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Without improvement of the clinical state, the boy still required oxygen and suffered from spiking fever throughout the day, with extensive sweating at night. Repeated CBC showed persistent leukocytosis (23.0 × 10⁹/L) and eosinophilia, and a new increase in CRP (106 mg/L).
As part of vasculitis work-up, the tests for autoantibodies (antinuclear antibodies, antineutrophil cytoplasmic antibodies, anti-saccharomyces cerevisiae antibodies) and serum calprotectin were normal, and screening for renal involvement did not detect any urine abnormalities, such as proteinuria or hematuria. Due to the lymphadenopathy on CT scan, the Mantoux test and the Interferon Gamma Release Assay (IGRA) test were performed to exclude tuberculosis. Furthermore, the Angiotensin Converting Enzyme (ACE) levels and Chitotriosidase levels for the diagnosis of sarcoidosis were normal for age. Lung function tests uncovered a severe reduction in lung vital capacity with 30% of the predicted value (Figure 3a). At this point, bronchoscopy was scheduled to visualize the airways, obtain samples by bronchoalveolar lavage (BAL) and to perform endobronchial biopsy for detailed tissue analysis. Simultaneously, a skin biopsy from one of the lesions on the right arm was planned.
On bronchoscopy, the macroscopic airway anatomy was described as normal, with the airway mucosa showing signs of discrete inflammatory changes and viscous secretions throughout the bronchial tree (Figure 3b). The differential leukocyte count from BAL shows 10% eosinophils (physiological value: <1%), 6% lymphocytes (physiological value: <10%) and 8% neutrophils (physiological value: revealed high number of cytomegalovirus (CMV) copies (38,000 IU/ml). Also, an elevated Candida species antigen count (157 pg/mL, physiological values: < 62.4 pg/mL), yet normal value of the Aspergillus galactomannan (0.12, physiological index
Elevated BAL levels of CMV copies with normal values in blood were highly suggestive of CMV lung infection, possibly CMV pneumonia. Serologically, there were elevated IgG levels for CMV (CMV IgG 71 U/ml, reference range 0 – 13 U/ml) but normal IgM levels for CMV (CMV IgM 14 U/ml, reference range 0 – 17 U/ml), which is not suggestive of a recent CMV primoinfection. Valganciclovir therapy was initiated at dose of 5 mg/kg/day.