Overview of IgA
Immunoglobulin type A nephropathy (IgAN) is the most common primary glomerulonephritis in the world and one of the leading causes of renal failure. It has been diagnosed in patients of almost all ages and all populations, though the prevalence does vary with geographic region. Symptoms vary from asymptomatic microscopic or macroscopic hematuria to nephritic syndrome or nephrotic syndrome and in some instances rapidly progressive glomerulonephritis. The disease has an unknown rate of progression to end-stage renal disease (ESRD) and the risk for progression has ranged from as low as 5-15% of patients 5 years after diagnosis to as high as 10-50% of patients 20 years after diagnosis.
Treatment of IgA Nephropathy
IgA nephropathy is common glomerulonephritis with many different clinical presentations that is a leading cause of ESRD worldwide. The disease process revolves around the production of galactose deficient IgA1 and autoantibodies against it. Mesangial IgA dominant or codominant staining on immunofluorescence is common in all cases of IgAN, but there is wide variability in the histologic features seen by light microscopy. The Oxford classification developed the MEST-C score showing that the varying histologic features seen in the biopsies of IgAN patients have prognostic significance. Correctly identifying the pertinent light microscopic features and diagnosing IgAN with an accurate MEST-C score is essential for the pathologist to help guide therapy and prognosis.
Fig. 1 Onset, progression, and treatment of IgA nephropathy. (Moriyama, 2019)
The initial step in the development of IgAN is a mucosal infection. Therefore, tonsillectomy is important for removing this trigger and stopping the subsequent production of Gd-IgA1. Tonsillectomy also suppresses the relapse of nephropathy in IgAN patients after achieving clinical remission by reducing the risk of tonsillitis and mucosal infections in the future. Furthermore, epipharyngeal abrasive therapy and oral care may have beneficial effects on the treatment of IgAN although there is no evidence supporting this. Corticosteroid therapy is the most effective method of suppressing GdIgA1, immune complexes with Gd-IgA1, and mesangial IgA deposition, owing to its immunosuppressive effects. The anti-inflammatory effects of corticosteroid therapy suppress glomerular inflammation, such as mesangial hypercellularity, endothelial hypercellularity, tuft necrosis, and cellular and fibrocellular crescents. It is critical to suppress active lesions in IgAN as soon as possible before glomerular inflammation spreads diffusely and globally to other glomeruli. If there are no active lesions in glomeruli, corticosteroid therapy should be carefully considered, referring to clinical findings and the patient’s social background. After active lesions are suppressed, prognosis depends on the stress on the remaining glomeruli in the form of glomerular hyperfiltration and glomerular hypertension, as well as on CKD management. RASIs are the most effective treatment to reduce glomerular hyperfiltration and hypertension. Once RASIs are started, they should be continued until progression to ESRD, because hyperfiltration and glomerular hypertension in the remaining glomeruli will become severe, along with the deterioration of renal function and the continuous decline in the number of remaining glomeruli. Importantly, hyperkalemia and teratogenicity must also be considered carefully. In CKD management, the risk factors for the progression of CKD should be well controlled.
Services at Creative Biolabs
Many researchers have reported the importance of IgA nephropathy. Creative Biolabs has also been focusing on non-igG antibodies over years. With rich experience and strong foundations, we have established a comprehensive technology platform offering high-quality non-IgG relevant services to global clients:
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If you are also focusing on non-IgG antibodies or you have any other questions about our services, please feel free to contact us for more information.
Reference
- Moriyama, T. Clinical and histological features and therapeutic strategies for IgA nephropathy. Clin Exp Nephrol. 2019, 23(9): 1089-1099.
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