Amyloidosis

Question 1

What is Amyloidosis?

Answer 1

1. Due to accumulation of fibrillary protein deposits in extracellular space –> disrupts the function of affected tissue
2. Types depends on the type of protein

Question 2

What are the types of presentations of amyloidosis?

Answer 2

1. Chronic lung condition leading to systemic amyloidosis (e.g. bronchiectasis)
2. Systemic amyloidosis (multiorgan) presenting with respi Sx
3. Localised pulmonary/resp tract amyloid deposits

Question 3

What are the Sx/Sg of amyloidosis?

Answer 3

Depending on sites involved

1. Tracheobronchial amyloidosis: obstructive Sx, haemoptysis or incidental imaging findings
2. PFT: restrictive with reduced DLCO
3. Persistent pleural effusion
4. SDB sec to cardiomyopathy, macroglossia, neuropathy & myopathy
5. Laryngeal amyloidosis –> hoarseness, haemorrhage
6. Amyloid pulmonary nodules (may cavitate or calcify) – strong assoc with Sjogren syndrome
7. Mediastinal/ hilar lymphadenopathy – may calcify & cause local obstruction

Question 4

How to diagnose amyloidosis?

Answer 4

Tissue Bx - with Congo red stain

Question 5

How to manage amyloidosis?

Answer 5

Depending on sites involved

Localized: resect the deposits, XRT to laryngeal deposits, stenting
Systemic: chemo