Granulomatosis with polyangiitis (GPA) may sometimes manifest as facial paralysis and ear pain — a potentially challenging diagnosis, according to the case report of a teenage patient.
GPA, also known as Wegener’s granulomatosis, is an autoimmune disorder with symptoms of tissue and blood vessel inflammation, commonly affecting the airways and kidneys.
Nearly 90% of GPA cases are triggered by anti-neutrophil cytoplasmic autoantibodies (ANCA) targeting the proteinase 3 (PR3) protein.
A team at the University of Cincinnati College of Medicine reported the case of a 14-year-old girl who arrived at the emergency room due to facial paralysis on one side and pain in both ears lasting for eight weeks.
Her primary care physician assumed she had an ear infection (otitis), and prescribed antibiotic ear drops. But her symptoms worsened and she experienced right facial swelling and a numbing sensation. Based on her progression, she was diagnosed with Bell’s palsy (temporary facial paralysis) and started treatment with prednisone.
At first, she responded to treatment and her symptoms resolved completely. But by the third cycle of treatment, she had persistent incomplete eye closure, and later developed ear pain, right neck pain, and severe back pain.
An additional clinical history revealed that she also had fever, appetite change, decreased activity, shortness of breath, cough, vomiting, and bilateral ear pain and pressure, with loss of right-side hearing.
At this stage, a physical exam did not reveal any eye and neck changes. Her ears showed signs of infection with thickened tympanic membranes, which could help explain the decreased hearing. Blood tests showed an increased white blood cell count and high C-reactive protein, suggesting an infection. She started antibiotic treatment with Rocephin (ceftriaxone).
Further evaluations revealed multiple masses in both lungs and inflammation with extensive areas of stiff soft tissue (opacification) in the ears. The clinical team decided to treat her ears with surgery.
Although first evaluations suggested an infectious disease, tests were negative for bacteria or fungi, and her condition continued to progress despite antibiotic treatment. Also, her gums swelled and blood was found in her urine.
Biopsies from her lungs and ears revealed granulomatous inflammation with localized tissue death and inflamed blood vessels (vasculitis). Also, her PR3 autoantibody levels were nearly seven times higher than the upper normal limit. Collectively, these features were consistent with a diagnosis of GPA.
She started an aggressive immunosuppression treatment regimen with methylprednisone, Rituxan (rituximab), and cyclophosphamide.
Ear symptoms have been reported in 19-45% of GPA cases, but when the first manifestation is ear-related, “GPA diagnosis can be difficult,” the authors said.
“GPA should be considered when patients do not improve despite adequate treatment of [ear] symptoms, when patients have unspecific symptoms suggesting systemic disease, or when other organs are involved,” the team wrote.