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Table 31.

Treatment Options for Common Manifestations of Systemic

Sclerosis

Manifestations Treatment

Cutaneous Methotrexate, mycophenolate, cyclophosphamide, and rituximab have been

manifestations shown to improve skin scores; for pruritus, antihistamines can be tried; low-dose
prednisone can also help; laser therapy can used for telangiectasias.

Musculoskeletal NSAIDs, intra-articular or low-dose prednisone, hydroxychloroquine, or

manifestations methotrexate can be used for arthralgia/arthritis; methotrexate, azathioprine, or
mycophenolate can be used for significant myositis.

Raynaud Cold avoidance; gloves; central warmth

phenomenon
Calcium channel blockers

Sildenafil; losartan; prazosin

Topical nitrates; low-dose aspirin

Digital sympathectomy

Gastroesophageal Avoid eating close to bed time; head of bed elevation

reflux disease
H2 blockers; proton pump inhibitors

Surgical fundoplication

Gastrointestinal Metoclopramide (avoid long-term use); domperidone (limited access program)

dysmotility
Erythromycin; cisapride (limited access program)

Gastric antral Ablation laser therapy

vascular ectasia
 Small intestine Rotating antibiotics
bacterial
overgrowth If fat malabsorption, cholestyramine

Pseudo- A promotility agent such as domperidone can be tried

obstruction
If resistant, octreotide may be useful

Constipation Fiber; stool softeners

Polyethylene glycol

Scleroderma renal ACE inhibitors (typically captopril)

crisis
Dialysis

Interstitial lung Mycophenolate mofetil

disease
Cyclophosphamide

Lung transplantation

Pulmonary arterial Supplemental oxygen; treat right-sided heart failure

hypertension
Prostanoids; endothelin receptor antagonists; phosphodiesterase-5 inhibitors;
soluble guanylate cyclase inhibitors

Combination therapy

Lung transplantation