Background Vasculitis, first described in 1866, describes a group of rare conditions that damage blood vessels leading to multiple organ dysfunction, affecting about 0.01% of the world’s population. Once experienced in practice, is a disease not forgotten. I will present a complicated case of C-ANCA/PR3 vasculitis. Case Presentation A 57-year-old female with a 40-pack year history of tobacco use, presented with complaint of fevers, malaise, and a painful purpuric rash, occurring after an earache one week prior. The rash started on her feet, later involving her torso and scalp. Four days after the rash started, she developed odynophagia with oropharynx ulcers.

Labs were consistent with acute kidney injury, anemia, elevated CRP, and sedimentation rate. She had proteinuria, hematuria and her infectious workup was negative. Initial skin biopsy showed a non-diagnostic vascular fluorescence pattern. By hospital day four, the C-ANCA/PR3 labs were positive. Initial chest CT showed diffuse tiny nodular infiltrate with larger irregular ground glass and nodular opacities and no pulmonary embolism. On day four, chest x-ray showed worsening severe diffuse bilateral alveolar lung disease, concerning for diffuse alveolar hemorrhage.

She developed worsening dyspnea and escalating oxygen needs, requiring transfer to the critical care unit for respiratory failure. With positive C-ANCA/PR3 and her clinical presentation, she received pulse dose steroids with solumedrol 1,000mg for five days. She later had a cyclophosphamide infusion after developing an allergic reaction to rituximab. She required surgical debridement of her wounds and inpatient rehab, eventually being discharged home one month later with reduced but functioning kidneys. Although she was on DVT prophylaxis during hospitalization, she was readmitted two days afterwards with high-risk saddle pulmonary embolism requiring embolectomy. Discussion Our patient received pulse dose steroids four days after admission. With high clinical suspicion, it is appropriate to treat without biopsy results. Treatment with steroids, rituximab, and/or cyclophosphamide are common. She required surgical debridement of wounds, inpatient rehab and later an embolectomy. ANCA-associated vasculitis has a two-threefold increased risk of developing a thrombotic event.

The gold standard for diagnosing vasculitis is kidney, lung, and/or skin biopsies. Due to her clinical picture and bleeding risk, we did not biopsy her kidneys or lungs. Her initial skin biopsy was non-specific, which is an inherent problem with skin sampling. Recognizing the history and physical exam of vasculitis is important for any specialty, allowing prompt laboratory testing, tissue biopsy, and coordination of the multiple specialties involved in the management of vasculitis.