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. 2023 Aug;43(6):1118-1121.
doi: 10.1007/s10875-023-01473-z. Epub 2023 Mar 24.

Steroid-Responsive Pulmonary Hypertension in a Pediatric Patient with Chronic Granulomatous Disease and Histoplasmosis

Leslie A Fogel  1 , R Mark Grady  1 ; SLCH Consortium; Mai He  2 , Maleewan Kitcharoensakkul  3
Collaborators, Affiliations

Steroid-Responsive Pulmonary Hypertension in a Pediatric Patient with Chronic Granulomatous Disease and Histoplasmosis

Leslie A Fogel et al. J Clin Immunol. 2023 Aug.
No abstract available

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Conflict of interest statement

The authors declare no competing interests.

Figures

Fig. 1
Fig. 1
Pathology from open lung biopsies. A The first lung biopsy showed non-necrotizing granulomas and prominent nodular fibrosis and hyphae on GMS stain (arrows). B Subsequent lung wedge resection in 1 year showed prominent nodular fibrosis suggesting “burn-out” granuloma (arrows). C Thickened wall arteries, some with occluded lumen, suggesting pulmonary hypertensive changes (arrows). Scale bar in A is 50 uM. Scale bars in B and C are 200 uM. D CT chest 6 months after the patient’s initial presentation when he had recurrence of pulmonary hypertension in the setting of histoplasmosis reactivation. The CT shows right ventricular hypertrophy and dilation, pulmonary micronodules, and ground-glass opacities. E CT chest 27 months after his initial presentation that shows clear lungs with post-surgical changes from prior open lung biopsy
See this image and copyright information in PMC

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