Paediatric rheumatologic emergencies: clinical spectrum and outcomes from a 3-year cohort study

Paediatric rheumatologic emergencies are rare but potentially fatal, and may present as the first manifestation of an underlying rheumatic disease. These conditions frequently present with nonspecific constitutional or organ-specific symptoms, posing a diagnostic challenge-particularly in children without a prior diagnosis. The objectives of this study were to evaluate the clinical characteristics and outcomes of paediatric rheumatological emergencies, comparing patients with and without known pre-existing rheumatologic conditions, and secondarily to suggest warning signs that may aid in the recognition of these situations. We conducted a retrospective cohort study over three-years at a tertiary paediatric rheumatology centre in western India. Medical records of all children (<18 years) diagnosed with rheumatologic diseases were reviewed. Demographic, clinical, and outcome data were analysed. Emergencies were defined as life-threatening or organ-threatening events requiring urgent evaluation or intensive care. Descriptive and comparative analyses were performed to evaluate outcomes between first-presentation and known rheumatic disease groups. Of 420 patients screened, 41 (9.8%) experienced rheumatologic emergencies; 30 (73.2%) presented as the first manifestation of disease. Common symptoms included fever (58.5%), dyspnea (46.3%), and seizures (17.1%). Major organ involvement included cardiovascular (31.7%), hematologic (26.8%), and central nervous (19.5%) systems. The most frequent acute emergencies were Macrophage Activation Syndrome (MAS) (23.4%), heart failure (19.15%), and respiratory failure (17.02%). Systemic Lupus Erythematosus (SLE) (51.06%) was the most common final diagnosis. Overall, 87.8% of patients recovered, while 12.2% succumbed, predominantly due to diffuse alveolar haemorrhage (DAH) and MAS.

Paediatric rheumatic diseases can present as life-threatening emergencies, frequently as the first manifestation of illness. Systemic lupus erythematosus was the most common underlying diagnosis in our cohort. Early recognition and timely initiation of immunosuppression may improve outcomes.

• Paediatric rheumatologic diseases can rarely present as acute, life-threatening emergencies. Their nonspecific symptoms often mimic infections or malignancy, leading to diagnostic delays and preventable mortality, particularly in low-resource settings with limited rheumatology expertise.

This study characterizes real-world pediatric rheumatologic emergencies and provides practical red-flag indicators for early recognition and timely immunosuppression to improve survival in resource-limited settings.