CHSU Discovery

Abstract:

Introduction and Background: Immunoglobulin A Vasculitis (IgAV), previously known as Henoch-Schonlein purpura, is a systemic vasculitis where IgA immune complexes are deposited primarily into small blood vessel walls such as the skin, joints, GI tract, and kidneys, leading to vascular inflammation and tissue destruction. IgAV mostly affects children and is rarely seen manifesting in the elderly. The disease’s etiology is hypothesized to be multifactorial, but preceding infections one to three weeks prior can play a huge role in triggering the disease. This case highlights the unique challenges in formulating a diagnosis and optimizing appropriate management. 

Material and Methods: A comprehensive evaluation of the patient's medical history, physical assessments, laboratory investigations, echocardiogram, antibody levels, and skin and renal biopsies. 

Results: We present a case of an 88-year-old male who came to the emergency department with new-onset dyspnea and wheezing for one day and was found to have acute renal failure. In addition he also had purpura scattered across his lower extremities and abdomen, as well as 2+ lower extremity pitting edema, which was first noticed tw o weeks prior. Initial labs were significant for severe anemia requiring transfusion, significant gross hematuria, 3+ proteinuria, and a BNP of 444. Chest X-ray showed bilateral superimposed pneumonia, and the echocardiogram revealed LV systolic dysfunction, with an EF of 35%. The patient was previously treated for a GI bleed and MSSA cellulitis of his left arm a month prior. After admission, his renal function worsened. Further workup revealed elevated IgA levels. A kidney biopsy was performed, which showed IgA-dominant immune complex glomerulonephritis with 10% active crescents. Treatment involved a combination of immunotherapies, including a low-dose corticosteroid and an immunosuppressive agent, mycophenolate. Eventually, the plan was to initiate Tarpeyo and Filspari on an outpatient basis. 

Conclusion: In the elderly population, IgAV often presents with atypical manifestations, including GI bleeding, renal impairment, and cardiovascular complications, further delaying diagnosis and increasing morbidity. This case report showcases the importance of early recognition and initiation of treatment via clinical symptoms while awaiting histological confirmation. Furthermore, the patient’s comorbidities, along with possible adverse effects and long-term consequences of immunotherapies, pose additional complexities in treatment selection and require frequent monitoring to minimize complications.