Background Clinical courses of Takayasu arteritis are of great variety. Its non-specific initial symptoms can cause the delay in diagnosis and lead to poor prognosis. However, the symptoms of very early phase of Takayasu arteritis and their effects on diagnosis delay and prognosis are unclear.
Objectives To clarify the characteristics of initial symptoms of Takayasu arteritis, the delay in diagnosis, and its relationship with prognosis.
Methods The consecutive patients with Takayasu arteritis with analysable information in our hospital were enrolled. Initial symptoms, laboratory findings before diagnosis, the duration from symptom onset to diagnosis, and prognosis were investigated. Initial symptoms were divided into 7 groups; cranial symptoms (dizziness, syncope, headache, neck pain, hemi-paralysis, and jaw claudication), visual symptoms (vision loss and visual field loss), extremities symptoms (claudication of extremities, coldness of limbs, bilateral difference in blood pressure, and limb numbness), cardiac/aortic symptoms (dyspnea on exertion, palpitation, and chest compression), hypertension, general illness (fever, fatigue, body weight loss, and arthralgia), and abnormal medical examinations (heart murmur, bruit on any extremities, and abnormal chest X-ray). Poor outcome was defined as a cardiovascular surgery or death.
Results A total of 98 patients were enrolled with the median observation period of 12.1 years (range, 1 month to 59 years). Eighty-seven (88.7%) were female and the mean age at diagnosis was 37 years. The mean duration from the initial symptoms to diagnosis was 600 days. Thirty-four (34.7%) patients had poor outcomes. The initial symptoms before diagnosis were cranial symptoms in 25%, visual symptoms in 5%, extremities symptoms in 20%, cardiac/aortic symptoms in 7%, hypertension in 9%, general illness in 26%, and abnormal medical examinations in 8%. The duration from symptom onset to diagnosis was 792, 52, 567, 752, 1318, 293 and 1014 days (p=0.10), respectively; the rate of poor outcome was 28, 25, 38, 86, 75, 22, and 25% (p=0.010), respectively. The duration from symptom onset to diagnosis was longer in the patients with poor prognosis than those without (837 vs 493 days, p=0.06). The patients with extremities symptoms were younger (29.6 vs 38.9 years, p=0.026) than those with the other symptoms, and patients with cardiac/aortic symptoms were older (49.7 vs 36.3 years, p=0.044). The patients without general illness showed lower levels of C-reactive protein (3.26 vs 5.86 mg/dl, p=0.048), erythrocyte sedimentation rate (38.3 vs 66.6 mm/h, p=0.01), and platelet counts (29.4*104 vs 34.0*104/μl, p=0.09), and resulted in poor outcome more frequently than those with (42.6 vs 22.6%, p=0.058).
Conclusions The initial symptoms of Takayasu arteritis before diagnosis varied widely, and majority of them were non-specific. Lacking inflammatory signs were related with delayed diagnosis and poor prognosis.
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Acknowledgements We thank all the members of the Division of Rheumatology.
Disclosure of Interest None declared