Esclerosis sistémica progresiva y enfermedad intersticial pulmonar aguda en niños:: a propósito de un caso

Childhood or juvenile scleroderma is the third most common pediatric chronic rheumatic disease following juvenile idiopathic arthritis (JIA) and systemic lupus erythematosus (SLE). It has two main forms: systemic and localized. The available information remains very limited, as few series have been published, and most of them include a small number of patients. On the other hand, the classification and management of the disease have evolved over time. The diagnosis of interstitial lung diseases (ILDs) in adults—and even more so in children— constitutes a significant challenge for clinicians. ILDs of inflammatory origin are rare in adults and can be considered almost exceptional in children; the largest series report no more than 50 cases. Autoimmune forms of IDLs in children represent a particularly complex category, as they involve aberrant immunological mechanisms that affect both the lungs and other organs. This can complicate diagnosis and treatment, and may worsen the prognosis. The relationship between systemic sclerosis in children and acute ILD is complex and remains under investigation. Early diagnosis, clinical assessment and appropriate treatment are essential to improve the prognosis and quality of life of affected pediatric patients. Given the particular relevance of both diseases and the limited information available in the literature, we present the case of a 6-year-old patient in whom both occurred at different stages of disease.