Case Presentation: A 64 year-old male with a past medical history significant for COPD presented with fever, abdominal pain and rash. Patient endorsed fever and chills for 2 weeks prior to inpatient hospitalization. Patient subsequently noted intermittent generalized abdominal pain associated with hematochezia. Patient noted onset of bilateral lower extremity rash the day of hospital admission. Vital signs on admission were significant for tachycardia and hypotension responsive to intravenous fluids. Physical examination revealed mild, diffuse abdominal tenderness, palpable purpura of the lower extremities and fine petechiae of the lower extremities and abdomen. Laboratory studies revealed leukocytosis, hematuria and proteinuria. Skin biopsy was performed given concern for Henoch Schonlein Purpura (HSP). Biopsy revealed granular IgA deposits within dermal vessel walls. Prednisone therapy was initiated. Hospital course was complicated by arthralgias, progressive vasculitic rash and AKI. Twenty-four hour urine protein was elevated to 1358 mg. Patient was placed on IV steroids and underwent renal biopsy. Biopsy revealed mesangial and focal proliferative glomerulonephritis with scattered mesangial deposits. Findings were consistent with mildly active HSP. Renal function improved on IV steroids. Course was further complicated by blurred vision and diplopia. MRI revealed punctate acute infarcts in the right midbrain and left occipital lobe. CTA was significant for high-grade stenosis of the intracranial left vertebral artery. Pulse steroid therapy was administered given concern for a vasculitic etiology of cerebrovascular accident (CVA). Patient improved on high dose steroid therapy and was transitioned to oral prednisone. Patient was discharged to home with plan for multidisciplinary outpatient follow up.
Discussion: HSP is a small vessel vasculitis that typically presents with purpura, arthralgias, abdominal pain and nephritis. HSP is primarily a disease of childhood and rarely presents in adults. CVA in the setting of HSP vasculitis is exceedingly rare. Four similar reports are described in the current medical literature with only one case report noted in an adult patient. Given the paucity of available literature, treatment options for HSP-associated CVA are ambiguous. Pulse dose steroids and cyclophosphamide have been reported as proposed therapeutic modalities.
Conclusions: Here we report a rare case of HSP and vasculitis-associated CVA in an adult patient presenting with fever, abdominal pain, rash, nephritis and arthralgias. This is the second report of its kind and the first to be reported in the United States. Therapeutic guidelines for the management of HSP-associated CVA are nonexistent given the rarity of this condition. Pulse steroids, however, are often used and were effective in the treatment of this patient.
To cite this abstract:Laudenslager, M; Honigman, J. A RARE CASE OF VASCULITIS-ASSOCIATED CVA IN AN ADULT PATIENT WITH HENOCH-SCHONLEIN PURPURA. Abstract published at Hospital Medicine 2018; April 8-11; Orlando, Fla. Abstract 675. https://www.shmabstracts.com/abstract/a-rare-case-of-vasculitis-associated-cva-in-an-adult-patient-with-henoch-schonlein-purpura-2/. Accessed September 18, 2019.