scleroderma
SCLERODERMA
Scleroderma is a connective tissue disorder characterized by skin thickening and fibrosis. It is rare, autoimmune and chronic.
It has an idiopathic cause and not contagious.
Early microvascular damage, mononuclear cell infiltrates and slowly developing fibrosis are the important features of the tissue lesions.
The leading causes of death are pulmonary fibrosis and pulmonary arterial hypertension.

Principles of Therapy

  • No drug has been clearly proven that would stop or reverse the skin thickening & tightening manifestation of scleroderma
  • Primarily for symptomatic treatment & prevention of complications of scleroderma

Pharmacotherapy

Dermatological symptoms

Topical therapy

  • First line option for dermal (superficial) & localized dermatological manifestations of scleroderma
  • Topical Tacrolimus
    • Effective for active, inflammatory morphea
  • High potency corticosteroids (eg Triamcinolone)
    • Effective treatment for morphea & adjuvant therapy for recalcitrant lesions in patients receiving phototherapy or systemic treatment
    • Treatment should be discontinued if no response has been observed after 10-12 wks of use as it can cause renal crisis
  • Topical Vitamin D
    • Inhibits fibroblast proliferation, collagen synthesis & activation of T lymphocyte
    • Treatment combination of Calcipotriol & Betamethasone diproprionate ointment showed improvement in skin lesions

Systemic therapy

  • Methotrexate w/ or w/out systemic glucocorticoids
  • For severe, disabling, progressive deep lesions
    • Methotrexate is the most appropriate systemic therapy for morphea
  • Immunomodulatory drugs
    • Eg D-Penicillamine, Mycophenolate mofetil, Cyclophosphamide
    • For the treatment of skin fibrosis in localized scleroderma & systemic sclerosis

Raynaud’s phenomenon

  • Calcium Channel Blockers
    • Eg Amlodipine, Diltiazem, Felodipine, Isradipine, Nifedipine
    • Standard drugs to open blood vessels & improve circulation
  • ACE Inhibitors
    • Eg Captopril
    • May help improve the local blood flow in Raynaud’s phenomenon
  • Prostacyclin Analogues
    • Eg Alprosdatil, Iloprost
    • Dilates the blood vessels & have anti-clotting properties
  • Phosphodiesterase-5 (PDE-5) Inhibitors
    • Eg Sildenafil, Tadalafil, Vardenafil
    • For improvement of the peripheral & pulmonary circulation
  • Endothelin Receptor Antagonists
    • Eg Bosentan
    • Reduces incidence of digital ulceration & ischemia

Gastrointestinal symptoms

  • Proton Pump Inhibitors
    • Eg Esomeprazole, Lansoprazole, Omeprazole, Pantoprazole, Rabeprazole
    • Treatment for gastric reflux symptoms of scleroderma
    • Acid reducing agents that would reduce the risk of having esophageal strictures
    • See Gastroesophaegeal Reflux disease management chart for more information
  • Prokinetic Agents
    • Eg Metoclopramide
    • Used for the management of esophageal hypomotility symptoms

Pulmonary symptoms

  • Immunosuppressants
    • Eg Azathioprine, Cyclophosphamide
    • Treatment of scleroderma-associated interstitial lung disease
    • Usually combined w/ glucocorticoids to prevent further lung deterioration in patients w/ fibrosing alveolitis
  • Glucocorticoids
    • Generally administered in combination w/ Cyclophosphamide in the treatment of pulmonary fibrosis
    • Monotherapy is not recommended as it can increase the risk of renal crisis
  • Endothelin Receptor Antagonists
    • Bosentan is approved for treatment of pulmonary hypertension
    • It antagonizes endothelin which mediates vasoconstriction 
    • See also Pulmonary Arterial Hypertension chart for more information
  • Phosphodiesterase-5 Inhibitors
    • Eg Sildenafil, Tadalafil
    • Used in the treatment of early stage of pulmonary hypertension
    • See also Pulmonary Arterial Hypertension disease management chart for more information
  • Prostacyclin Analogues
    • Eg Alprodastil, Epoprostenol, Iloprost
    • Primary agents for treating pulmonary hypertension
    • Epoprostenol works by relaxing blood vessels & increasing blood to the lungs

Musculoskeletal symptoms

  • Nonsteroidal Anti-Inflammatory Drugs (NSAIDs)
    • Eg Ibuprofen
    • Used to relieve pain, inflammation, swelling & stiffness
  • Low-Dose Corticosteroids
    • Eg Prednisone
    • Used to lessen swelling, redness, itching & allergic reactions
    • Also used in patients w/ tenosynovitis & refractory arthritis
  • Immunosuppressants
    • Eg Methotrexate
    • Used to treat inflammatory arthritis

Renal symptoms

  • ACE Inhibitors
    • Eg Captopril
    • First line antihypertensive agent used to prevent renal crisis

Non-Pharmacological Therapy

Phototherapy

  • First line of therapy for localized scleroderma & to relieve skin thickening
  • Ultraviolet (UV) light therapy
    • UVA 1 is most preferred but if not available may use broad band UVA or narrow band UVB
    • Studies shows that it helps in the production of enzymes that promotes collagen breakdown as well as UV-induced immunomodulation
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