Approach Considerations
Inpatient care
Patients with juvenile systemic sclerosis (JSSc) are admitted to the hospital on a limited basis, and then only to treat critical care emergencies, often in an ICU (renal crisis, cardiac and/or respiratory failure, surgical emergencies).
Other treatments should be accomplished in ambulatory care units or at home whenever possible without sacrificing patient safety.
Outpatient care
Outpatient care needs to be individualized and independent activities of daily living, good nutrition, exercise, and a healthy and positive attitude should be stressed.
The importance of continued schooling and independent living cannot be overemphasized.
Medical Care
Pharmacologic management of juvenile systemic sclerosis (JSSc) has been abysmal. The treatment of children with chronic rheumatic disease is multifaceted and requires attention to general health measures including nutrition, rest, maximizing school attendance, and exercise. Treating a child with systemic sclerosis requires a team approach, ideally including a nurse educator, physical therapist, occupational therapist, nutritionist, and social worker. No treatment or combination of medical or surgical treatments has proven unequivocally efficacious in JSSc. However, therapeutic strategies have been developed that are directed toward the individual patient and the organ systems involved in that patient.
Vascular therapy may take on several forms and is not necessarily pharmacologic. Early on, Raynaud phenomenon may respond to avoidance of tobacco, cold exposure, and vasoconstricting medications.
Biofeedback has been helpful in some patients with the development of tissue ischemia of digital tip ulcers. Local management of digital ulcers is indicated.
The arthritis of systemic sclerosis may respond to nonsteroidal anti-inflammatory drugs (NSAIDs) but to a lesser extent than the arthritis associated with other connective tissue diseases.
Surgical Care
Because involvement in patients with juvenile systemic sclerosis (JSSc) widely varies, surgical management must be individualized.
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Surgery to release contractures is occasionally indicated, and a few patients benefit from the surgical release of entrapped nerves.
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Emergency life-saving surgery in patients with juvenile systemic sclerosis who have a ruptured viscus may be required.
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Amputation should be considered only in extreme cases and if no other therapeutic options have proven effective.
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Sympathectomy as a treatment of the peripheral vascular disease is no longer used.
Consultations
The treatment of severe, chronic and debilitating pediatric diseases such as juvenile systemic sclerosis (JSSc) requires a team approach.
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The pediatric rheumatologist team leader must be a specialist experienced in the care of patients with JSSc.
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The team should also include a pediatric gastroenterologist, pediatric nephrologist, pediatric cardiologist, and pediatric pulmonologist.
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The team should also include a nurse educator, occupational therapist, physical therapist, nutritionist, and social worker.
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Telemedicine may play a role in long-distance consultation and treatment of patients with JSSc who reside far from full-service institutions.
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Recent experimental therapies that necessitate other consultations, such as stem cell, renal, cardiac, and lung transplantation, are beyond the scope of this discussion. The author does not recommend organ transplantation in any child with JSSc.
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An 8-year-old girl with overlap syndrome with evolution to progressive systemic sclerosis (PSS).
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Photo of hands revealing sclerodactyly. This demonstrates the progression of disease over 7 years.
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Chest radiograph revealing diffuse, coarse interstitial marking with bilateral lower lobe bronchiectasis.
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Axial CT scan of the chest of a 15-year-old female adolescent with progressive systemic sclerosis (PSS).
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Esophagram revealing dysmotility.
