Amyloidosis: Multisystem spectrum of Disease with Pathologic Correlation
doi.org/10.1148/rg.202…

Check out this new @RadioGraphics article by @markdsugi et al. reviewing the full spectrum of amyloidosis.
#RGphx #Tweetorial #RadRes

@markdsugi
@MSalomaoMD
@sanjeevbhalla Image
AL: most common form of systemic amyloidosis in the US & seen with plasma cell dyscrasia.

AA: chronic infection/inflammation, solid organ malignancy & familial conditions.

ATTR: significant cause of morbidity and mortality related to cardiomyopathy.
#RGphx Image
CNS β-amyloid deposits ➡️cerebral amyloid angiopathy (CAA) & Alzheimer disease (AD).

CAA is an important cause of multiple intracerebral hemorrhages or cerebral microbleeds, or alternatively, a single hemorrhage in the presence of cortical superficial siderosis.
#RGphx Image
To evaluate AD, 2 PET tracers have complementary roles:
18F-florbetapir: binds β-amyloid fibrils & abnormal ⬆️ cortical gray matter uptake = amyloid deposits

18F-FDG (glucose analog): ⬇️uptake in post cingulate cortex, precuneus & parietotemporal cortices seen in AD
#RGphx Image
MRI findings in cardiac amyloidosis include ⬆️ native T1 myocardial signal (nulling before blood pool) & diffuse subendocardial or transmural LGE
#RGphx Image
Tracheobronchial amyloidosis ➡️ focal or segmental circumferential airway thickening including the posterior membrane +/- irregular calcs
#RGphx Image
2 types of parenchymal pulmonary amyloidosis:
1) Nodular= solitary mass (amyloidoma) or multiple nodules, both can progressively calcify.
2) Diffuse alveolar septal type: basilar & subpleural consolidation w/ septal thickening & micronodules

#RGphx Image
Renal amyloidosis most often occurs in systemic AL amyloidosis in the US. CT findings include parenchymal thinning and raely amorphous calcifications, which mimics medullary nephrocalcinosis. Ultrasound findings are nonspecific with echogenic parenchyma.
#RGphx Image
Urinary tract amyloidosis is usually localized, most commonly involving the urinary bladder. CT findings include focal areas of wall thickening, irregular luminal narrowing & linear submucosal or intramural calcs.
#RGphx Image
Liver is commonly involved in systemic amyloidosis. CT findings include hepatomegaly, masses, calcs, diffuse hypoattenuation (mimics steatosis), heterogeneity & post contrast mottled appearance. ⬆️ liver stiffness on MR elastography. #RGphx Image
AA amyloidosis on CT shows long-segment circumferential mural thickening & stratification (target sign) of the small bowel or areas of focal ulceration. AL amyloidosis has deposits in the submucosa and muscularis propria & CT shows nodular polypoid masses.
#RGphx Image
Peritoneal amyloid deposition on CT appears as soft tissue infiltration of omental or mesenteric fat & have progressive dystrophic calcification over time.
#RGphx Image
Breast involvement is rare but may occur in AL amyloidosis & can be localized or systemic forms of disease. Localized forms can present as palpable mass w/ imaging features that are difficult to distinguish from malignancy. Thus, tissue biopsy necessary.

#RGphx Image
Carpal tunnel syndrome is the most common initial presentation of age-related, systemic wild-type ATTR. MRI features include thickening of the flexor tendons, osseous erosions & median nerve flattening
#RGphx Image
Thanks for following along and don’t forget to check out the complete article @RadioGraphics here: doi.org/10.1148/rg.202…

And keep an eye out for more #RGphx #tweetorial
#Radres #RadEd #FOAMrad

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More from @MollyCarnahan

11 Mar
1 of 3 infants has a misshaped skull & 1/2500 have craniosynostosis. This new @RadioGraphics Fundamentals presentation reviews CT findings of craniosynostosis & common syndromic causes. #RGphx #radres
Craniosynostosis: Understanding the Misshaped Head bit.ly/30aRqDY
Before 9 months of age, only the metopic suture can be fused normally. Sagittal, coronal & lamboid sutures narrow during childhood but should not fuse. CT is imaging modality of choice- describe shape/synostosis, ICP & vascular anatomy

2/11 #RGphx
In non-syndromic patients, usually 1 suture is involved: sagittal > coronal > metopic > lamboid.

Let’s review the key CT features of each.

3/11 #RGphx
Read 11 tweets

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