Lung disease (LD) is an increasingly recognized complication of systemic juvenile idiopathic arthritis (sJIA). As there are no currently available guidelines for pulmonary screening in sJIA, we ...sought to develop such an algorithm at our institution.
A multidisciplinary workgroup was convened, including members representing rheumatology, pulmonary, stem cell transplantation, and patient families. The workgroup leaders drafted an initial algorithm based on published literature and experience at our center. A modified Delphi approach was used to achieve agreement through three rounds of anonymous, asynchronous voting and a consensus meeting. Statements approved by the workgroup were rated as appropriate with moderate or high levels of consensus. These statements were organized into the final approved screening algorithm for LD in sJIA.
The workgroup ultimately rated 20 statements as appropriate with a moderate or high level of consensus. The approved algorithm recommends pulmonary screening for newly diagnosed patients with sJIA with clinical features that the workgroup agreed may confer increased risk for LD. These "red flag features" include baseline characteristics (young age of sJIA onset, human leukocyte antigen type, trisomy 21), high disease activity (macrophage activation syndrome MAS, sJIA-related ICU admission, elevated MAS biomarkers), respiratory symptoms or abnormal pulmonary examination findings, and features of drug hypersensitivity-like reactions (eosinophilia, atypical rash, anaphylaxis). The workgroup achieved consensus on the recommended pulmonary work-up and monitoring guidelines.
We developed a pulmonary screening algorithm for sJIA-LD through a multidisciplinary consensus-building process, which will be revised as our understanding of sJIA-LD continues to evolve.
BACKGROUND Kikuchi-Fujimoto disease (KFD) is a rare self-limited necrotizing lymphadenitis which is likely under-diagnosed in pediatric patients who present with fever of unknown origin and ...lymphadenopathy. Definitive diagnosis is challenging as it requires an invasive open lymph node biopsy or lymph node needle aspiration cytology that shows pathologic findings of histiocytic necrotizing lymphadenitis. CASE REPORT We report the case of one of the youngest patients diagnosed with KFD in the United States, at the age of 7 years. KFD has a higher prevalence in patients of Asian descent, but this patient was an African American. This case report shows the often convoluted and complicated course these patients undergo with their presenting complaints of fever of unknown origin and lymphadenopathy and highlights particular clinical findings that suggest KFD. CONCLUSIONS This patient is one of the youngest persons diagnosed with KFD in the United States, with an atypical ethnic background. It is likely that KFD is under-recognized and under-diagnosed in this population. With a broad differential diagnosis for fever of unknown origin and lymphadenopathy, awareness of KFD as a potential diagnosis may reduce other unnecessary investigations. The increased risk of patients with KFD of developing systemic lupus erythematosus (SLE) accentuates the importance of an accurate diagnosis and appropriate referral for heightened surveillance after recovery.
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