Health Concerns

Scleroderma

Diagnosis and Conventional Treatment

Physicians may suspect scleroderma based on presentation of the characteristic signs and symptoms mentioned previously. Although there is no specific test for scleroderma, a diagnostic workup may include a detailed medical history, laboratory findings, and skin biopsy (NIAMS 2010; Nashel 2012; Khoo 2011).

Doctors may test for autoantibodies against topoisomerase (Scl-70), centromere-associated proteins, and nuclear antigens (ANA); this can aid in diagnosis but must be supported by other evidence (Grassegger 2008). Rheumatoid factor, another autoantibody, may be present in some patients as well. Abnormalities on routine lab tests may help identify involvement of specific organs (Ferri 2013). Elevated levels of the inflammatory marker C-reactive protein (CRP) have been associated with worse outcomes in some patients and may help influence treatment decisions (Muangchan 2012). Diagnosing scleroderma can be difficult due to the significant variability in disease presentation.

Scleroderma has no known cure. There are a variety of medications that can help palliate symptoms and reduce complications of scleroderma, but medications that modify the course of the disease are lacking (Kowal-Bielecka 2009; Opitz 2011; Baumhakel 2010; Gayraud 2007).

Treatments for Organ-Specific Complications of Scleroderma (Hinchcliff 2008)

Complication

Treatment

Raynaud's phenomenon

  • Drugs that can improve blood flow:
    • α – Adrenergic blockers
    • Angiotensin-II receptor blockers
    • Long-acting calcium channel blockers (dihydropyridines)
    • Pentoxifylline (Trental®)
  • Digital sympathectomy (surgery that interrupts the small nerves to the arteries feeding the finger)

Skin fibrosis

Immunomodulatory drugs (D-penicillamine [Cuprimine®], mycophenolate mofetil [Cellcept®], cyclophosphamide [Cytoxan®])

Gastroesophageal reflux disease (GERD)

  • Antacids
  • Drugs that reduce stomach acid:
    • Histamine H2 blockers
    • Proton pump inhibitors

Intestinal dysmotility and/or bacterial overgrowth

  • Antibiotics
  • Correction of nutritional deficiencies
  • Promotility agents

Pulmonary (lung) fibrosis or alveolitis

cyclophosphamide (Cytoxan®; an immune-suppressing drug)

Pulmonary arterial hypertension

  • Diuretics
  • Supplemental oxygen
  • Drugs that minimize blood vessel clots (Warfarin [Coumadin®])
  • Drugs that improve blood flow including:
    • Endothelin-1 receptor inhibitors (bosentan [Tracleer®])
    • Phosphodiesterase-5 inhibitors (sildenafil [Revatio®])
    • Prostacyclin analogues (epoprostenol [Flolan®], treprostinil [Remodulin®], iloprost [Ventavis®])

Scleroderma renal crisis

  • Dialysis
  • Blood pressure lowering drugs (eg, short-acting angiotensin-converting enzyme inhibitors)

Side effects of conventional treatment vary with medication, and may include fluid buildup in the feet, constipation and gastrointestinal disturbances, fatigue, flushing, allergic symptoms, gingivitis (inflammation of the gums), and erectile dysfunction. Steroids such as prednisone are frequently used for their anti-inflammatory action. However, steroids have significant side effects, including loss of bone density and weight gain.