Granulomatosis with polyangiitis (GPA) was first described in the British Medical Journal in 1897 by Scottish otolaryngologist Peter McBride.1 GPA is a relatively rare, systemic necrotizing vasculitis that can make diagnosis challenging. The incidence has been estimated anywhere between two and 12 cases per million.2 GPA mainly affects adults between the ages of 45 and 60. Most commonly, the upper and lower airways (90%), the kidneys (80% in the first two years of disease) and the skin are involved, but patients can have a multitude of other symptoms.3
In this case presentation, we aim to increase awareness, as well as discuss developments in our understanding of the role of anti-neutrophil cytoplasmic antibodies (ANCAs) and their importance in the clinical monitoring and treatment of GPA.
Case Presentation
A 44-year-old Caucasian male with a five-year history of severe headaches, cough, paroxysmal nocturnal dyspnea, tinnitus, hearing loss, changes in his voice and sinus congestion was evaluated and treated multiple times with intranasal oxymazoline, various antibiotic regimens and systemic steroids. Due to only partial relief and recurrent upper respiratory symptoms, an ear, nose and throat (ENT) consultation was obtained.
The initial ENT evaluation revealed narrowing of the nasal vestibules, boggy and enlarged nasal turbinates with a normal orocavity and oropharynx. During the waxing and waning course of his symptoms, he also reported multiple episodes of epistaxis and purulent nasal secretions that, at times, he described as “tissue coming out from his nose.” When he was receiving treatment with antibiotics and steroids, his symptoms would briefly improve. Then, shortly after discontinuing treatment, his symptoms would recur.
The patient was referred to an allergy/immunology specialist, who started him on subcutaneous immunotherapy. The patient reported minimal, if any, improvement. He continued to experience debilitating symptoms.
He was then referred to an infectious disease specialist. Upon evaluation, no signs of active or remote infectious process were discovered as a source of his symptoms. Prior to the rheumatology evaluation, the patient noted a few emergency department visits in the previous 12 months for intense, throbbing headaches, epistaxis and an extensive rash over his chest, worsening of his hearing and voice changes during the day. He required myringotomy and aspiration of his right ear. After many visits to the ENT office, the patient was referred to rheumatology for further evaluation.
A comprehensive review of systems revealed that at least for the previous 12 months, the patient had experienced fevers, chills, night sweats, extreme fatigue, lack of appetite, unintentional weight loss of 15 lbs., dyspnea on exertion, arthralgias, myalgias, heartburn, hiccups and frequent eructation. Occasionally, he would note swelling of his knees and ankles. The past two months, he had flu-like symptoms accompanied by severe headaches. He had two episodes of extensive purpuric rashes on his torso. He continued to complain about tinnitus, hoarseness and changes in his voice, especially toward the end of the day.
Several studies have shown that rituximab may be superior to cyclophosphamide in achieving clinical remission at six & 12 months after initiating induction therapy. Rituximab has also been shown to be superior to cyclophosphamide to achieve immunosuppression in patients with PR3-positive ANCA-vasculitis.
The patient made the remark that “only prednisone helps me.” He denied wrist or foot drop, dry mouth or eyes, malar rash, oral and nose ulcerations, alopecia or hair thinning, abdominal pain, blood in the urine or in the stool, and frothy urine.
