Treatment

• Raynaud’s phenomenon, ulceration and gangrene
• Skin conditions
• Facial problems
• Inflammation and stiffness of the joints
• Muscle weakness and myositis
• Gastrointestinal complications
• Cardiac complications
• Renal complications
• Lung complications

The course of scleroderma is unpredictable and varies, the prognosis being worse for those with early organ complications such as kidney, heart and lung involvement.

However, “there have been significant recent advances in the management of scleroderma, mainly related to improved treatment of organ-based complications and to the appreciation that successful management depends upon accurate diagnosis, sub-setting, staging within the subset and screening for specific complications. Many advances have occurred in parallel with the improved treatment of other medical conditions such as hypertension and gastro- oesophageal reflux disease.” (Ref: Oxford textbook of Medicine 4th Edition)

There is currently no curative treatment. However, there are many measures which help control the disease and its complications, including multidisciplinary team interventions. As Scleroderma is a rare, complex disease, in severe cases, often treatments in specialist centres can include the use of off-label and non-licensed drugs.

A list of these is as follows:

Raynaud’s phenomenon, ulceration and gangrene

A person may have up to 20 to 40 attacks of Raynaud’s phenomenon per day.

• Hand-warmers and avoidance of precipitating factors such as cold and stress
• Management of associated complications such ulcerations and calcinosis as a priority
• Removal of precipitating factors such as beta blockers if possible
• Vitamins, fish oils and evening primrose oil
• Vasodilators such as:
  • Calcium channel blockers (e.g. Nifedipine, Amlodipine, felodipine, Diltiazem, Nicardipine))
  • ACE Inhibitors (e.g.Captopril, Enalapril, Quinapril)
  • Alpha- adrenoreceptor blocking drugs (e.g. Prazosin),
  • SSRIs (Selective Serotonin Reuptake Inhibitors) e.g. Fluoxetine, Paroxetine,
  • Peripheral vasodilators (e.g. Thymoxamine, Nafidrofuryl, Pentoxyfylline, Inositol nicotinate)
  • Topical vasodilators (e.g. GTN patches or creams)
• Prostacyclin infusion (Iloprost) in severe refractory cases.
• Endothelium Receptor Antagonists (in new presenting ulceration in the digits refractory to conventional therapies).

Skin conditions

• Oil- based creams, sunscreens, humidifiers,
• Warmth and exercise (to aid blood flow)
• With rapid progressive changes, high dose immunosuppressive and steroids should be considered
• Dental care, drinks and saliva substitutes for small, dry mouth

Facial problems

• Facial exercises and physiotherapy to help keep facial muscles supple

Inflammation and stiffness of the joints

• Physiotherapy and exercises to maintain suppleness of joints (especially in the hands)
• Associated skin lubricants to prevent contractures
• Aspirin and Non-Steroidal Anti-inflammatory Drugs (NSAIDs) for joint pain
• Rest when joints are inflamed

Muscle weakness and myositis

• Steroids may be necessary
• Methotrexate
• Azathioprine
• Immunoglobulin

Gastrointestinal complications

• Antacids and proton-pump inhibitors or H2-blockers to block stomach acid production
• Endoscopy may rectify reflux or stricture of the oesophagus although oesophageal dilatations may be needed
• Nutritional supplements and antibiotics for malabsorption and bacterial overgrowth in the bowel
• Modify stool consistency with appropriate medication (e.g. Fybogel)

Cardiac complications

• Pacemaker for arrhythmias
• NSAID’s for pericarditis
• Cardiomyopathy and cardiac failure – appropriate treatment, such as digoxin, spironolactone, loop diuretics
• Myocarditis – appropriate treatment

Renal complications

• Hypertension or hypertensive crisis arising from kidney disease [and subsequent rapidly developing kidney failure] requires intensive control of blood pressure; drug of choice is ACE-Inhibitor; dialysis may be necessary.

Lung complications

• Pulmonary hypertension – use oxygen, oral anticoagulants, calcium channel blockers and Prostacyclin infusion and oral endothelin receptor antagonists
• Pulmonary fibrosis (Fibrosing Alveolitis) – use (only in those with active inflammatory disease) immunosuppression such as cyclophosphamide, Azathioprine, with small doses of steroids to protect the kidneys from renal crisis.

Amended November 2008