Thursday, March 11, 2010

Scleroderma

Scleroderma - Chronic
Rahul Soman, M. Pharm


 


 

Definition  

An autoimmune connective tissue disease characterized by uncontrolled fibrosis affecting the skin and internal organ systems; also called systemic sclerosis


 

Medical History  

* Occupational Exposure

* Infectious disease

* Pregnancy

* Genetic predisposition


 

Findings  

* Sclerosis of the skin

* Raynaud's phenomenon

* Sclerodactyly

* Capillary finding

* Telangiectasia

* Finger ulcer

* Calcinosis

* Skin pigmentation

* Fatigue

* Heartburn

* Arthralgia

* Myalgia

* Dysphagia - Chronic

* Muscle contracture

* Nausea


 

Tests  


 

Suspected or known scleroderma  

* Capillaroscopy: Dilated loops, distorted architecture, and destruction of capillaries are findings on microscopic examination of the nailfold in scleroderma .


 

Suspected scleroderma (systemic sclerosis)  

* ANA measurement: A positive antinuclear antibody (ANA) test result occurs in approximately 60% to 90% of patients with systemic sclerosis .


 

Suspected and known CREST syndrome (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia)  

* Antibody to centromere measurement: A positive anti-centromere antibody finding is suggestive of CREST syndrome .


 

Raynaud phenomenon (RP)  

* Antibody to centromere measurement: A positive anti-centromere antibody (ACA) may suggest Raynaud phenomenon (RP) .


 

Suspected and known systemic sclerosis (scleroderma)  

* Antibody to centromere measurement: A positive anti-centromere antibody (ACA) finding by immunofluorescence is associated with systemic sclerosis .


 

Suspected systemic sclerosis (scleroderma)  

* Antibody to Scl-70 measurement: Higher levels of anti-Scl-70 antibody are helpful to differentiate systemic sclerosis from systemic lupus erythematous .


 

Suspected mixed connective tissue disease (MCTD)  

* RNP antibody measurement: A positive anti-nuclear ribonucleoprotein (anti-RNP) result is required for the diagnosis of mixed connective tissue disease (MCTD) and a negative result practically excludes MCTD .


 

Suspected interstitial lung disease  

* Pulmonary function test: Pulmonary function studies often show evidence of restrictive lung disease but may also appear normal or show evidence of obstructive lung disease.


 

To exclude or characterize the contribution of underlying lung disease in the initial evaluation of all patients with pulmonary hypertension  

* Pulmonary function test: Pulmonary function testing is a necessary part of the initial evaluation of all patients with pulmonary hypertension .


 

Suspected and known scleroderma  

* Echocardiography: Comprehensive echocardiography may detect pulmonary artery hypertension and impaired cardiac function early in the course of systemic sclerosis .


 

Suspected and known scleroderma  

* CT of chest: High-resolution CT of the chest is a valuable procedure for the assessment of pulmonary and esophageal involvement in systemic sclerosis .


 

Suspected and known scleroderma  

* 12 lead ECG: In scleroderma, a 12-lead ECG may show evidence of dysrhythmias, ischemia, ventricular dysfunction, and pulmonary artery hypertension .


 

Suspected and known scleroderma  

* Plain chest X-ray: Bibasilar pulmonary fibrosis seen on chest radiograph is a minor criterion in the diagnosis of scleroderma . Chest radiograph may also show evidence of pulmonary artery hypertension .


 

Differential Diagnosis  

* Mixed connective tissue disease

* Undifferentiated connective tissue disease

* AL amyloidosis

* Complex regional pain syndrome, type I

* Fasciitis with eosinophilia syndrome

* Graft-versus-host disease, chronic

* Porphyria cutanea tarda

* Scleredema

* Scleromyxedema

* Acute scleroderma renal crisis

* Systemic sclerosis, diffuse

* Systemic sclerosis with limited cutaneous involvement

* CREST syndrome

* Localized scleroderma


 

Treatment  


 

Drug Therapy  


 


 

Raynaud phenomenon in scleroderma patients  


 

NIFEDIPINE  

Adults: 10 mg orally 3 times a day


 

LOSARTAN POTASSIUM  

Adults: 50 mg orally once daily


 

ILOPROST  


 


 


 

Scleroderma renal crisis  


 

CAPTOPRIL  

Adults: 12.5 to 100 mg/day orally in divided doses


 

ENALAPRIL MALEATE  

Adults: 5 to 15 mg orally once daily


 


 

Interstitial lung disease in scleroderma patients  


 

CYCLOPHOSPHAMIDE (Related toxicological information in CYCLOPHOSPHAMIDE AND RELATED AGENTS)  

Adults: 1 to 2 mg/kg/day orally


 


 

Pulmonary hypertension in scleroderma patients  


 

SILDENAFIL CITRATE  

Adults 50 mg orally 2 or 3 times a day


 

BOSENTAN  

Adults: Initial dose 62.5 mg orally twice daily for 4 weeks; maintenance dose up to 125 mg orally twice daily; liver function should be tested before initial administration and then monthly.


 

ILOPROST  


 


 

EPOPROSTENOL SODIUM  


 


 


 

Limited or diffuse systemic sclerosis  


 

METHOTREXATE SODIUM  

Adults: 15 to 25 mg orally once weekly


 

COLCHICINE (Related toxicological information in COLCHICINE)  

Adults: 0.6 mg orally twice daily


 

Procedural Therapy  


 

Scleroderma renal crisis  

* Renal dialysis


 

Non-Procedural Therapy  


 

Scleroderma  

* Patient Education


 

 
 

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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