Figure 5.

Evolution of severe organ involvements in 52 patients from first pediatric to last adult evaluations. Severe sarcoidosis were defined as: (i) lung involvement with Composite Physiologic index (CPI) > 40 and/or pulmonary hypertension and/or pulmonary fibrosis extent at computed tomography (CT) of more than 20% of the lung [15]; (ii) evidence of central nervous system sarcoidosis requiring a treatment; (iii) heart involvement with spontaneous sustained ventricular tachycardias, arrythmias requiring defibrillator, conduction block requiring pacemaker, heart failure with left ventricular ejection fraction under 40%; (iv) liver involvement requiring a transplantation; (v) kidney involvement requiring chronic dialysis or transplantation. A: Five patients with initial severe lung involvement (CPI > 40), improved (normal pulmonary function and thoracic radiography). B: Two patients with antehypopituitarism that resolved under treatment, one patient with sequels as facial peripheric palsy and hypopituitarism requiring hormonal supplementation. C: one patient with renal, ocular, hepatic sarcoidosis diagnosed at five years old with multiple relapses that led to renal failure at eighteen years old, requiring dialysis. D: one patient with subnormal pulmonary function in pediatric follow-up who experiences worsening in adulthood with lung fibrosis and pulmonary aspergillosis. E: Two patients with uveitis in pediatric follow-up (and pulmonary involvement) who presented neurosarcoidosis at adulthood evaluation (one and four years after onset of disease, respectively).