably genetically determined, characterized by periods of quiescence and exacerbations.

Age of onset: Rare <5 years (but as early as the1st year of life).

Sex ratio: More in females (4:1 before puberty, 8:1 afterwards)

Diagnosis of SLE:

      SLE should be suspected in any patient who has features involving 2 or more organs or systems.

      A person is said to have SLE if any 4 or more of the 11 criteria in table 1 are present serially or simultaneously during any interval of observation.

Clinical presentations that should raise suspicion of SLE :   SLE may present initially as:

-           Alopecia, Raynaud’s phenomenon, chronic urticaria

-           Idiopathic thrombocytopenic purpura

-           Lymphadenopathy &/or hepatosplenomegaly

-           Isolated hematuria, proteinuria or frank nephrotic syndrome

-      Transverse myelitis         - Hypo- or hyper-thyroidism

Table 1: Update of the ACR criteria for diagnosis of SLE.

 

Item

Definition

Malar rash

Fixed erythema, flat or raised, over malar areas, sparing nasolabial folds.

Discoid rash

1.Erythematous 2.Raised patches 3.Adherent scaling 4.Follicular plugging 5. Atrophic scars.

Photosensitivity

 

Oral ulcers

Or nasopharyngeal, usually painless.

Non-erosive arthritis

2 or more peripheral joints (pain, tenderness, effusion).

Pleuritis or pericarditis

Pleuritis: pleuritic pain, rub, effusion.

Pericarditis: rub, pericardial effusion, proved by ECG.

Renal disorder

Persistent proteinuria >0.5 gm/dl or > 3+ by dipstick. OR

Cellular casts.

Neurologic disorder

Seizures OR psychosis: after exclusion of other causes e.g. drugs, uremia, ketoacidosis.

Hematologic disorder

Hemolytic anemia + Reticulocytosis. OR

Leukopenia: <4,000/mm3 on > 2 occasions. OR

Lymphopenia: <1,500/ mm3 on > 2 occasions. OR

Thrombocytopenia: <100,000/ mm3. Not drug-induced

Immunologic disorder

Anti- DNA. OR Anti-Sm. OR

Anti-phospholipid antibodies.

Positive ANA

Not drug-induced (as phenytoin..).

Laboratory Investigations in SLE:

q        Immunologic tests: as in table 1 +/- lupus band test..

q        Tests to assess disease activity: ESR, C3, C4, anti-DNA.

q        Tests to evaluate organ systems: e.g.

         Hematologic: Complete blood counts, PT, PTT.

         Renal function tests, renal biopsy.

         Pulmonary function tests

         Heart: ECG, echocardiography

         CNS: EEG, CT scan, SPECT, PET.

         Capillaroscopy

q        Tests to detect drug side effects: See table 4.

 When to do a renal biopsy?

-           Nephritis

-           Suspicion of nephritis (hematuria, pyuria, casts)

-           Cyclosporine A therapy (yearly).

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Major Tasks of the Primary Care Physician (PCP):

The 4 major tasks of a PCP are shown in the following diagram.

 

       
 
   
 

 

 

 

 

 

 

 

 

 

 

 

   Fig 1:Tasks of the primary care physician in the diagnosis & management of SLE.

 

 When to Refer to a Pediatric Rheumatologist ?

1-       To confirm the diagnosis of SLE.

2-       To assess disease activity and severity

3-       To provide management strategy

4-       To manage uncontrolled or severe disease or complications

5-       Before surgical procedures

Assessment of Disease Activity:

This is based on clinical grounds, laboratory evaluation and a SLE disease activity index as the SLEDAI. (See below)

Classification of Disease Severity:

Mild SLE: Characterized by

-           Disease is clinically stable

-           Not life threatening

-           Normal organ system functions

-           No significant toxicity from SLE therapy

Severe life threatening SLE: For example

-           Cardiac: coronary vasculitis, endocarditis, myocarditis.

-           Pulmonary: hemorrhage, pneumonitis, fibrosis, emboli.

-           Hematologic: Hemolytic anemia, leukopenia, platelets <50,000 / mm3 , thrombosis.

-           GIT: Mesenteric vasculitis, pancreatitis.

-           Renal: Rapidly progressive glomerulonephritis.

-           CNS involvement

-           Severe myositis, diffuse rash with ulcers or blisters.

General Lines of Therapy of SLE:

- Patient education:

a.  Realistic expectations

b.  Avoid excess UV light exposure

c.  Avoid exhaustion

d.  Identify symptoms & signs of flare

e.  Comply with treatment

- Social support

- Rest when the patient feels a disease flare

- Diet:

a.  To prevent obesity, hyperlipidemia, osteoporosis.

b.  To supply antioxidants: as vitamins E & C, selenium, manganese.

c.  To supply anti-inflammatory N3- polyunsaturated fatty acids as in olive oil, fish oil.

d.   To supply potassium, calcium, vitamin D (under steroid therapy).

- Topical sunscreens (minimum sun protection factor 15)

- Analgesics when needed

- Topical glucocorticoids for rash (avoid high potency preparations on the face)

Immunization of SLE Patients:

-  Patients on immunosuppressive drugs should not receive live attenuated vaccines.

-   Influenza vaccine should be given.

- Herpes zoster, pneumococcal and hemophilus influenza vaccines are recommended if possible.

Treatment of mild SLE: as above +

- NSAIDs: For fever, arthritis and mild serositis.

 e.g. Ibuprofen 20-40 mg/kg/d in 3-4 divided doses after meals.

- Antimalarial drugs: For skin and joint manifestations

e.g. hydroxychloroquine 6-7 mg/kg/d in 2 divided doses with meals for 2 mo then decrease to 5 mg/kg/d.

- Low dose steroids: If not responding to the above drugs.

 0.25-0.5 mg/kg/d for 4-6 wks then taper gradually (table 2).

Treatment ofsevere SLE: See table 3.

 

  

Table 2: Oral Prednisone Regimen for SLE.

Initial therapy for 4-6 wks: individualize

Average dose: 0.25-0.5 for mild & 0.25-2 mg/kg/d for severe dis.

            Nephritis: 30-60 mg/ d

            Hematologic crisis: 60-100 mg/d

            CNS disease: 60-100 mg/d

Maintenance therapy: Steroid tapering

            Dose 60 to 20 mg/d: Decrease by 2.5-5 mg/wk

            Dose 20 to 10 mg/d: Decrease by 1-2.5 mg/wk

            Dose 10 mg/d: Decrease by 0.5-1 mg/2-4 wks.   

 

 

 

 

 

 

Table 3: Treatment of Severe, Life Threatening SLE

- Corticosteroids:

Prednisone (oral): See table 4.

Pulsed IV methyl-prednisolone

10-30 mg/kg/d (max. 1 gm) for 1-3 doses. 

- Immunosuppressive drugs:

Azathioprine (Imuran): 1-2 mg/kg/d

Cyclophosphamide (Cytoxan):

Oral: 1-2 mg/kg/d

            IV pulses: 500-750 mg/m2/mo for 7 doses        

                                       then /3mo for 10 doses.

Cyclosporine A (Neoral Sandimmun, Abrammun): 5-6 mg/kg/d.

- IV gamma globulin: for hemolysis or thrombocytopenia.

- Plasmapheresis: for CNS or cytopenias.

- Plasma exchange & anticoagulants for thrombotic events.

- Dialysis & transplantation: for end-stage renal disease.

 

 


 

Monitoring of SLE Patients

Frequency of visits:

In mild disease ? Every 3 months.

Severe and stable disease ? Every month.

Active disease ? Weekly.

At the start of immunosuppressive therapy ? Weekly.

In every visit:

1-       Clinical monitoring

2-       Laboratory monitoring:

Urinalysis – ESR – CBC – Serum Creatinine     

+ As needed:

In renal involvement: 24 hr urinary protein, creatinine clearance, calcium, phosphorus, alkaline phosphatase, sodium, potassium, anti-DNA, C3.

In patients with hemolytic anemia: reticulocyte count.

3-      Drug monitoring: As in table 4

 

Table 4: Monitoring Strategy for Drugs Used in SLE

Drug

Toxicity

Monitoring

NSAIDs

GIT bleeding, hepatic, renal, hypertension.

Yearly CBC & s. creatinine

Cortico-          

    steroids

Hypertension, hyperglycemia, hyperlipidemia, osteoporosis, cataract

Bl pr, visual changes, ur glucose /3mo, cholest./yr, bone densitometry/yr.

Hydroxy-

  chloroquine

Macular damage

Fundoscopic & visual fields/4mo.

Azathioprine

Myelosuppression, hepatotoxic, …

CBC/1-2 wk then/1-3 mo, AST/yr

Cyclo-phosphamide

Myelo- & immuno-suppression, malignancy, hemorrhagic cystitis.

CBC & urinalysis /mo, urine cytology

/yr for life.

Cyclosporine

   A

Interstitial nephritis, hypertension, hyperlipidemia, immunosuppression.

 

Bl pr & creatinine clearance /wk for 4 wks then /mo, serum trough level, renal biopsy/ yr.

                          SLEDAI: Data Collection Sheet

Patient’s Name:     ------------ Date:  ------ Physician:    --------  Chart no: --

[Enter weight (value) in Score column if descriptor present or in preceding 10 d. Exclude other causes. Compare with total score of last visit]

Weight

Score

Descriptor

8

_____

Seizure.

8

_____

Psychosis.

8

_____

Organic brain syndrome.

8

_____

VisVisual disturbance.

8

_____

Cranial nerve disorder: New onset.

8

_____

Lupus headache.

8

_____

Cerebrovascular accident: New onset.

8

_____

Vasculitis.

4

_____

Arthritis: > 2 joints.

4

_____

Myositis.

4

_____

Urinary casts: Heme-granular or RBCs casts.

4

_____

Hematuria: > 5 RBCs /HPF.

4

_____

Proteinuria: 0.5 gm/24 hr.

4

_____

Pyuria: >5 WBCs/HPF.

2

_____

New inflammatory type rash

2

_____

Alopecia: Recent, patchy or diffuse.

2

_____

Mucosal ulcers: Oral or nasal.

2

_____

Pleurisy: Pain,  rub, or effusion

2

_____

Pericarditis.

2

_____

Low complement: CH50, C3, or C4.

2

_____

Increased DNA binding

1

_____

Fever: >38o C.

1

_____

Thrombocytopenia: <100,000/mm3

1

_____

Leukopenia: <3000/mm3.

 Finally, SLE requires teamwork of a primary care physician, a pediatric rheumatologist, a psychiatrist, a physiotherapist, an ophthalmologist, a dentist and a social worker.