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Updated: May 21, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease

Published on: June 16, 2020

[Early onset pediatric sarcoidosis, diagnostic problems].

G Deverrière¹, A Flamans-Klein, D Firmin

¹Service des urgences pédiatriques, groupe hospitalier du Havre, 55 bis, rue Gustave-Flaubert, 76600 Le Havre, France.

Archives De Pediatrie : Organe Officiel De La Societe Francaise De Pediatrie

|June 2, 2012

Summary

Pediatric sarcoidosis is rare and challenging to diagnose due to non-specific symptoms. Early recognition of this multisystem inflammatory disease is crucial for timely management and avoiding long-term complications.

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Last Updated: May 21, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease

Published on: June 16, 2020

Area of Science:

Pediatric Rheumatology
Immunology
Rare Diseases

Background:

Sarcoidosis is a rare multisystem inflammatory granulomatous disease of unknown origin.
Pediatric sarcoidosis presents differently than in adults, with distinct clinical patterns based on age.
Diagnosis is challenging due to non-specific symptoms and lack of a definitive test.

Observation:

Children under 5 often present with a triad of rash, arthritis, and uveitis.
Older children may exhibit multisystem disease with lung infiltration and hilar lymphadenopathy, similar to adults.
A 4.5-year-old male initially diagnosed with juvenile rheumatoid arthritis later developed cutaneous involvement, prompting a sarcoidosis diagnosis.

Findings:

Serum angiotensin-converting enzyme (ACE) levels can be elevated in pediatric sarcoidosis, aiding diagnosis.
Histopathological examination showing epithelioid gigantocellular granuloma without caseating necrosis is the gold standard.
The case highlights delayed diagnosis due to the absence of the classical triad and lack of pulmonary involvement.

Implications:

Recognizing the unique presentations of pediatric sarcoidosis is vital for early diagnosis.
Prompt diagnosis and management can prevent long-term complications, such as steroid dependence.
Further research into pediatric sarcoidosis is needed to improve diagnostic strategies and patient outcomes.