Amyloidosis - bronchial and diffuse nodular pulmonary involvement

Case contributed by Dr Bruno Di Muzio

Presentation

Sjogren syndrome.

Patient Data

Age: 60 years
Gender: Female

There are a few bilateral solid pulmonary nodules that show irregular contours, with a few of them also having calcifications. The right main bronchus is encased by an ill-defined soft tissue mass that has tiny flecks of calcifications.

CT

CT Chest (2 years later)

The previously demonstrated nodules have remained stable in number, but have increased in size since the last scan. The encasement and narrowing of the right main bronchus appear overall unchanged. 

Yellow arrows depicting the pulmonary nodules that have developed some calcification and the soft tissue encasing and narrowing the right bronchus. 

Microscopy: Two tan tissue fragments 5mm & 9mm. 

Microscopy: The biopsy consists of bronchial mucosa with subepithelial deposition of an amorphous eosinophilic material that stains positively with Congo red. There is no malignancy.

Immunoperoxidase stains show that the amyloid is strongly positive for kappa light chain. There is some weak staining for transthyretin, of doubtful significance. Lambda light chain and amyloid A stains are negative.
These findings indicate amyloidosis of light chain (AL) type. This result needs to be interpreted in the context of clinical findings and other investigations.

Conclusion: Right main bronchus biopsy: Amyloidosis. 

Case Discussion

This case illustrates a histologically confirmed focal amyloidosis causing right bronchial encasement. There are also multiple pulmonary nodules, a few of them demonstrating flecks of calcifications, thought to represent nodular pulmonary amyloidosis given the imaging appearances, minimal interval change over time, and confirmed tracheobronchial amyloidosis.

There is a known association between pulmonary amyloidosis AL type and Sjogren syndrome

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Case information

rID: 60156
Published: 31st May 2018
Last edited: 31st May 2018
System: Chest
Inclusion in quiz mode: Included

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