**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.**
Camille Cohen, Department of Nephrology, Hôpital Bichat, 46 Rue Henri Huchard, 75018 Paris, INSERM U1149 centre de recherche sur l’inflammation, Université Paris Cité.

**Main Body of the Abstract**
Typical presentation of renal AL-amyloidosis consists of massive albuminuria resulting in nephrotic syndrome. However, renal Al may sometimes feature predominant vascular deposition and present with lower degree of proteinuria.
We retrospectively collected data for 35 French patients with kidney biopsy-proven AL-amyloidosis and proteinuria/creatininuria ratio < 1g/g at diagnosis. At diagnosis, median proteinuria was 0.58 g/g (0.37-1) with median serum creatinine level of 167 (127-213) µmol/L and median eGFR of 36.2 (24.3-49.6) ml/min/1,73 m2 (CKD-EPI equation). Cardiac involvement was present in 67% of cases (stage 3 in 61%). Hypertension was present in 51% of patients at diagnosis.
Monoclonal gammopathy was detected in 94% of patients. Hematological diagnosis was MGCS (n=24, 69%), symptomatic multiple myeloma (n=8, 23%), and Waldenström’s macroglobulinemia (n=3, 9%). Central review of kidney biopsies revealed predominant vascular amyloid deposits in all cases (kappa n=12, lambda n=23). Nine percent of patients displayed severe (>50%) interstitial fibrosis. Proteomic analysis of kidney biopsies from 5 patients with lambda LC deposits revealed an overrepresentation of the VLambda 6-57 variability subgroup.
First-line treatment consisted of dexamethasone, alkylating agent and bortezomib triplet regimen (n=14, 50%), melphalan-dexamethasone doublet (n=8) or rituximab-bendamustine for IgM clones. The hematological response rate was 43%, with CR-VGPR rates of 38%. Renal response occurred in 17% of patients. Median overall survival was 82 months (178 months if VGPR/CR vs 16 months if NR/PR (p=0.002)). Renal median survival was 56 months.
In conclusion, low-grade proteinuria vascular renal AL-amyloidosis is associated with severe initial presentation and poor outcomes.



**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.**
Camille Cohen, _Department of Nephrology, Hôpital Bichat, 46 Rue Henri Huchard, 75018 Paris, INSERM U1149 centre de recherche sur l’inflammation, Université Paris Cité._

**Main Body of the Abstract**
Typical presentation of renal AL-amyloidosis consists of massive albuminuria resulting in nephrotic syndrome. However, renal Al may sometimes feature predominant vascular deposition and present with lower degree of proteinuria.
We retrospectively collected data for 35 French patients with kidney biopsy-proven AL-amyloidosis and proteinuria/creatininuria ratio < 1g/g at diagnosis. At diagnosis, median proteinuria was 0.58 g/g (0.37-1) with median serum creatinine level of 167 (127-213) µmol/L and median eGFR of 36.2 (24.3-49.6) ml/min/1,73 m2 (CKD-EPI equation). Cardiac involvement was present in 67% of cases (stage 3 in 61%). Hypertension was present in 51% of patients at diagnosis.
Monoclonal gammopathy was detected in 94% of patients. Hematological diagnosis was MGCS (n=24, 69%), symptomatic multiple myeloma (n=8, 23%), and Waldenström’s macroglobulinemia (n=3, 9%). Central review of kidney biopsies revealed predominant vascular amyloid deposits in all cases (kappa n=12, lambda n=23). Nine percent of patients displayed severe (>50%) interstitial fibrosis. Proteomic analysis of kidney biopsies from 5 patients with lambda LC deposits revealed an overrepresentation of the VLambda 6-57 variability subgroup.
First-line treatment consisted of dexamethasone, alkylating agent and bortezomib triplet regimen (n=14, 50%), melphalan-dexamethasone doublet (n=8) or rituximab-bendamustine for IgM clones. The hematological response rate was 43%, with CR-VGPR rates of 38%. Renal response occurred in 17% of patients. Median overall survival was 82 months (178 months if VGPR/CR _vs_ 16 months if NR/PR (p=0.002)). Renal median survival was 56 months.
In conclusion, low-grade proteinuria vascular renal AL-amyloidosis is associated with severe initial presentation and poor outcomes.



A Subset Of Patients With Renal AL-Amyloidosis Present Without Significant Proteinuria And Display Poor Renal Outcomes PA58 (#177)

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.