Presentations

Abstract

Introduction: According to the National Kidney Foundation, there are an estimated 60,000 Americans living with IgA nephropathy. Since its discover by Dr. Jean Berger, we have continued to learn more about the pathophysiology, the progression of disease, and treatments; yet, there are still no FDA approved treatments. there is still controversy among the literature about treatment strategy and specific risk factors for the development of end stage renal disease (ESRD) in patients with IgA nephropathy with crescents. Patients with crescentic IgA nephropathy have variable presentations which brings challenges to diagnosing and treating this population. Presenting symptoms for crescentic IgA nephropathy range from mild proteinuria and hematuria to gross hematuria, hypertension, and acute kidney injury, though crescents are more likely to be found on biopsy in patients who present with AKI and gross hematuria. Delay in diagnosis may lead to significantly more morbidity and mortality as the degree of crescents correlates with progression to ESRD and reduced life expectancy. It is crucial to continue to learn about this disease including the way it clinically presents, progression of disease, and effective treatments. It

Case Information: A 30-year-old female presented to the emergency department with worsening nausea, fatigue, left flank pain, and hematuria. She was noted to have a creatinine of 2.3 with urine that was positive for leukocytes, moderate amount of blood and RBCs with hyaline casts. The patient developed significant edema, creatinine continued to increase, and she developed new onset worsening proteinuria greater than 1 gram per day.

Discussion/Clinical Findings: Renal biopsy showed mesangial immunoreactivity for IgA and C3 deposition with 36% crescentic glomeruli on microscopy. Findings were consistent with IgA nephropathy with crescent glomerulonephritis, Oxford IgA classification score of M1 E1 S0 T0 C2. Nephrology was consulted and started the patient on 3 days of pulse IV solumedrol 250mg and oral mycophenolate for immunosuppression. After pulse steroid course was completed, creatinine slowly decreased, and her condition improved. She was discharged on oral prednisone 60mg for 6 months, oral mycophenolate 1000 mg BID, and losartan 25mg QD. IgA Nephropathy is an autoimmune condition that is a common cause of glomerulonephritis. While it can be idiopathic, it is often triggered by mucosal infections of the upper respiratory or gastrointestinal systems. Based on histological findings on kidney biopsy, patients are classified into five categories including mesangial hypercellularity (M), endocapillary hypercellularity (E), segmental glomerulosclerosis (S), tubular atrophy/ interstitial fibrosis (T), and the presence of crescents (C). The presence of crescents as a predictor of disease progression has recently been added to the Oxford Classification system in 2017 as it has been found to be associated with accelerated rate of renal decline to end stage renal disease when not promptly and appropriately treated with immunosuppressive therapy and strict blood pressure control. Crescents on glomerular biopsy are only present in approximately 20-30% of patients with IgA nephropathy and only approximately 3% of biopsy may meet the criteria for C2 IgA nephropathy.

Conclusion: Unfortunately, appropriate treatment of rapidly progressive glomerulonephritis (RPGN) in IgA Nephropathy has yet to be clearly identified, as it has previously been excluded in many studies due to the rapid rate of decline and severity of disease in most patients. Previous research has focused on the use of immunosuppressive therapies as the mainstay in treatment including various corticosteroids, cyclophosphamide, calcineurin inhibitors, mycophenolate, and azathioprine. Proposed metrics to predict disease progression include the degree of daily proteinuria (> 1g/day), hypertension, and Oxford Classification. More research is needed in order to ascertain ideal management for patients who present with severe disease.