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Review
. 2024 Jun;54(7):1059-1074.
doi: 10.1007/s00247-024-05962-0. Epub 2024 Jun 8.

Connective tissue disease-associated lung disease in children

Andrew H Schapiro  1   2 , Cara E Morin  3   4 , Kathryn A Wikenheiser-Brokamp  5   6   7   8 , Aki A Tanimoto  3   4
Affiliations
Review

Connective tissue disease-associated lung disease in children

Andrew H Schapiro et al. Pediatr Radiol. 2024 Jun.

Abstract

Connective tissue diseases are a heterogeneous group of autoimmune diseases that can affect a variety of organ systems. Lung parenchymal involvement is an important contributor to morbidity and mortality in children with connective tissue disease. Connective tissue disease-associated lung disease in children often manifests as one of several radiologic-pathologic patterns of disease, with certain patterns having a propensity to occur in association with certain connective tissue diseases. In this article, key clinical, histopathologic, and computed tomography (CT) features of typical patterns of connective tissue disease-associated lung disease in children are reviewed, with an emphasis on radiologic-pathologic correlation, to improve recognition of these patterns of lung disease at CT and to empower the pediatric radiologist to more fully contribute to the care of pediatric patients with these conditions.

Keywords: Children; Computed tomography; Connective tissue disease; Lung; Pathology; Pediatric.

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Conflict of interest statement

The authors declare no competing interests.

Figures

Fig. 1
Fig. 1
Nonspecific interstitial pneumonia (NSIP) histopathology. a Cellular NSIP with characteristic diffuse alveolar septal expansion by chronic inflammatory cells and minimal fibrosis (H&E, 100×). b Fibrotic NSIP with diffuse, temporally uniform alveolar septal expansion by fibrosis without significant architectural distortion, as well as mild chronic inflammation (H&E, 40×)
Fig. 2
Fig. 2
13-year-old female with systemic sclerosis. a, b Axial CT images without contrast showing subpleural ground-glass opacity, mild reticulation, and mild traction bronchiolectasis in the lower lobes and anterior upper lobes (black arrows) suggesting nonspecific interstitial pneumonia. c, d Follow-up axial CT images without contrast at 19 years of age showing increased reticulation and traction bronchiolectasis suggesting worsening fibrosis
Fig. 3
Fig. 3
Lymphoid interstitial pneumonia histopathology. Characteristic dense cellular infiltrate expanding the alveolar septa (arrow) with a peribronchiolar lymphoid follicle (*) and an adjacent cystically dilated space (**) (H&E, 40×)
Fig. 4
Fig. 4
14-year-old female with Sjogren syndrome. Axial CT image with contrast showing extensive ground-glass opacity, peribronchovascular cysts (black arrow), ill-defined centrilobular nodules (white arrow), and interlobular septal thickening (arrowhead) suggesting lymphoid interstitial pneumonia. Right middle lobe collapse and bronchial dilation are also present (open arrow)
Fig. 5
Fig. 5
Organizing pneumonia histopathology. Branching fibroblastic tissue with a myxoid stroma filling distal airspaces (arrows) associated with a mild chronic inflammatory infiltrate characteristic of organizing pneumonia (H&E, 200×)
Fig. 6
Fig. 6
20-year-old male diagnosed with dermatomyositis when he was 17 years old. a, b Axial and (c) coronal CT images with intravenous contrast showing areas of peripheral perilobular opacity (black arrows) and reversed halo sign (white arrow) in the lower lobes and anterior left upper lobe suggesting organizing pneumonia
Fig. 7
Fig. 7
Diffuse alveolar damage (DAD) histopathology. a Acute DAD with hyaline membranes (black arrow) and intra-alveolar edema admixed with red blood cells (*) resulting from capillary injury (H&E, 400×). b Organizing DAD with interstitial fibrosis (**) and alveolar epithelial cell hyperplasia (white arrow) (H&E, 400×)
Fig. 8
Fig. 8
18-year-old female diagnosed with systemic lupus erythematosus when she was 16 years old presenting acutely. Axial CT image with intravenous contrast showing extensive bilateral ground-glass opacity and consolidation suggesting acute lupus pneumonitis
Fig. 9
Fig. 9
Histologic spectrum of systemic juvenile idiopathic arthritis-associated pulmonary alveolar proteinosis and endogenous lipoid pneumonia. a Pulmonary alveolar proteinosis with predominant proteinaceous material (*) with few cholesterol clefts (arrow) filling alveolar spaces (H&E, 200×). b Mixed features of pulmonary alveolar proteinosis and endogenous lipoid pneumonia (H&E, 200×). c Endogenous lipoid pneumonia with more abundant macrophages and cholesterol clefts than typically seen in pulmonary alveolar proteinosis along with an associated fibroinflammatory response (**) (H&E, 200×)
Fig. 10
Fig. 10
11-year-old male with systemic juvenile idiopathic arthritis. a, b Axial CT images without intravenous contrast showing mild subpleural consolidation in the anterior upper lobes and peripheral lower lobes (black arrows) as well as mild peribronchovascular consolidation in the lower lobes (white arrows). c Histologic image showing subpleural (black arrow) and peribronchiolar (white arrow) lung involvement (H&E, 40×)
Fig. 11
Fig. 11
2-year-old male with systemic juvenile idiopathic arthritis. a, b Axial CT images with intravenous contrast showing ground-glass opacity, interlobular septal thickening, and intralobular lines in a crazy paving pattern in a subpleural distribution in the anterior upper lobes and peripheral lower lobes (black arrows), and in a peribronchovascular distribution in the lower lobes (white arrows). c, d Follow-up axial CT images with intravenous contrast at 5 years of age showing extensive consolidation at sites of prior ground-glass opacity and interlobular septal thickening
Fig. 12
Fig. 12
4-year-old female with systemic juvenile idiopathic arthritis. a Axial CT image without intravenous contrast showing widespread interlobular septal thickening. b Follow-up axial CT image without intravenous contrast at 5 years of age showing diffuse ground-glass opacity, new subpleural cysts (arrow), and less pronounced septal thickening. c Histologic image showing subpleural cystic spaces (black arrows) containing proteinaceous material (H&E, 40×)
Fig. 13
Fig. 13
13-year-old female with mixed connective tissue disease. a, b Axial CT images without intravenous contrast showing ground-glass opacity, reticulation, and bronchiolectasis in the lower lobes and anterior upper lobes with areas of subpleural sparing in the lower lobes (black arrows) suggesting nonspecific interstitial pneumonia
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