PARAPROTEIN RELATED KIDNEY DISEASE – A STUDY FROM TERTIARY CARE CENTER IN INDIA

Introduction:

Paraprotein–related kidney disease represents a complex group of diseases caused by an abnormal paraprotein secreted by a clone of B cells or plasma cells, which leads to diverse kidney disorders. Identification of this entity has become more prevalent as a result of advances in Immunofluorescence(IF) techniques where light chain detection has become routine practice while evaluating renal histopathology.Identification of paraprotein-related kidney disease is important which points towards the monoclonal origin of this entity. So clone directed therapies may help in achieving remission

This study aims  To analyse the spectrum of clinical characteristics and renal pathological manifestations of patients with Paraprotein-related kidney disease and follow up for 6 months to look for recovery

Methods:

It’s a single centre prospective study from Jan 2021 to July 2024 , 107 cases of biopsy-proven paraprotein-related kidney injury who have light chain restriction in IF or light microscopy showing myeloma cast nephropathy, at tertiary care centre in south India , were included  and then  following data collected  : baseline demographics , routine lab parameters , Skeletal survey, SPEP, UPEP , SFLC, bone marrow examination +/- genetic testing ) and prospectively followed up for 6 months  and looked for renal recovery and malignancy recovery

Results:

107 patient with biopsy-proven paraprotein related kidney disease were included in the study. 

Mean age of the study population was 52+12years.

Male:Female ratio was1.7:1. 

Results of histopathological findings showed  Light chain cast nephropathy(CN)in39.4% of cases, light chain deposition disease(LCDD) was seen in 25.4% of cases and AL Amyloidosis was seen in18.3%. 16.9% of patients had more than one pathological abnormality in renal biopsy examination.

Among clinical presentation Chronic Kidney Disease(CKD) was seen in 35.5% of the study cohort, followed by Acute Kidney Injury (AKI) seen in 24.2 of the study cohort.

Hypertension was seen in 78.2% of patients at the time of presentation

Among laboratory abnormalities Anemia was seen in 91% of cases , Albumin-Globulin (AG) ratio reversal was seen in 57.7% and nephrotic range proteinuria was seen in 54% of cases. 

45% of cases required Hemodialysis on presentation.

We have follow up data at 6 months of 50 cases out of 107  , of which 18 patients have renal recovery 36%

Conclusions:

Paraprotein related kidney diseases presented with a diverse clinical spectrum out of which CKD was the most common followed by AKI and Nephrotic syndrome.

Pathologically, cast nephropathy was the most common followed by light chain deposition disease and amyloidosis.

Proliferative Glomerulonephritis with monoclonal immunoglobulin deposition disease (PGNMID), a new block in this spectrum was exceedingly identified in our study population

I have no potential conflict of interest to disclose.

I did not use generative AI and AI-assisted technologies in the writing process.