A minor "otitis media" can cause deafness, eye damage, and heart disease? Beware of this rare disease!

Grandma Li, who just celebrated her 60th birthday, got a strange disease this year. At the beginning of the year, she felt that her ears were not working well. At first, she thought it was normal to have a little hearing loss because of her old age, so she didn’t pay much attention to it. But within half a month, she felt that her hearing loss had worsened, and she also had dizziness , pus in her ears , and both ears were stuffy and blocked , which was very uncomfortable.

Grandma Li went to a local hospital and was diagnosed with " chronic secretory otitis media ". She underwent a myringotomy and a small tube was placed in the incision to allow fluid in the middle ear to flow out and air to enter, thereby reducing pressure in the middle ear.

Unexpectedly, after the operation, Grandma Li's hearing loss continued to worsen, the symptoms of pus in the ear did not seem to improve much, and her ears were painful, accompanied by severe headaches and tinnitus . Seeing this, her family was very anxious and decided to go to the last stop in the hearts of the people - Peking Union Medical College Hospital for a check-up.

At the outpatient department of ENT at Union Hospital, the doctor conducted a series of related examinations for Grandma Li. The pure tone audiometry results showed bilateral extreme sensorineural hearing loss , and the bacterial and fungal cultures of ear secretions were negative. The attentive doctor noticed that Grandma Li's eyes were a little red during the physical examination, so he asked about the condition of her eyes.

Grandma Li said that she had red eyes and stinging eyes as if sand had entered her eyes for more than half a month, but the ear condition was getting worse and worse, so she didn't pay attention. When the ENT doctor heard this, he felt that Grandma Li's illness was not like a single otitis media, so he suggested that Grandma Li go to the rheumatology and immunology department to see them and ask them to further investigate the cause.

After arriving at the rheumatology and immunology clinic, the doctor learned about Grandma Li's condition and carefully performed a physical examination on her. In addition to the symptoms of red eyes and deafness, the doctor found that Grandma Li had irregular heartbeat, uneven first heart sound , and a slow heart rate of only 40 beats per minute. After further questioning about Grandma Li's heart condition, it was learned that Grandma Li had high blood pressure , which was well controlled by taking antihypertensive drugs. She did not have coronary heart disease, but had felt a little anxious in the past month.

The doctor looked through Grandma Li's medical records and found an electrocardiogram from that time, which showed sinus rhythm. After confirming that Grandma Li's palpitations had recently occurred along with otitis media and red eyes , he immediately re-performed an electrocardiogram on Grandma Li.

The patient's outpatient electrocardiogram showed third-degree atrioventricular block

Sure enough, Grandma Li's heart also had problems. The electrocardiogram results showed that the atrioventricular conduction was completely disconnected, which was a typical manifestation of atrioventricular block .

Considering that at least three of Grandma Li’s organs are damaged (eyes, ears, and heart), especially the severe conduction block in her heart, which puts her at risk of sudden death, and that there may also be other undetected organ damage, the doctor recommended that Grandma Li be hospitalized immediately for treatment.

Soon, Grandma Li was admitted to the rheumatology and immunology ward. After completing routine examinations (liver and kidney function and urine routine were normal), she was quickly referred to a multidisciplinary consultation within the hospital and MDT diagnosis and treatment was organized.

The MDT diagnosis and treatment model of Peking Union Medical College has been established and implemented since the founding of the hospital in the 20th century, and has been gradually improved since the 21st century. It is usually initiated by the attending physician and multidisciplinary experts discuss cases to solve the diagnosis and treatment problems of difficult, severe and rare diseases.
Under the MDT model, experts from multiple disciplines can comprehensively analyze the patient's condition at the first time, integrate the opinions of various departments, cover all possible treatment strategies, and specify the most reasonable treatment plan for the patient, thereby avoiding misdiagnosis, maximizing patient benefits, and improving medical efficiency and quality.

In the multidisciplinary consultation, the ophthalmologist diagnosed immune scleritis and keratitis.

The cardiology consultation stated that echocardiography revealed aortic valve thickening, mild aortic regurgitation, and a minimal pericardial effusion.

Neurological consultation ruled out the possibility of cerebrovascular disease.

Dental consultation ruled out Sjögren's syndrome.

The radiology department read the patient's temporal bone CT, lung CT, cochlear MRI, and sinus enhanced CT after admission and found that in addition to the eyes, ears, and heart, there were also mastoiditis, bilateral sinusitis, and multiple small nodules and patchy shadows in the lungs.

The patient's lung CT results, the arrow (left) shows a lung nodule, the arrow (right) shows a small amount of pericardial effusion

Thin scan CT of the temporal bone. Arrows (left and upper right) indicate maxillary sinus inflammation, and arrows (lower right) indicate bilateral otomastoiditis.

The respiratory department considered that the multiple small lung nodules were caused by the primary disease and recommended follow-up.

The psychology department considered that the patient had a depressive episode accompanied by anxiety and physical symptoms due to bilateral hearing loss. In addition to relevant anti-anxiety and anti-depressant drug treatment, he also needed listening, empathy, and increased companionship and support from family members.

The otolaryngology department is considering improving hearing through cochlear implantation, but the condition is currently acute and surgical treatment may aggravate the condition. Elective surgery is required after the condition stabilizes. At the same time, the immunology department can assist in performing tissue biopsy of middle ear lesions and perform pathological diagnosis to clarify the primary disease.

Combined with the above multidisciplinary discussions, the patient had a chronic disease course involving multiple systems including the middle ear, mastoid, sclera, nose and sinuses, lungs, and heart. Combined with the elevated serum inflammatory indicators, although the anti-neutrophil cytoplasmic antibodies were negative, according to the 2022 European League Against Rheumatism/American College of Rheumatology GPA classification criteria, the patient was located in small blood vessel involvement, combined with the manifestations of nasal/sinusitis (3 points), sensorineural hearing loss (1 point), imaging consistent with sinusitis and mastoiditis (1 point), and pulmonary nodules (2 points), the classification criteria for granulomatosis with polyangiitis were met, so the doctor made the final diagnosis: granulomatosis with polyangiitis .

Granulomatosis with polyangiitis is a relatively rare autoimmune disease. It can affect multiple tissues and organs in the body. The middle ear, inner ear, and mastoid are commonly affected, with symptoms such as otitis, sensorineural hearing loss, vertigo, and chondritis. The eyes may appear red and edematous, and inflammation and obstruction of the nasolacrimal duct, conjunctivitis, scleritis, uveitis, or retinal vasculitis may occur, and may even lead to permanent blindness. The lungs may present as single or multiple pulmonary nodules, sometimes causing symptoms such as chest pain, shortness of breath, cough, and hemoptysis.

Grandma Li's symptoms can be well explained by this disease, and the middle ear soft tissue biopsy performed on Grandma Li by the ENT department also showed a large number of granulations in the middle ear cavity, which also well verified the doctor's diagnosis.

However, heart block is a rare clinical manifestation of granulomatosis with polyangiitis. When a rare disease meets a rare manifestation, it undoubtedly brings more difficult challenges to doctors' diagnosis and treatment. Considering the serious clinical symptoms of Grandma Li, the rheumatologist immediately treated her with sufficient glucocorticoids combined with immunosuppressants.

Under the hormone shock, Grandma Li's condition was quickly brought under control. The red eyes, headaches, and tinnitus symptoms gradually disappeared, pus discharge from both ears stopped, the degree of heart block gradually improved, and the number of lung nodules gradually decreased. After her condition stabilized, she underwent a "cochlear implant" and her hearing improved significantly. Finally, with nearly a year of outpatient follow-up and management by the rheumatology and immunology department, Grandma Li's condition was completely relieved.

Granulomatosis with polyangiitis is a type of anti-neutrophil cytoplasmic antibody-associated vasculitis, with a prevalence of about 1/25,000, an average age of onset of 40 years, and an unknown cause. The patient's prognosis depends on the severity and extent of the disease, as well as whether the treatment is timely. After treatment, about 80% of patients can achieve complete remission, but half of them eventually relapse; relapse can be maintained during remission or after treatment is terminated, and continued treatment or enhanced treatment can usually control the disease. Therefore, such patients must seek medical attention in a timely manner, follow up regularly, avoid relapse, and avoid delays that may delay the disease.

As for doctors, they need to do a good job in the diagnosis and differential diagnosis of such rare diseases, be good at observation and thinking in clinical diagnosis and treatment, cultivate systematic diagnosis and treatment thinking with patients as a whole, and actively combine multiple disciplines to provide timely and adequate treatment to patients, so as to minimize the pain of patients and improve their prognosis.

References

[1] He Xin, Zhang Guizhi, Lu Tao, et al. Heart block: granulomatosis with polyangiitis or IgG4-related disease? [J]. Journal of Peking Union Medical College, 2023, 14(1): 379-384.

[2] Robson JC, Grayson PC, Ponte C, et al. 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Granulomatosis With Polyangiitis［J］. Arthritis Rheumatol, 2022, 74: 393-399.

▌The popular science topic comes from the "Union Medical Journal" column on difficult and rare diseases [Union Medical Journal] Heart block: Granulomatosis with polyangiitis or IgG4-related disease?

Editor: Liu Yang and Zhao Na

Proofreading: Li Na, Li Yule, Dong Zhe, Li Huiwen

Producer: Wu Wenming

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