Overview of Treatment

Overall, treatment of the scleroderma patient is focused on the rapid reduction of inflammation, prevention and treatment of fibrosis, and organ-specific therapy.

Immunosuppressive medications such as methotrexate and cyclophosphamide are used in relatively high doses to quickly reduce the inflammatory process in the early stages of diffuse SSc. Despite the usual side effects of these medications early in treatment, the disease can be quieted and patients gain significant relief from debilitating signs and symptoms.

Corticosteroids in doses higher than 10-15 mg per day have been found to be counterproductive in patients with diffuse disease, sometimes precipitating acute SRC. They sometimes are used for joint or tendon symptoms.¹

The calcium channel blockers, such as nifedipine, are useful in opening blood vessels in the skin and heart. ACE inhibitors have been used to good effect in the kidney, and bosentan or epoprostenol improve blood flow in the lungs. Antiplatelet therapy (low-dose aspirin) can help prevent clotting, allowing flow better through partly occluded vessels.¹ These medications are augmented by warm clothing including gloves, even in the hot months, small packets of hand warmers and immediate smoking cessation.

GERD can be treated with protein pump inhibitors, raising the head of the bed with books or bricks and teaching the patient how to take medications to reduce gastric irritation. A small number of patients will need total parenteral nutrition to maintain nutrition. Finally, antibiotics are used to treat the bacterial overgrowth and malabsorption that results from lower bowel gastroparesis.

In general, patients with scleroderma should be treated by rheumatologists as their primary specialist, although other specialists clearly are needed as contributors to overall care. Nurses are important members of the care team. In clinics where large numbers of scleroderma patients are seen, the nurse may be a resource for patient and family education and emotional support.

Indications for Rapid Referral & Treatment

Early diffuse disease with rapidly advancing skin involvement, acute onset high blood pressure, seizures, new proteinuria and an early decrease in pulmonary function are all signals the patient may be in trouble.^1,5

These serious symptoms of scleroderma may be seen within the first 3-5 years after diagnosis, and any one of them signals the patient should be seen for rapid workup and treatment. For SRC, hospitalization is the rule.¹ Early intervention in SRC is crucial to reduce morbidity and mortality in this potentially catastrophic complication of SSc.

Patient Education & Support

"The role of the nurse, regardless of the setting, is a multifaceted one: caregiver, health educator and referral liaison for both medical and ancillary care, and a critical link between physician, patient and family."¹

Nursing care of patients with Raynaud's phenomenon (95 percent of SSc patients) centers around education regarding keeping warm, medications and modifications in care when pain, infection and ulcerations occur. It is important for patients to understand when there is an ischemic episode in the extremities there is sometimes demonstrable ischemia in the lungs, heart and kidneys; therefore, keeping warm, even in the summertime, maintaining their medications and stopping smoking will protect not only the skin but also the internal organs.

When digital ulcers occur, wound care specialists can be consulted for treatment options. It is important to wear gloves when cleaning house to protect the hands from harsh chemicals, bumps and abrasions as much as possible.

The Skin

Since SSc's most prominent sign is hardening of the skin, patients have great difficulties with this area. Not only is it painful, it also is disfiguring and debilitating, causing the typical "mouse face" as well as telangiectasias, clawed fingers, joint pain and itching from the entrapment of superficial pain nerves. Skin breakdown can occur from excessive dryness.

It must be emphasized that only time and immunosuppressives have been found to actually soften the skin, not specific moisturizers or massage, though they can feel good and may be preventive.

Telangiectasias, visible as small red or purple spots on the fingers, palms, face and lips can be covered with special cosmetics, which are waterproof and not perfumed.