Wegener’s Granulomatosis
Patient Presentation
KMM was a 58-year-old female who presented to the ER complaining of frequent headaches and a cough for approximately 2 months. Additional symptoms on arrival to the ER included nosebleeds, worsening shortness of breath, generalized weakness, and recent weight loss. Past medical history included hyperlipidemia, osteoarthritis, thyroid disorder, fatty liver disease, and diabetes. Initial vital signs: BP 132/76, HR 79, RR 17, T 36.8, Oxygen saturation 95% on room air.
Differential Diagnosis
Pneumonia, influenza, Lyme disease, lupus, glomerulonephritis
Diagnosis
Labs were drawn on KMM to rule out infection, including blood cultures x2 and a UA/UC to rule out a UTI. All cultures showed no growth to date. The urinalysis showed significant red blood cells present. Anti-neutrophil cytoplasmic antibodies (ANCA) were positive in the blood, which are consistent but not diagnostic of Wegener's granulomatosis. KMM had an abnormal chest x-ray, which was followed up with a CT scan. The scan showed both cavitating and non-cavitating nodules. Based on the abnormal CT, physicians ordered a bronchoscopy with biopsy. The lung biopsy was diagnostic of Wegener’s granulomatosis, an autoimmune disorder that causes blood vessels to become inflamed.
Treatment
A three day course of IV steroids was started on KMM, Solu-Medrol® 1000mg once every 24 hours. Afterwards, KMM was started on PO prednisone, dosed at 60mg based on 1mg/kg/day. In addition to the steroids, treatment included Rituximab 325mg/m^2 IV once a week for four weeks to treat the immediate flare up. Because of the agents that make up the Rituximab, KMM was premedicated with IV Benadryl® and PO Tylenol® at 30 minutes prior to the transfusion and every four hours while the medication was infusing. Management of pain was controlled with Percocet® 5/325 every 6 hours as needed.
Outcome
KMM was discharged from the hospital after receiving her 4th dose of Rituximab®, and was instructed to continue the prednisone. At the time of discharge, the disease was considered to be in remission. Pain was well managed, and KMM no longer required narcotic intervention. Her shortness of breath was much improved, as well as generalized fatigue. It is important that KMM continue with her medication, however, as untreated Wegener’s has a high mortality rate and may resurface within 2 years of stopping treatment.
Case created by Kathleen Manty, 2012.