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