Publication Date

11-2024

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Abstract

IgA Vasculitis is a commonly seen presentation in the pediatric population. The constellation of symptoms typically includes purpuric rash, joint pain, abdominal pain and hematuria. This 12 year old female was seen by her pediatrician for rash on her legs, shortness of breath, cough and night sweats that all started around 2 months ago. Additionally, a 25 pound weight loss was noted over the past year. The rash appeared as nonblanching purpura on her lower extremities, lending suspicion to IgA Vasculitis. She was first treated for UTI with cefuroxime, and then treated with steroids. Urinalysis showed persistent hematuria and she was referred to nephrology. At her first visit, she was sent to the Emergency Department for tachypnea and increased work of breathing. Baseline labs showed normal electrolytes and kidney function, elevated inflammatory markers (CRP of 19.1 and ESR of 26), and mild leukocytosis. Urinalysis showed 3+ blood and 3+ protein. Chest CT demonstrated extensive bilateral peribronchial cuffing, multifocal bronchiectasis, scattered upper lobe airspace disease, and a diffuse treein-bud nodular pattern. Initial differential included vasculitis, disseminated tuberculosis or fungal infection, or primary pulmonary disease. Ultimately, multiple specialty services were consulted. Rheumatology workup included ANCA vasculitis panel, ANA, C3, C4 and IgG levels, all negative. Nephrology obtained a kidney biopsy, which was consistent with IgA Nephropathy. Infectious Diseases requested multiple AFB sputum cultures and gastric aspirates, along with Quantiferon Gold and Fungal cultures, with the only positive finding being Pseudomonas aeruginosa growth in the sputum. Lastly, Pulmonology recommended sweat chloride tests, both positive; so whole exome sequencing was obtained and showed 2 pathogenic mutations in the CFTR gene, confirming a diagnosis of cystic fibrosis. The final diagnosis was Cystic Fibrosis with chronic Pseudomonas infection and secondary IgA vasculitis and Trikafta. Her PFTs continue to improve and she has not re quired readmission to the hospital since the nephritis. She was treated with pulse steroids, IV antibiotics and eventually started on Cellcept and diagnosis was made. While this patient did have a secondary IgA Vasculitis, not all of her symptoms were typical. Severe lung involvement is uncommon in IgA Vasculitis and requires additional workup. Additionally, while cystic fibrosis is well diagnosed with newborn screening, it should be considered in appropriate clinical scenarios when newborn screening was not obtained.

Disciplines

Pediatrics

Notes

Presented at the American College of Rheumatology (ACR) Convergence 2024; Washington, D.C.; November 14-19, 2024.

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