Journal of Hematology & Thromboembolic Diseases
Open Access

Overview of Immunosuppression on Henoch Schonlein Purpura in Renal Failure

Sudipta Bhowmik^{* *}Correspondence: Sudipta Bhowmik, Department of Clinical laboratory, Keele University Medical School, Cheltenham, United Kingdom, Email:

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Description

IgA Nephropathy (IgAN) and Henoch-Schönlein Purpura Nephritis (HSPN) are currently thought to be linked illnesses. In fact, in a pair of identical twins, one may exhibit IgAN while the other displays HSPN. Moreover, both disorders exhibit comparable IgA abnormalities and histologic characteristics. IgAN has a consistent clinical pattern that includes a slowly progressing disease with proteinuria that gradually increases, loss of renal function, and flare-ups of macroscopic hematuria in 50% of patients. Contrarily, HSPN typically manifests as an initial acute episode that resolves completely in the majority of patients. A small percentage of patients experience persistent proteinuria and progressive chronic renal failure. When HSPN presents, nephrotic and/or arthritic symptoms are more prevalent. End-Stage Renal Failure (ESRF) brought on by HSPN is rarely in adults, but in a large children's population from Necker-Enfants Malades Hospital, incidence reached 5.1%. It is challenging to determine the prevalence of HSP from the literature. The annual incidence ranges from 6.1/100,000 children in The Netherlands to 20.4/100,000 children in the UK using the contentious American College of Rheumatology criteria for vacuities categorization, which may lead to over diagnosis. It is commonly accepted that ageing reduces the incidence of HSP. Studies have indicated a range of 20 to 100% of children presenting with renal dysfunction. In one study, 49% of patients with the typical leucocytoclastic cutaneous vacuities and IgA deposits-which served as the basis for the diagnosis presented with aberrant urine symptoms. Up to 20% of children with HSPN develop Chronic Kidney Disease (CKD) 20 years following the diagnosis in selected series, compared to fewer than 5% in unselected series. In adults, CKD risk is increased. Among 388 patients followed for at least 5 years in four published datasets, it ranges from 35% to 69%. Adults also more frequently present with joint complaints.

Immunosuppression’s

There have been few high-quality RCTs examining the use of immunosuppression in the management of IgAV nephritis with histological findings. Because there are few and often low-quality research trials, the Cochrane collaboration came to the conclusion that there is a substantial lack of evidence to support treatment for established nephritis. Using the best available evidence, new international consensus guidelines have recommended oral prednisolone as the first-line treatment for mild renal illness (those with a normal renal function and mild/ moderate proteinuria 250 mg/mmol, which typically belongs to class II, III, and IV, or IIIa histological alterations) along with either azathioprine, mycophenolate mofetil, or intravenous cyclophosphamide for moderate nephritis (50% crescents on biopsy and decreased renal function or severe persistent proteinuria, typically histological class IIIb).

Intravenous corticosteroids plus intravenous cyclophosphamide are advised to achieve remission in severe nephritis (defined as >50% crescents on renal biopsy and decreased renal function or severe proteinuria >250 mg/mmol, histological class IV-V), followed by a term of maintenance therapy. They came to the conclusion that there doesn't seem to be a place for oral cyclophosphamide or calcineurin inhibitors in this illness.

Conclusion

Unless there is renal involvement, the mainstays of treatment for patients with Henoch-Schönlein Purpura are symptomatic and supportive care. Acetaminophen and Non-steroidal Anti- Inflammatory Drugs (NSAIDs) can be used to treat fever and joint pain, but they must be avoided if the kidneys or gastrointestinal system are affected. For the treatment of renal, musculoskeletal, and gastrointestinal symptoms, early oral prednisone administration is helpful. Prednisone cannot prevent renal disease, although it lowers the likelihood that children may acquire a chronic renal illness. Evidence suggests that prednisone lowers the length and severity of stomach discomfort during the first two weeks of treatment, according to randomized control studies carried out.