Sarcoidosis associated pulmonary arterial hypertension

Case contributed by Dr Bruno Di Muzio

Patient Data

Age: 30 years
Gender: Male

Prominent pulmonary artery notch in keeping with pulmonary hypertension. Also bilateral hilar lymphadenopathy. Small right lower lobe calcified granuloma. An impression of subtle bilateral airspace pacification, but no consolidation. No pleural effusion. 

Diffuse soft centrilobular ground-glass nodules without apicobasal gradient. Focal hyperlucency in the anterobasal right lower lobe in keeping with air trapping. 8mm subpleural nodule right middle lobe is unchanged from 5 years ago, considered benign. RLL calcified granuloma. No reticulation, architectural distortion, or traction bronchiectasis to suggest fibrosis. Pleural spaces remain clear.

Bilateral hilar and mediastinal lymphadenopathy without calcification. Enlarged central pulmonary arteries (pulmonary trunk 4.8cm) with right ventricular dilatation and interventricular septal bowing, consistent with pulmonary arterial hypertension. No pericardial effusion. No central venous line. 

11 mm cyst (average attenuation 6HU) in hepatic segment IVa/II. Remaining visualized upper abdomen is within normal limits.  No suspicious osseous erosion.

Macroscopy: A. Labelled "Left lung". Left lung weighing 524 g measuring 255 x 225 x 105 mm. Pleura appears pale cream/tan with a smooth texture. Bronchovascular resection margins are clear and hilar lymph nodes are carbon pigmented. On sectioning, parenchyma appears diffusely pale cream/tan with a spongy texture. No lesions are identified. Vessels appear thickened with areas of calcification. No thrombi are identified. B. Labelled "Right lung". right lung weighing 630 g measuring 225 x 220 x 125 mm. Pleura appears pale cream/tan with a smooth texture. Within the right middle lobe, there is a black/cream subpleural nodule measuring 9 x 7 x 5 mm. Bronchovascular resection margins are clear, and lymph nodes are carbon pigmented. On sectioning, parenchyma appears diffusely pale cream/tan with a spongy texture. No lesions are identified. Vessels appear thickened with areas of calcification. No thrombi are identified. 

Microscopy: Throughout the interstitium, there are frequent non-necrotising granulomas, primarily in a bronchovascular distribution, comprising discrete aggregates of epithelioid histiocytes surrounded by a rim of lymphocytes. There is no necrosis and the Ziehl-Neelsen and PAS stains for acid-fast bacilli and fungi are negative. The right-sided subpleural nodule identified macroscopically is an intraparenchymal lymph node enlarged by non-necrotising granulomatous inflammation. The lung parenchyma has preserved architecture but does contain scattered foci of interstitial pneumonitis comprising alveolar pneumocyte hyperplasia and a mononuclear inflammatory infiltrate within the alveolar interstitium. Hilar lymph nodes also contain non-necrotising granulomatous inflammation. Large pulmonary arteries show atheroma formation including eccentric intimal fibrosis with an infiltrates of foamy macrophages. Small intraparenchymal muscular arteries show concentric and medial hyperplasia with marked narrowing of lumens. There is no vasculitis. No plexiform or angiomatoid lesions are identified. There is no evidence of malignancy. 

Conclusion: Left and right native lungs, explants: Intraparenchymal and nodal non-necrotising granulomatous inflammation, consistent with sarcoidosis. Mild patchy interstitial pneumonitis. Arterial changes are consistent with pulmonary hypertension.

Case Discussion

This is quite a difficult chest case of a young adult presenting with pulmonary hypertension stigmata. The lungs are not fibrotic and show a few scattered small calcified granulomas and widespread centrilobular soft ground-glass opacities, which can be seen in severe pulmonary hypertension of any cause. Enlarged lymph nodes are also nonspecific and can be seen in PAH of multiple causes. 

Diagnosis of sarcoidosis was clinically suspected and further confirmed on histopathology, but hard to be inferred on imaging.  

Please refer to a companion case on pulmonary veno-occlusive disease to see the subtle differences from this case (septal thickening and mosaicism). 

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Case information

rID: 71719
Published: 26th Apr 2020
Last edited: 26th Apr 2020
System: Breast
Inclusion in quiz mode: Excluded