Case Presentation: A 75-year-old woman with a past medical history of latent tuberculosis, hypertension, and type 2 diabetes mellitus presented with three days of non-bloody, non-bilious emesis, diarrhea, abdominal pain, and a new onset of weakness, pitting edema, and rash with burning sensation in the bilateral lower extremities. Admission labs were notable for elevated creatinine of 1.67 mg/dL (baseline 0.8 mg/dL), nephrotic range proteinuria, hematuria, and hypoalbuminemia of 2.4 g/dL. Renal function did not improve with intravenous fluids. Skin biopsy revealed focal IgA deposits in vessel walls, suggestive of IgA leukocytoclastic vasculitis, and renal biopsy demonstrated IgA-associated crescentic glomerulonephritis suggesting IgA vasculitis. Further etiological work up, including computed tomography scans (to assess for malignancy), infectious testing, and rheumatologic were all negative. She was treated with three days of methylprednisolone, resulting in significant improvement of her rash and gastrointestinal symptoms. On discharge, the patient was prescribed prednisone, for a total of 14 days treatment, scheduled follow-up with Rheumatology and with her primary care physician for further malignancy screening (due to association of adult IgA vasculitis with solid malignancies).
Discussion: Immunoglobulin A (IgA) vasculitis, previously known as Henoch-Schönlein purpura, is a leukocytoclastic vasculitis characterized by deposition of IgA immune complexes within small vessels of the skin, gastrointestinal tract, joints, and kidneys. The pathogenesis remains unknown, although proposed triggers include certain infections, insect bites, vaccinations, medications, systemic diseases, malignancy, and dietary allergens. While it is the most common systemic vasculitis in pediatric patients, it is relatively rare in adult and geriatric patients. This unusual presentation of IgA vasculitis, complicated by IgA nephritis, in an elderly female shows the importance of early diagnosis and treatment, especially in atypical patient populations. This patient met three of the four American College of Rheumatology criteria for diagnosis, including palpable purpura, acute abdominal pain, and biopsy showing granulocytes in the wall of small arterioles or venules. The presence of cutaneous and abdominal manifestations are also atypical in adults with IgA vasculitis. A multi-center cohort study in Japan found that rates of hypertension, impaired renal function, hypoalbuminemia, and crescentic glomerulonephritis were significantly higher among the geriatric patient population.
Conclusions: Although more common in the pediatric population, IgA vasculitis in adults can occur, with a mean age of presentation at 50 years old, although rare in the geriatric age group. While the prognosis of both adult and geriatric patients treated with aggressive corticosteroid use and renin-angiotensin-system blockade was favorable, the data indicates that the outcomes are poorer in these populations, as compared to pediatric patients. This case highlights the importance of prompt diagnosis and management of vasculitic diseases in older patients, to minimize disease progression and co-morbid complications.