Anyone else here with AApoAIV (Apolipoprotein A-IV) Amyloidosis

@shookercooker Welcome to Mayo Clinic Connect. I’m not sure what this is that you have had. But there is one thing I know being a member of Mayo Clinic Connect, you are not alone! It may take awhile for members with similar experiences to chime in. Is this rare?

AApoAIV (Apolipoprotein A-IV) amyloidosis is an extremely rare form of systemic amyloidosis, often presenting as a rare cause of cardiac amyloidosis. Studies suggest it represents a very small fraction of cases, with one, this JACC study, identifying AApoAIV in only 0.6% (13/2096) of surveyed cardiac amyloidosis cases.
It also affects the kidneys and intestines.

Hello, @shookercooker. I've added your conversation to the Blood Cancers and Disorders group, where there are others who've talked about their experiences with amyloidosis. Even if there isn't anyone with this specific type of amyloidosis, we're here to listen and support you.

Are you comfortable sharing what type of symptoms you've been experiencing?

I had idiopathic intestinal pain for a few decades. Last September /October things became acute for six weeks. Iwas treated for an possible infection. After 4 emergency room visits finally got to see a gastroenterologist. My caloprotectin was 431. After another 3 weeks was scheduled for a colonoscopy. The half gallon of treatment did not go through causing extreme pain. I was diagnosed with a small bowel blockage. Emergency surgery removed 8" of jejunum. The biopsy came back as amyloidosis. I made an appointment at Mayo. Blood, fat and bone marrow revealed no amyloids. Mass spectrometry was inconclusive. In January finally had the colonoscopy. A biopsy of the duadenum revealed more amyloids. This time the mass spectrometry test at Mayo showed AApoAIV (Apolipoprotein A-IV) Amyloidosis. I am going back to Mayo early June for kidney ultrasound with possible biopsy and a cardiac MRI. AApoAIV (Apolipoprotein A-IV) Amyloidosis tends to deposit the amyloids in the micro vessels of the intestines, kidneys, and heart.

@shookercooker , I just happened upon this group. I was diagnosed with AAPO AIV amyloidosis in 2021 through biopsy after having a partial nephrectomy on left kidney due to renal cell carcinoma. I knew something wasn’t quite right after surgery, however, was shocked and scared to get this diagnosis . It affects my kidneys, duodenum, and nervous system. My heart hasn’t been affected, although I have cardiac issues. The frustrating part of this disease is that I don’t know any more about it today than I did 5 years ago! There is little to no information on this particular for of amyloidosis.

@granof6
You are right about the little information.
Here is some information, if you don't know it already:

Apolipoprotein A-IV Cardiac Amyloidosis Diagnosed by Mass Spectrometry–Based Proteomic Analysis https://www.sciencedirect.com/science/article/pii/S266608492600505X

Rare Forms of Cardiac Amyloidosis: Diagnostic Clues and Phenotype in Apo AI and AIV Amyloidosis https://pmc.ncbi.nlm.nih.gov/articles/PMC10351905/

(1124) - Amyloid Unmasked: A Rare Case of Apolipoprotein A- IV Cardiac Amyloidosis
https://www.jhltonline.org/article/S1053-2498(24)00784-8/fulltext
I hope it helps.