Cogan’s Syndrome is characterized by corneal inflammation associated with vestibular cochlear disease, progressively worsening and leading to hearing loss within two years. There are, however, atypical cases with other lesions or prolonged disease progression beyond two years.
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| Eye manifestations in Cogan’s Syndrome (Photo: TTO) |
Cogan’s Syndrome is commonly encountered in young individuals, with over 30% of cases presenting within the context of systemic diseases. The organs frequently involved include the cardiovascular system, kidneys, musculoskeletal system, nervous system, digestive system, skin, and mucous membranes. Tests to detect the syndrome include inflammation syndromes and immune system abnormalities.
To date, the exact pathological mechanism has not been clearly identified. The condition predominantly affects individuals aged 20 to 40 years. The incidence is fairly similar between genders: approximately 52% in males and 48% in females. HLA factor analysis indicates that affected individuals possess HLA A9, BW35, and CW4. Cogan’s Syndrome may partly manifest as a component of systemic diseases such as rheumatic disease, Graves’ disease, and Crohn’s disease.
Recent research shows a close association between this syndrome and perivascular inflammation. Additionally, scientists have pointed out the role of Chlamydia trachomatis, a sexually transmitted infection, as over 50% of individuals exhibiting Cogan’s Syndrome test positive for Chlamydia antibodies.
Common Symptoms
– Eye lesions: occur in 42% of cases, typically affecting both eyes. Symptoms include eye pain (50%), redness (74%), photophobia (50%), tearing (50%), and reduced vision (42%). Upon examination, inflammatory nodular infiltration may be observed at the posterior cornea. Other lesions can include keratitis, uveitis, and orbital inflammation. Severe manifestations like corneal ulcers and perforations are rarer. Generally, patients experience gradual vision loss, although some may become blind in a very short time.
– Vestibular cochlear signs: The disease often begins with vestibular syndromes and profound deafness. Symptoms may include tinnitus, which resolves and then reappears with greater severity. Patients may also experience nausea, nystagmus, and balance issues.
Symptoms typically manifest over a few days and then gradually diminish. However, subsequent relapses may worsen the condition and lead to reduced hearing. Most cases result in complete deafness within 2 to 3 years. Standard X-rays may miss lesions due to a lack of apparent symptoms, but CT or MRI can reveal calcification, obstruction of the semicircular canals, vestibule, and cochlea.
– Systemic signs: Fever occurs in 39% of cases. Weight loss is noted in 26% of cases. Patients often experience fatigue, loss of appetite, and difficulty walking, with some losing more than 10 kg in a short period.
– Symptoms in other organs: Cardiovascular system issues may include aortic regurgitation, mitral regurgitation, and myocarditis. Vascular manifestations can encompass arterial wall thickening, limb ischemia, abdominal pain due to vascular occlusion, intermittent claudication, and Raynaud’s syndrome. Imaging may reveal vascular blockages, diffuse thrombosis, and aneurysms. Depending on the affected blood vessels, organs like the kidneys, brain, and heart may demonstrate symptoms such as renal failure, hypertension, cerebrovascular accidents, and myocardial infarction.
Skeletal system signs include arthritis, joint swelling, joint deformities, osteoarthritis, muscle pain, and muscle atrophy. Patients often present with dry, atrophic skin and mucous membranes, and may experience itchy papules and erythema. Muscle reflexes may be diminished, with sensory neuropathy and chronic dermatitis. Gastrointestinal symptoms may include diarrhea, blood in the stool, and prolonged abdominal pain.
In many cases, hepatomegaly and splenomegaly can be palpated. Pulmonary manifestations may include interstitial pneumonia, with patients experiencing dry cough or hemoptysis. During acute episodes, patients may have mild shortness of breath, pleuritis, or pleural effusion. Occasionally, patients may exhibit associated endocrine symptoms such as obesity, hyperthyroidism, hypothyroidism, or testicular atrophy.
Diagnosis: Laboratory tests typically yield limited results and can only be detected during acute episodes. Leukocytosis, thrombocytosis, mild anemia, and coagulation abnormalities may be present. Inflammatory responses are evident with elevated erythrocyte sedimentation rate, increased fibrinogen, and elevated gamma and alpha-2 globulins. Liver function is usually within normal limits. Urinalysis may show protein and red blood cells. Lumbar puncture may reveal elevated protein levels and occasionally a few white blood cells.
The disease progresses through multiple acute episodes, with increasing severity and gradual loss of organ function. The most evident change is the reduction in hearing. Within 1 to 2 years, patients may require hearing aids or vestibular surgery. Furthermore, once damage occurs to vital organs such as the heart and kidneys, the disease can take a more severe course, significantly impacting quality of life.
Treatment and Progression: Treatment primarily involves corticosteroids, commonly prednisolone at a dosage of 1 mg/kg/day. This medication is effective during disease flare-ups. Early treatment can prevent damage to the eyes and systemic involvement. However, the response in the ears is limited, and hearing loss is often challenging to improve. When corticosteroid treatment is ineffective, additional medications such as azathioprine, cyclophosphamide, cyclosporin A, or methotrexate may be considered.
