Pulmonary sarcoidosis with fibrosis

Case contributed by Laura Duerden


Presents with a chronic cough. Reports a history of lung disease many years ago but cannot recall the details.

Patient Data

Age: 70 years
Gender: Male

There is asymmetric increased density of the left hilum and enlargement of the hilar structures.  There is a band of opacity extending from the left hilum in the left upper zone.  The right hilum is also enlarged.

There are enlarged mediastinal and bilateral hilar lymph nodes.  Most of the lymph nodes are calcified.  Both coarse nodal calcification and 'icing sugar' calcification are present.

In both upper lobes, there is fibrosis with distortion of the bronchovascular structures around the hilar regions and perihilar mass-like consolidation. The left upper lobe airway is occluded and lingula airway is narrowed as they pass through the perihilar mass-like consolidation. There are small nodules throughout the lungs that have a centrilobular and subpleural distribution.  Similar to the distribution of fibrosis, the nodules are seen predominantly in the upper lobes.

Review of the patient's historic casenotes revealed a prior diagnosis of sarcoidosis that had been made on histological examination of a lymph node biopsy many years previously.

Case Discussion

Sarcoidosis is a granulomatous disorder of unknown cause. Non-caseating granulomas may form in any body tissue and the lungs are frequently affected. Granulomas form along the pulmonary lymphatics, so the distribution of nodularity is often centrilobular and subpleural.  Granulomas can coalesce to form fibrous masses and regions of dense consolidation in the lungs which are centered around the perihilar bronchi and vessels, usually in the upper lobes.  Pulmonary sarcoidosis progresses to fibrosis in 20-25% of patients and the fibrosis will remain once the disease is no longer active.  

Airway occlusion and stenosis occur either due to compression by adjacent lymph nodes or distortion due to fibrosis, as in this case, or granulomas within the airways that become fibrotic over time.

When granulomas within lymph nodes start to calcify, the calcification is very fine and powdery – described as ‘icing sugar’.  The calcification gradually becomes more coarse and increases in density over time.

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