Sarcoidosis - fibrocystic changes

Case contributed by Dr Bruno Di Muzio

Presentation

Patient has been followed by the respiratory unit along the years due to chronic respiratory distress.

Patient Data

Age: 50 years
Gender: Male
X-ray

Chest radiograph

PA projection: there is diffuse reticulonodular infiltrates throughout both lungs with mid and upper zones predominance and apical architectural distortion. The macro nodules are somewhat confluent. The pleural spaces are clear. The cardiomediastinal contours appear normal. 

CT

CT Chest

CT Chest: Although there are features of fibrosis within the apices, mostly characterized by coarse septal bands and traction bronchiectasis, the predominant pattern is made of nodules, ground-glass opacities, and alveolar opacities suggestive of granulomatous inflammation that may be reversed with therapy. 

X-ray

Chest radiographs (4 years since presentation)

PA and lateral projections: Progression of the architectural distortion, which now only relatively spares the bases, and is characterized by coarse septal bands and bronchiectasis. Again, the cardiomediastinal contours appear normal, with no gross lymphadenopathy. 

CT

CT Chest (4 years since presentation)

HRCT Chest: The fibrotic changes have significantly progressed, characterized now not only by coarse septal bands and traction bronchiectasis, but showing also honeycomb-like cysts and bullae, with perhaps some volume loss. The granulomatous inflammation is much less prominent than years ago but is still present with a widespread distribution (mostly characterized by the nodules and ground glass opacities). 

CT

CT Chest (5 years since presentation)

HRCT Chest: The lung architectural distortion due to fibrotic changes have again progressed, and now the granulomatous inflammation characterized by nodules and ground glass opacities is minimal. 

CT

CT Chest (8 years since presentation)

HRCT Chest: Note how the fibrotic changes have progressed along the years: there is lung volume loss with architectural distortion caused by coarse septal bands, traction bronchiectasis, honeycomb-like cysts, and bullae. The rupture of those subpleural cysts/bullae caused bilateral spontaneous pneumothoraces. 

Case Discussion

This case illustrates the evolution of pulmonary sarcoidosis in its fibrotic-cicatricial stage along the years, culminating with bilateral spontaneous pneumothoraces as a sequela of advanced bullous disease: emphysematous bleb or necrosis of a subpleural sarcoid granuloma 1. The patient has a confirmed diagnosis of sarcoidosis and has been followed up by the respiratory clinic along the last 10 years, now in consideration for a lung transplant. 

In about 20% of patients, sarcoidosis evolves to prominent lung fibrosis as in this case. Note that the fibrocystic changes have a predominant upper and middle zones distribution 1.  

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Case information

rID: 63669
Published: 1st Jul 2019
Last edited: 14th Aug 2019
System: Chest
Inclusion in quiz mode: Included

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