Thursday, March 11, 2010

Systemic lupus erythematosus

    Systemic lupus erythematosus - Chronic
Rahul Soman, M. Pharm


 

Definition  

A chronically remitting and relapsing, inflammatory, multisystemic autoimmune disorder primarily affecting women of childbearing age .


 

Ongoing Assessment  


 

Clinical Evaluation  


 

Renal Disease  


 

Renal biopsy findings help guide pSLE management, especially decisions pertaining to low- or high-dose corticosteroid and immunosuppressant therapies.  

* Kidney biopsy


 

Hematologic involvement  


 

Suspected autoimmune hemolytic anemia  

* Direct Coombs test: A positive direct Coombs test requires further analysis of IgG or complement on red blood cells to determine the hemolytic mechanism .


 

Suspected or known systemic lupus erythematosus  

* Complete blood count: Hematologic abnormalities (anemia, leukopenia, and/or thrombocytopenia) are characteristic of active systemic lupus erythematosus (SLE) .


 

Cardiac Involvement  


 

Suspected pericarditis  

* 12 lead ECG: The ECG is an essential tool for evaluating acute pericarditis, and is helpful in assessing constrictive pericarditis and cardiac tamponade .


 

Endocrine involvement  


 

Suspected diabetes mellitus  

* Plasma fasting glucose measurement: Fasting plasma glucose levels of 126 mg/dL (7 mmol/L) or greater are diagnostic of diabetes mellitus. In the absence of unequivocal hyperglycemia, results must be confirmed with repeat testing on another day .


 

Suspected or known hypothyroidism  

* Thyroid stimulating hormone measurement: Overt hypothyroidism is classified as a TSH above the upper limit of the reference range in the presence of a low serum free thyroxine (FT4) . Subclinical hypothyroidism is a laboratory diagnosis defined as a TSH above the upper limit of the reference range in the presence of a normal FT4.


 

Management Classification  


 

Renal Biopsy-Based Management Classification in Lupus Nephritis  


 

Renal biopsy findings help guide pSLE management, especially decisions pertaining to low- or high-dose corticosteroid and immunosuppressant therapy.  

* Kidney biopsy


 

Suspected and known chronic kidney disease  

* Creatinine measurement, serum: Serum creatinine levels should be obtained in all patients with known or suspected chronic kidney disease to estimate the GFR .


 

Suspected and known lupus nephritis  

* Urinalysis: In systemic lupus erythematosus, urinalysis findings of hematuria, casts, pyuria, and/or proteinuria indicate lupus nephritis.


 

Medical History  

* Female

* Family history of Systemic lupus erythematosus

* Infectious disease

* Occupational Exposure

* Environmental Exposure

* Stress


 

Findings  

* Headache

* Impaired cognition

* Mental state finding

* Pleurisy

* Breath sounds - finding

* Cramping pain

* Cranial nerve disorder

* Dry cough

* Dyspnea - Chronic

* Hemoptysis

* Idiopathic livedo reticularis

* Lupus vasculitis

* Myocarditis

* Pericarditis secondary to systemic lupus erythematosus

* Peripheral neuropathy - Chronic

* Psychotic disorder

* Retinal vasculitis

* Seizure

* Severe visual impairment

* Venous thrombosis, phlebitis and thrombophlebitis

* Abnormal weight gain

* Alopecia due to underlying disease

* Arthralgia

* Bleeding gums

* Butterfly rash

* Discoid lupus erythematosus

* Edema of eyelid

* Fever

* Has nosebleeds - epistaxis

* Jaccoud's syndrome

* Joint swelling

* Keratoconjunctivitis sicca

* Loss of appetite

* Lymphadenopathy

* Malaise and fatigue

* Mucosal ulcer

* Myalgia

* Nausea, vomiting and diarrhea

* Photosensitivity

* Pitting edema

* Subacute cutaneous lupus erythematosus

* Systemic lupus erythematosus arthritis

* Weight loss

* Chorea in systemic lupus erythematosus

* Raynaud's phenomenon


 

Tests  


 

Suspected or known systemic lupus erythematosus  

* Complete blood count: Hematologic abnormalities (anemia, leukopenia, and/or thrombocytopenia) are characteristic of active systemic lupus erythematosus (SLE) .


 

Suspected systemic lupus erythematosus  

* Antibody to SM measurement: A positive result on a Smith antibody assay usually confirms diagnosis of systemic lupus erythematosus, but a negative result does not exclude the diagnosis .


 

Suspected systemic lupus erythematosus  

* Antibody to double stranded DNA measurement: High titers of anti-dsDNA antibodies are nearly pathognomonic for SLE, especially if coincident with other autoantibodies such as anti-Sm .


 

Suspected systemic lupus erythematosus  

* ANA measurement: Antinuclear antibodies are detected in 98% to 100% of patients with systemic lupus erythematosus .


 

Suspected and known lupus nephritis  

* Urinalysis: In systemic lupus erythematosus, urinalysis findings of hematuria, casts, pyuria, and/or proteinuria indicate lupus nephritis.


 

Suspected C3 deficiency  

* C3 complement assay: Immune complex diseases such as systemic lupus erythematosus (SLE) have been associated with decreased levels of complement C3 .


 

Suspected C4 deficiency  

* C4 complement assay: C4 deficiency is associated with certain nephritic autoimmune diseases .


 

Suspected systemic lupus erythematosus  

* Antibody to SS-A measurement: A positive anti-Ro/SSA test is highly indicative of SLE and may help confirm the diagnosis in a patient with otherwise uncertain findings .


 

Suspected systemic lupus erythematosus  

* Antibody to SS-B measurement: A positive titer on an anti-SS-B antibody test means that a patient may be 40 times more likely than controls to develop systemic lupus erythematosus .


 

Guideline recommendation to assist in diagnosis of SLE and as baseline for monitoring disease activity .  

* Erythrocyte sedimentation rate measurement


 

Suspected and known chronic kidney disease  

* Creatinine measurement, serum: Serum creatinine levels should be obtained in all patients with known or suspected chronic kidney disease to estimate the GFR .


 

Differential Diagnosis  

* Rheumatoid arthritis

* Drug-induced lupus erythematosus

* Immune thrombocytopenic purpura

* Wegener's granulomatosis

* Primary antiphospholipid syndrome

* Mixed connective tissue disease

* Primary Sjögren's syndrome

* Systemic sclerosis

* Polymyalgia rheumatica

* Primary fibromyalgia syndrome


 

Treatment  


 

Drug Therapy  


 


 

Mild systemic lupus erythematosus (SLE)  


 

IBUPROFEN (Related toxicological information in IBUPROFEN)  

Adults: 600 mg orally 3 times daily; total dosage not to exceed 3.2 g/day


 

ASPIRIN (Related toxicological information in SALICYLATES)  

Adults: 650 mg orally every 4 hours; total dosage not to exceed 3.6 g/day


 

HYDROXYCHLOROQUINE SULFATE  

Adults: 200 mg orally 1 or 2 times daily (indefinitely)

Pediatrics: 5 mg/kg orally daily; not to exceed 400 mg daily


 

PREDNISONE  

Adults: Up to 10 mg orally daily; may use alternate day dosing


 

Betamethasone Dipropionate  

Adults: Thin film applied to skin lesions 1 or 2 times daily; total dosage not to exceed 45 g weekly; not to be used with occlusive dressing


 


 

Moderate to severe systemic lupus erythematosus (SLE)  


 

PREDNISONE  

Adults: Up to 10 mg orally daily; may use alternate day dosing; severe disease 40-60 mg orally daily (maximum 100 mg orally daily)


 

METHOTREXATE SODIUM  

Adults: 7.5-25 mg orally or IM once weekly


 

METHYLPREDNISOLONE  

Adults: Up to 1 g IV infusion over 30 minutes once daily for 3 consecutive days


 

AZATHIOPRINE (Related toxicological information in AZATHIOPRINE/MERCAPTOPURINE)  

Adults: 1-3 mg/kg orally daily


 

CYCLOPHOSPHAMIDE (Related toxicological information in CYCLOPHOSPHAMIDE AND RELATED AGENTS)  

Adult: 0.5-1.0 g/m2 body surface area intravenously once monthly


 

Non-Procedural Therapy  


 

Prevention of exacerbations and complications of systemic lupus erythematosus  

* Lifestyle Modifications

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SYSTEM BASED CLASSIFICATION OF DISEASES

SYSTEM BASED CLASSIFICATION OF DISEASES

Bone and Joint Diseases

  1. Gout and Hyperurecemia
  2. Osteoarthritis
  3. Rheumatoid Arthritis
  4. Acute coronary Syndroms

Cardiovascular Diseases

  1. Arrhymias
  2. Cardiopulmanary Resuscitation
  3. Heart Failure
  4. Hypertension
  5. Hyperlipidemia
  6. Ischemic Heart Diseases
  7. Shock
  8. Stroke
  9. Venous Thromboembolism

Dermatrologic Diseases

  1. Acne
  2. Psoriasis
  3. Skin Disorders and Cutaneous Drug Eruptions

Endocrine Diseases

  1. Cirrhosis
  2. Portal Hypertension

Gastrointestinal Diseases

  1. Irritable Bowel Syndrome
  2. Constipation
  3. Diarrhea
  4. Gastroesophagal Reflux Disease
  5. Hepatitis, Viral
    1. Hepatitis A
    2. Hepatitis B
    3. Hepatitis C
  6. Nausea and Vomiting
  7. Pancreatitis
  8. Peptic Ulcer disease

Gynecologic and Obstetric Diseases

  1. Contraception
  2. Hormone therapy

Hematologic Diseases

  1. Anemia
    1. Megaloblastic Anemia

i. Megaloblastic Anemia due to Folate Deficiency

ii. Megaloblastic Anemia due to Vitamine B12 Deficiency

    1. Sickle Cell anemia
    2. Hemolytic Anemia
    3. Iron Deficiency Anemia
    4. Aplastic Anemia
    5. Iron Deficiency Anemia

Infectious Diseases

  1. Central Nervous System infections
  2. Endocarditis
  3. Fungal infections, Invasive
  4. Gastrointestinal Infection
  5. HIV / AIDS
  6. Intra-Abdominal Infection
  7. Respiratory Tract infections, Lower
  8. Respiratory Tract infections, Upper
  9. Sepsis and Septic Shock
  10. Sexually transmited Diseases (STD)
  11. Skin and soft tissue infection
  12. Tuberculosis
  13. Urinary tract infection and prostatitis

Neurologic Diseases

  1. Epilepsy
  2. Headache
    1. Migraine
    2. Cluster Headache
  3. Pain management
  4. Parkinson’s Diseases
  5. Status epilepticus

Nutritional Diseases

  1. Enteral Nutrition
  2. Obesity
  3. Parentaral Nutrition

Onchologic Diseases

  1. Breast cancer
  2. Colorectal Cancer
  3. Lung cancer
  4. Lymphomas
  5. Prostate cancer
  6. Cervical Cancer
  7. Esophageal Cancer
  8. Gastric Cancer
  9. Head and Neck Cancer
  10. Lung Cancer
  11. Ovarian Cancer
  12. Pancreatic Cancer
  13. Primary bone Cancer
  14. Primary Brain cancer
  15. Testicular Cancer
  16. Thyroid Gland Cancer
  17. Urinar Bladder cancer
  18. Uterine Cancer

Ophtalmic Diseases

  1. Glaucoma

Psychiatric Diseases

  1. Alzhimer’s Diseases
  2. Anxiety Disease
  3. Bipolar Diseases
  4. Depressive diseases
  5. Schizophrenia
  6. Sleep Diseases
  7. Substance-Related Diseases

Renal Diseases

  1. Acid base Diseases
  2. Acute renal Failure
  3. Chronic Renal Failure
  4. Drug Dosing in renal insufficiency
  5. Electrolyte Homeostasis

Respiratory Diseases

  1. Allergic Rhinitis
  2. Asthma
  3. Chronic Obstructive Pulmonary Diseases

Urologic Diseases

  1. Benign Prostatic, Hyperplasia
  2. Erectile Dysfunction
  3. Urinary Incontinence