Updated: Friday, December 1, 2017, 10:17 AM
While rotating through Penn Presbyterian Medical Center in West Philadelphia, I met a gentleman who was no stranger to health problems. The 64-year-old retired engineer already knew he had diabetes, high blood pressure, mild kidney dysfunction, high cholesterol, chronic bronchiectasis (widening of the lung’s airways), ulcerative colitis (a chronic, inflammatory bowel disease), and an enlarged prostate, but we didn’t cross paths for any of these conditions.
Instead, he came to the hospital because his lungs and kidneys had stopped working.
His symptoms started slowly, first with shortness of breath, then progressing to fevers and chills. He went to his primary care physician, and was prescribed antibiotics for suspected pneumonia. Over the next few weeks, he felt worse and went to a nearby hospital. Despite getting intravenous antibiotics there, his lungs deteriorated so much that he had to be placed on a ventilator to help move air in and out of the lungs. This isn’t an uncommon measure for severe pneumonia, but it wasn’t enough for this patient, who was flown to Penn Presbyterian.
By the time he arrived, his lungs had started to bleed into the airways. Between the blood and inflammation in his lungs, the ventilator couldn’t help him get enough oxygen. He essentially was suffocating, and had to be placed on Extracorporeal Membrane Oxygenation (ECMO) to artificially oxygenate his blood, a measure reserved for the most desperately ill patients.
At the same time, his kidney function started to decline, and blood appeared in his urine. He was at risk of needing dialysis.
What caused this patient’s life-threatening symptoms?
Our patient’s body was basically attacking itself. He had an autoimmune syndrome known as perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA) vasculitis.
In p-ANCA vasculitis, antibodies target certain types of white blood cells called neutrophils (specifically the cytoplasm within the cells), which causes white blood cells to attack small blood vessels of specific organs, in this case his lungs and kidneys. Unfortunately, the trigger for p-ANCA vasculitis remains unknown.
Treatment for this disease involves suppressing the immune system, usually with steroids or chemotherapy, and removing the antibody from circulation through a filtration process known as plasmapheresis.
Our patient received all of these therapies and was able to breathe on his own again. He avoided dialysis entirely, and was discharged from the hospital. He continues on long-term immunosuppression therapy through a combination of periodic infusions and oral steroids to prevent recurrence of his disease, and is doing well.
Shivam Joshi, MD, is a fellow of clinical nephrology in the Perelman School of Medicine at the University of Pennsylvania. He would like to thank Dr. Amanda Leonberg-Yoo, Dr. Tiffany Wong, and Dr. Timothy Moore in assisting with the patient’s care and for reviewing this article.