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Fatal Pulmonary Veno-Occlusive Disease and Systemic Juvenile Idiopathic Arthritis: Case Report and Literature Review

Silvia Escalada-Pellitero1, Alberto García-Salido2, Daniel Clemente-Garulo3

  • 1Pediatric Critical Care Unit, Hospital Infantil Universitario Niño Jesús, Madrid, Spain.

Reumatologia Clinica
|January 5, 2023
PubMed

Insights

Systemic juvenile idiopathic arthritis (sJIA) can cause severe pulmonary complications, including pulmonary hypertension. Early recognition via specific tests is crucial for managing this rare but serious risk in children with sJIA.

Area of Science:

  • Rheumatology
  • Pediatrics
  • Pulmonology

Background:

  • Systemic juvenile idiopathic arthritis (sJIA) is a severe childhood inflammatory condition with significant morbidity and mortality.
  • Pulmonary complications (PC) in sJIA, particularly pulmonary hypertension, are infrequent but life-threatening.
  • Pulmonary venous-occlusive disease is a rare but fatal complication of sJIA.

Approach:

  • A case study of a school-age female with sJIA who succumbed to pulmonary venous-occlusive disease.
  • A narrative literature review was conducted to analyze PC in sJIA.
  • Clinical evolution and necropsy findings were detailed.

Key Points:

  • Pulmonary hypertension in sJIA is linked to uncontrolled disease and biologic therapies.
  • High disease activity and polypharmacy (including disease-modifying anti-rheumatic drugs) are critical indicators for PC.
  • Chest X-ray, electrocardiogram, and echocardiogram are recommended for early diagnosis of PC.

Conclusions:

  • Pulmonary complications represent a critical diagnostic challenge in sJIA.
  • Prompt recognition and diagnostic workup for PC are essential in sJIA patients with active disease.
  • Multimodal diagnostic approaches are vital for timely intervention in sJIA-associated pulmonary hypertension.

Keywords:
Artritis idiopática juvenil sistémicaDeathDisease-modifying anti-rheumatic drugsFármacos antirreumáticos modificadores de la enfermedadHipertensión pulmonarMuertePulmonary hypertensionSystemic juvenile idiopathic arthritis

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