Srijit Ghosh1, Srijita Das1, Joy Mukherjee2, Salik Abdullah1, Rupsa Mondal1, Shirin Sultana1, Aayush Sehgal3, Tapan Behl4. 1. Guru Nanak Institute of Pharmaceutical Science and Technology, Panihati, Kolkata 700114, West Bengal, India. 2. Bengal School of Technology, Sugandha, Hooghly 712102, West Bengal, India. 3. Chitkara College of Pharmacy, Chitkara University, Patiala 140401, Punjab, India. 4. Chitkara College of Pharmacy, Chitkara University, Patiala 140401, Punjab, India. Electronic address: tapan.behl@chitkara.edu.in.
Abstract
BACKGROUND: IgA nephropathy (IgAN) has become the most prevalent form of glomerulonephritis affecting almost 1.3% of the total population worldwide. It is an autoimmune disorder where the host autoantibody forms an immune complex with the defective galactose-deficient IgA1 and gets deposited at the mesangium and endocapillary region of glomeruli. IgA has the capability to activate alternative and lectin complement cascades which even aggravates the condition. Properdin is directly associated with IgAN by activating and stabilising the alternative complement pathway at the mesangium, thereby causing progressive renal damage. OBJECTIVE: The present review mainly focuses on correlating the influence of properdin in activating the complement cascade at glomeruli which is the major cause of disease exacerbation. Secondly, we have described the probable therapies and new targets that are under trials to check their efficacy in IgAN. METHODS: An in-depth research was carried out from different peer-reviewed articles till December 2020 from several renowned databases like PubMed, Frontier, and MEDLINE, and the information was analysed and written in a simplified manner. RESULTS: Co-deposition of properdin is observed along with IgA and C3 in 75%-100% of the patients. It is not yet fully understood whether properdin inhibition can attenuate IgAN, as many conflicting reports have revealed worsening of IgAN after impeding properdin. CONCLUSION: With no specific cure still available, the treatment strategies are of great concern to find a better target to restrict the disease progression. More research and clinical trials are required to find out a prominent target to combat IgAN.
BACKGROUND:IgAnephropathy (IgAN) has become the most prevalent form of glomerulonephritis affecting almost 1.3% of the total population worldwide. It is an autoimmune disorder where the host autoantibody forms an immune complex with the defective galactose-deficientIgA1 and gets deposited at the mesangium and endocapillary region of glomeruli. IgA has the capability to activate alternative and lectin complement cascades which even aggravates the condition. Properdin is directly associated with IgAN by activating and stabilising the alternative complement pathway at the mesangium, thereby causing progressive renal damage. OBJECTIVE: The present review mainly focuses on correlating the influence of properdin in activating the complement cascade at glomeruli which is the major cause of disease exacerbation. Secondly, we have described the probable therapies and new targets that are under trials to check their efficacy in IgAN. METHODS: An in-depth research was carried out from different peer-reviewed articles till December 2020 from several renowned databases like PubMed, Frontier, and MEDLINE, and the information was analysed and written in a simplified manner. RESULTS: Co-deposition of properdin is observed along with IgA and C3 in 75%-100% of the patients. It is not yet fully understood whether properdin inhibition can attenuate IgAN, as many conflicting reports have revealed worsening of IgAN after impeding properdin. CONCLUSION: With no specific cure still available, the treatment strategies are of great concern to find a better target to restrict the disease progression. More research and clinical trials are required to find out a prominent target to combat IgAN.