The symptoms associated with Takayasu arteritis (TA) are extremely diverse which ultimately leads to delays in diagnosing. This article describes the case of TA that presented with two coexisting unusual symptoms of neck pain and a prolonged cough. An 28 year old Japanese woman with a 6-month experience of colitis ulcerative was diagnosed with chronic neck pain in the left side with fever, cough, and neck pain. It was reported that the neck discomfort and fever began about five months ago. Her symptoms temporarily improved after nonsteroidal anti-inflammatory drugs however, they returned within a month of her most recent visit to the hospital. The presentation was coupled with dry cough. A physical examination showed a discrepancy in blood pressure and a systolic blood-pressure to be 10 mm lower on her left hand than her right arm. There was also an bilateral carotid bruit the left radial pulse was weak and radio-radial delay that did not show any cooling in the upper extremities and the presence of pulsatile linear tenderness in her left neck in the carotid common artery. There was no supraclavicular or infraclavicular noise was observed. The rate of erythrocytes being deposited was higher at 66 mm/hour. After receiving the images of a contrast-enhanced computed imaging the patient was diagnosed with TA. The symptoms all improved after prednisone treatment. In particular the cough and neck pain are both symptoms in late-stage of TA and are present in 9.7 percent and 1.5 percent of patients, respectively. While her non-specific symptoms may be easily misdiagnosed the repeated flare-up of symptoms requires careful examination of a specific physical exam.
In the end cough and neck pain are two common manifestations of TA that could result in doctors not diagnosing it correctly. It is crucial to identify neck cough and pain as the most common symptoms in patients suffering from TA.
Introduction
Takayasu arteritis (TA) is a major vasculitis of the vessel that mostly affects the aorta as well as its major branches [11. The majority of women suffer from the condition, and the age at which they begin to develop is typically in the range of 10 to 40 [22. The disease is widespread in prevalence, with the most incidence in Asia and, in particular, Japan [22. It is distinguished by a constant, fluctuating and waning in its clinical course [22. The first symptoms are generally not specific and later symptoms are caused by swelling of the affected arteries [33. The majority of patients have symptoms that are subtle as well as the recognition of TA is often delayed for months, or even years, particularly in patients with unusual symptoms [33. This report describes a patient with TA present with two rare signs of neck pain that is linear and a prolonged cough.
Case Presentation
An 28 year old Japanese woman who has an history of ulcerative colitis (UC) came to our hospital complaining of the left side of her neck, fever and cough. The diagnosis was made for UC when she began to experience haematochezia 6 months prior to her appearance and was started mesalazine. Since since then the frequency of haematochezia has reduced. It is believed that the symptoms associated with neck pain as well as fever first appeared about five months prior to this manifestation and improved within a months of anti-inflammatory nonsteroidal medication (NSAID) treatments. At the time her diagnosis was that she had viral lymphadenitis. However, her symptoms continued to recur within a month of her most recent visit to the hospital with an ongoing dry cough. The pain was within her anterior cervical region. it was a series of random moments of constant tingling with fluctuation and was cured by NSAIDs. The pain was sometimes so severe that it would wake her up in the midnight. She did not mention any aggravating factors or pain on that neck’s left side.
The vital signs of her included blood pressure of 104/66 mmHg , and 90/61 mmHg in her left and left arm respectively heart rate of 96 beats per minute; respiratory rate, 16 breaths per minute; and temperature of 36.8. Physical examination revealed that she was found to have bilateral carotid bruits, as well as slight pulsatile linear tenderness on the left side of her neck near the carotid vein. Furthermore the left radial artery was a weakly pulsed one that was delayed by radioradial. There was no right neck tenderness or thyroid tenderness or lymphadenopathy was observed. The extremities were all warm on the four limbs, but no supraclavicular nor infraclavicular bruit was observed. After obtaining a more detailed background, she reported an intense pain in the arm on her left, specifically when she was washing her hair with her left hand, or typing at work. She was in sick leave for a week prior to the presentation due to neck pain and the discomfort in her left arm. A full blood count revealed an extremely low level of haemoglobin in the range of 9.4 mg/dL (normal range: 13.7-16.8 g/dL) and an elevated platelet count of 448 10 3/mL (normal range between 158-348 3/mL). There was no evidence of leucocytosis. The metabolic panel that was the baseline for this study was not notable. Further laboratory tests showed an increased erythrocyte-sedimentation speed (ESR) at an average of 66 millimeters per hour (normal range 3- 15 mm/h) and a C-reactive Protein (CRP) levels of 13.3 mg/dL (normal range: 0-0.14 mg/dL). Thyroid-stimulating hormone levels were within normal ranges.
We suspected vasculitis of large vessels as she displayed blood pressure differences in her arms and carotid artery noise and tenderness as well as small radial pulses unilaterally arm claudication, and flare-ups of fever that were accompanied by inflammation. We therefore performed an image-based computed tomography with contrast enhancement, that revealed thickening of the wall of the arterial that affected the aortic arch the common carotid arteries as well as the left subclavian artery (Figures 1 2). In accordance with the American College of Rheumatology (ACR) classification criteria we identified the patient as having TA. She was prescribed 50 mg doses of prednisone (1 mg/kg) daily for four weeks. All symptoms were relieved and the dose of prednisone was gradually decreased to 20 mg and it was then maintained as a maintenance dosage. About 15 months after the beginning of treatment, neck pain returned; hence methotrexate prescription was given as an addition to prednisone. The patient’s symptoms improved steadily and, as a result, prednisone was gradually decreased to 10 mg a day. When she began methotrexate treatment the patient did not experience any symptoms that were exacerbating and her laboratory results such as CRP levels as well as ESR and ESR, were stable. consequently, both prednisone and methotrexate were totally tapered off 56 weeks following her initial appearance. Following the follow-up visit examination, the patient was found to be positive for HLA-B52.
Discussion
We report on the case of an individual with TA who was diagnosed with major complaints of neck pain and a prolonged cough, which was not apparent until a later stage of treatment. A diagnosis for TA can be a challenge particularly when symptoms that are rare are present. The symptoms of TA is very different based on the degree of vascular involvement and the severity of progress. The most frequently observed symptoms in TA are symptoms of the constitution that include weight loss and fever [12. Myalgias and arthritis have been identified as typical symptoms that occur in around 40% of patients [33. The symptoms are evident from an early stage, indicating the acute, non-specific inflammation symptoms.
Cough and neck pain typically occur in the final stage of TA in 9.7 percent [1 and 1.5 percent (see figure 2) of patients at the time that arterial insufficiency caused by thickening of the vessel wall due to inflammation is observed. The reason for coughing in TA isn’t well known; one theory is that inflammation in the vessels triggers the cough reflex by stimulating the vagus nerve [22. In this instance there was a co-existence of two uncommon symptoms that resembled symptoms of the upper respiratory tract delayed diagnosis. While her symptoms that are not specific can be misdiagnosed, her frequent exacerbation of symptoms required an attentive physical exam. This identified a blood pressure difference in her arms (more than 10 mmHg) and carotid artery bruit as well as weak unilateral radiatal pulses. The patient fulfilled all of the requirements of the ACR classification criteria and was diagnosed as having TA.
The connection of UC and TA is also documented in HLA-B52 positive patients similar to our case [5,66. Studies have previously revealed that the incidence of UC in patients suffering from TA was 6.4 percent, and the incidence in TA among UC patient was 0.21 percent [55. A previous analysis of 32 patients suffering from TA and UC discovered that the conditions mostly affected women (male and female proportion 1:1) and were more prevalent than in Asian regions (25 patients in Japan and two in India and two for Pakistan in Pakistan and Sri Lanka), with the average age of beginning either of the conditions being 25.6 years (15-56 years) [66. In this study, UC was antecedent to TA in 21 instances, TA was antecedent to UC in five cases and six cases were diagnosed with both conditions concurrently (see figure 6). In our case, the patient was experiencing neck pain that is a sign of late-stage symptoms about a month after being diagnosed with UC. The patient might have already had symptoms that were not specific at the moment of UC beginning. The prevalence of these disorders within Japan’s Japanese population is thought as a result of the large frequency in the HLAB52 variant in Japan [77.
This case highlights the importance of recognizing that cough and neck pain are unusual manifestations of TA and can be missed and ignored by doctors as signs of TA. This case also illustrates the fact that people with UC are at a greater chance of developing TA when compared in comparison to other people.
Conclusions
A diagnosis of TA can be difficult due to the lack of specific symptoms. It is crucial to recognize the presence of cough and neck pain as an uncommon manifestation of TA. This case highlights that those who have an background of UC are susceptible to developing TA Another rare disorder in those who are positive for HLA-B52.
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