When an Ear Infection Isn't an Ear Infection: A Real Case of Granulomatosis with Polyangiitis (Wegener's Disease)

One of the most challenging aspects of medicine is that diseases do not always present in the way we expect.

Some conditions mimic infections, orthopedic problems, neurological disorders, or even psychological illnesses. Autoimmune diseases are particularly notorious for this.

The following story, shared with the patient's permission and with identifying details removed, illustrates how granulomatosis with polyangiitis (GPA), formerly called Wegener's granulomatosis, initially appeared to be a stubborn ear infection. It is one patient's course, not a template for how the disease behaves in general.

A referral during a busy clinic day

Several years ago, during a particularly busy clinic day, an ophthalmologist colleague called me regarding one of her close friends.

She explained that he had developed severe joint pains over the previous five days. The pain had affected multiple joints, including fingers, wrists, elbows, shoulders, knees and ankles.

My clinic schedule was already full, and I initially suggested arranging an appointment over the next few days. However, my colleague strongly felt he needed to be evaluated sooner. I agreed to see him briefly between appointments.

That decision proved to be important.

A medical history that told a story

As I reviewed his history, a pattern began to emerge.

Three months earlier, he had started experiencing recurrent nosebleeds. Shortly afterward, he was diagnosed with sinusitis. Then he developed what was thought to be a right ear infection.

The ear problem became increasingly difficult to treat. Despite multiple courses of antibiotics, the symptoms persisted. His condition eventually became severe enough to require hospitalization because of significant dizziness. Ear tubes were inserted in an attempt to treat the presumed resistant infection.

Ten days before our consultation, another symptom appeared: anterior uveitis, an inflammatory condition affecting the eye. Throughout this period, he had also been experiencing low-grade fever.

Now, on top of all these problems, he had developed sudden inflammation and pain affecting multiple joints.

When multiple symptoms are considered together

General point: symptoms are often viewed separately — nosebleeds, sinusitis, ear infection, eye inflammation, joint pain, fever. It is sometimes useful to ask a different question:

"Could these symptoms be part of the same disease?"

In this patient, the combination of persistent upper-airway problems with eye inflammation, fever and sudden polyarthritis raised the possibility of a systemic vasculitis.

What GPA is

GPA is an ANCA-associated vasculitis (AAV): a group of conditions involving inflammation of small blood vessels that can affect multiple organs. Involvement commonly includes the upper airways (nose, sinuses, ears), the lungs and the kidneys, and other sites — eyes, joints, skin and nerves — can also be affected.¹

Importantly, recurrent ear or sinus infections do not ordinarily indicate GPA; they are common and usually have ordinary causes. What may justify broader assessment is persistent or unusual ENT symptoms accompanied by systemic features — such as fever, weight loss, kidney abnormalities, lung symptoms, eye inflammation, nerve symptoms or raised inflammatory markers.

Why the ear problem was not settling

In this patient, the ear inflammation was ultimately immune-mediated rather than bacterial, so antibiotics were not addressing the underlying process.

As a general point, however, antibiotics failing does not prove that an illness is autoimmune. Treatment failure has many explanations — resistant or atypical organisms, inadequate drainage, structural problems, fungal infection or an incorrect original diagnosis. It is one observation among many, not a diagnostic test.

Investigation

I explained to the patient and his family that I suspected an autoimmune disease rather than a persistent bacterial infection, and recommended blood tests, assessment for organ involvement, and planning of treatment.

General point: no single investigation establishes the diagnosis. Blood tests, ANCA serology and imaging each contribute, but ANCA can be negative in some patients and positive in other conditions, so results are interpreted alongside the clinical picture. Biopsy and other investigations may help establish the diagnosis when clinically appropriate; the investigations chosen depend on which organs appear to be involved rather than following one fixed protocol for every patient.

The family's understandable concern

Later that evening, I received a call from the patient's son. He was understandably worried. If his father truly had an ear infection, wouldn't steroids make the infection worse?

His concern was entirely reasonable. I explained why I did not think we were dealing with a bacterial infection and that delaying treatment carried its own risks. The family preferred to wait for the results of a planned sinus biopsy before starting treatment.

The diagnosis is confirmed

Several days later, the biopsy results became available. They confirmed the diagnosis of granulomatosis with polyangiitis.

General point on treatment: treatment depends on disease severity and organ involvement. For organ-threatening or life-threatening ANCA-associated vasculitis, current recommendations use remission-induction immunosuppression — commonly rituximab or cyclophosphamide — with glucocorticoids, followed by an appropriate maintenance strategy.¹

In this patient's case, treatment was started with glucocorticoids alongside an immunosuppressive agent, with the aim of controlling the disease while reducing the steroid dose as the disease came under control. Within a week:

• Joint pain improved significantly
• Fever subsided
• General well-being improved
• Inflammation began coming under control

Unfortunately, in this particular patient some hearing loss remained because of damage that had already occurred before the diagnosis was established. That outcome is specific to his case and does not apply to every patient with GPA.

His daughter later joked that her father did not have hearing loss at all — he simply had "selective hearing" and could hear exactly what he wanted to hear and conveniently miss everything else. Thankfully, he maintained his sense of humor.

A memorable patient

The gentleman continued to follow up with me for many years. At every visit, he would stop at the door, place his hands together in a traditional gesture of respect, smile warmly, and say:

"Dr. Darwish, you saved me. I will never forget that."

While medicine is always a team effort involving many healthcare professionals, I have never forgotten him either.

Granulomatosis with polyangiitis: features that can occur

GPA is a rare autoimmune disease classified as a vasculitis, meaning inflammation of blood vessels. Features that may occur — in varying combinations, and not all in any one patient — include:

• Chronic sinusitis
• Recurrent nosebleeds
• Ear pain, persistent middle ear inflammation or hearing loss
• Eye inflammation (uveitis or scleritis)
• Joint pain and swelling
• Fever and fatigue
• Lung abnormalities
• Kidney involvement

Prompt recognition and appropriate treatment are important because uncontrolled vasculitis can cause irreversible organ damage, although treatment cannot always reverse damage that has already occurred.¹

Key takeaway

Most sinus and ear problems are ordinary and resolve. But where ENT symptoms are persistent or unusual and occur alongside systemic, kidney, lung, eye, nerve or inflammatory features, it is reasonable to consider whether an underlying systemic disease could explain the whole picture.

This case is shared with permission. Identifying details have been modified or omitted to protect patient privacy.