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Sarcoidosis of early onset. A challenge for the pediatric rheumatologist
1Children's Hospital, Department of Pediatric Rheumatology, Garmisch-Partenkirchen, Germany.
Insights
Early onset sarcoidosis, a multisystemic granulomatous disease, presents before age four with rash, polyarthritis, and uveitis. This condition has a poor prognosis, but immunosuppressive therapy may offer improvement.
Area of Science:
- Pediatrics
- Rheumatology
- Immunology
Background:
- Early onset sarcoidosis is a rare multisystemic granulomatous disease.
- It often presents before age four with characteristic symptoms.
- Misdiagnosis as juvenile chronic arthritis is common due to dominant polyarthritis.
Purpose of the Study:
- To describe the clinical presentation and long-term outcomes of sarcoidosis in children under four.
- To differentiate early onset sarcoidosis from later onset forms.
- To evaluate the impact of immunosuppressive therapy.
Main Methods:
- Retrospective case series of twelve pediatric patients with sarcoidosis.
- Follow-up duration ranged from 5 to 30 years (mean 11.5 years).
- Clinical data including symptoms, organ involvement, and treatment response were analyzed.
Main Results:
- All patients presented before age four with rash, polyarthritis, and uveitis.
- Additional symptoms included fever, hepatosplenomegaly, parotid swelling, and hypertension.
- Significant morbidity observed with cardiac (8/12) and cerebral (4/12) involvement; one death occurred.
- Severe lung changes developed in two patients, and all had sequelae.
- Some patients showed improvement with immunosuppressive therapy.
Conclusions:
- Early onset sarcoidosis is a severe, multisystemic disease with a poor prognosis and significant sequelae.
- Distinguishing it from juvenile chronic arthritis is crucial for appropriate management.
- Immunosuppressive therapy may be beneficial in managing this condition.
Abstract:
The clinical picture of twelve patients with sarcoidosis commencing under 4 years of age is presented. Follow-up ranged from 5 to 30 years with a mean of 11.5 years. Two patients were mother and daughter, the other cases were sporadic. All children developed the typical symptom triad of rash, polyarthritis and uveitis with onset before their 4th birthday. Further symptoms comprised fever, hepatosplenomegaly, parotid swelling and hypertension. Eight patients suffered from cardiac and four from cerebral involvement; one girl died from cerebral involvement at the age of 12. Two developed severe lung changes. The prognosis was poor with sequelae in all patients. Several children seem to have improved on immunosuppressive therapy. Early onset sarcoidosis is a multisystemic granulomatous disease; since polyarthritis is a dominant manifestation, many patients are diagnosed as having juvenile chronic arthritis. The early onset form must be distinguished from later onset sarcoidosis, which mainly affects children over 8 years of age or young adults. The disease types differ in their presentation, symptoms, course and prognosis; some of the clinical features and the histology, however, are identical.