Author Listing - Lead/ Submitting Author (we will be communicating with this individual): Name, Institution, City and State where the work was done. Co-authors will be added in a separate section.
Samih Nasr, Mayo Clinic, Rochester, MN

Main Body of the Abstract
Apolipoprotein CII amyloidosis (AApoCII) is a rare (0.07%) form of hereditary amyloidosis that predominantly involves the kidney. Very little is known about its clinicopathologic and outcome characteristics.
All US renal AApoCII cases identified retrospectively from our amyloid cases typed by mass spectrometry between 2008 and 2023 were reviewed.
There were 24 patients (71% female; 100% Caucasian; median age 73 years (range 53-98)), who presented with proteinuria (100%, median 3.3 g/day), hypertension (82%), renal insufficiency (63%, median serum creatinine 1.6 mg/dl), edema (50%), hypoalbuminemia (45%), and nephrotic syndrome (33%). 32% had family history of renal failure. No patients had clinicopathologic evidence of other organ involvement. Serum M-spike was detected in 16%.
Histologically, AApoCII involved glomeruli in 100% (with nodular appearance in 88%). Mild vascular and interstitial involvement was present in 50% and 33%, respectively. All cases were Congo red-positive (weak in 63%).
On median follow up of 38 months (range 7-144) in 20 patients, 11 (55%) progressed to ESRD at a median of 48 months (range 15-132) and 3 (15%) died. The 1-, 2-, and 5-year renal and patient survival rates were: (100%, 71%, and 42%) and (100%, 88%, and 70%), respectively.  Median final creatinine and proteinuria in patients with kidney survival were 2.3 mg/dl (range 0.8-4.2) and 3.6 g/day (range 1.3-6.9).
AApoCII is typically diagnosed in elderly Caucasian females who present with proteinuria and renal insufficiency. Family history is lacking in 2/3. It appears to be renal-limited in most patients. Patient survival is favorable, whereas renal survival is guarded.

**Author Listing - Lead/ Submitting Author (we will be communicating with this individual): Name, Institution, City and State where the work was done. Co-authors will be added in a separate section.**
Samih Nasr, Mayo Clinic, Rochester, MN

**Main Body of the Abstract**
Apolipoprotein CII amyloidosis (AApoCII) is a rare (0.07%) form of hereditary amyloidosis that predominantly involves the kidney. Very little is known about its clinicopathologic and outcome characteristics.
All US renal AApoCII cases identified retrospectively from our amyloid cases typed by mass spectrometry between 2008 and 2023 were reviewed.
There were 24 patients (71% female; 100% Caucasian; median age 73 years (range 53-98)), who presented with proteinuria (100%, median 3.3 g/day), hypertension (82%), renal insufficiency (63%, median serum creatinine 1.6 mg/dl), edema (50%), hypoalbuminemia (45%), and nephrotic syndrome (33%). 32% had family history of renal failure. No patients had clinicopathologic evidence of other organ involvement. Serum M-spike was detected in 16%.
Histologically, AApoCII involved glomeruli in 100% (with nodular appearance in 88%). Mild vascular and interstitial involvement was present in 50% and 33%, respectively. All cases were Congo red-positive (weak in 63%).
On median follow up of 38 months (range 7-144) in 20 patients, 11 (55%) progressed to ESRD at a median of 48 months (range 15-132) and 3 (15%) died. The 1-, 2-, and 5-year renal and patient survival rates were: (100%, 71%, and 42%) and (100%, 88%, and 70%), respectively.  Median final creatinine and proteinuria in patients with kidney survival were 2.3 mg/dl (range 0.8-4.2) and 3.6 g/day (range 1.3-6.9).
AApoCII is typically diagnosed in elderly Caucasian females who present with proteinuria and renal insufficiency. Family history is lacking in 2/3. It appears to be renal-limited in most patients. Patient survival is favorable, whereas renal survival is guarded.

Clinicopathologic and Outcome Characteristics of Renal Apolipoprotein C-II Amyloidosis PC24 (#452)

We invite you to attend the XIX International Symposium on Amyloidosis to be held in Rochester, MN.

This symposium is organized as a hybrid meeting (with the focus on in-person) held from Sunday, May 26 through Thursday, May 30, 2024.

Together with the ISA Board and members of Mayo Clinic, a very attractive scientific hybrid program is being planned. The program will cover a variety of types of systemic amyloidosis: ATTR (hereditary and wild-type), AL (light-chain), AA and less frequent forms. There will be key lectures, specific symposia including those company sponsored, oral presentations and poster sessions of accepted abstracts.

COURSE DESCRIPTION
This program will provide a comprehensive review of state-of-the-art education in systemic amyloidosis: basic science, pathophysiology, diagnosis, current and emerging treatments. Scientific presentations will include competitive oral and poster abstracts. The rapid evolution of scientific discovery and expanding treatment options has created tremendous interest in the field and this meeting will fill and important gap in educating scientists and clinicians.