430. Arthritis as a atypical presenting feature in a case of Takayasu arteritis
Creators: name: Hazarika1, Kasturi
Publisher: name: Zenodo
Year: 2022
Subjects: Vasculitis
ANCA
Abstracts
Giant Cell Arthritis
MPA
GPA
EGPA
Takayasu
IgA vasculitis

description: <strong>Presentation of Case:</strong> A 21-year-old girl was on our follow up as a case of polyarticular juvenile idiopathic arthritis (JIA) for the past 6 years. Her complaints of joint pain started when she was around 10 years of age. It was subacute in onset, symmetrical, additive arthritis involving wrists, elbow, knees, and shoulders. There was no history of inflammatory low back ache, no history suggestive of uveitis and no personal or family history of psoriasis. There was history of intermittent fever which wasn’t documented and often attributed by the patient to severe pain. She was on methotrexate full dose appropriate to her body weight for the initial two years to which her arthritis had responded well. In the subsequent years methotrexate was tapered and stopped in view of growing intolerance of the patient towards methotrexate and her joint complaints being in remission. After being off medication for around a year and a half, patient visited our tertiary care hospital in view of unrelenting fever of 2 months duration, increased fatiguability and a recently developed pounding sensation in the neck. Patient had documented weight loss of around 5 kgs in the past 2 months. No features suggestive of photosensitivity, Raynaud’s phenomenon, or other features suggestive of any underlying connective tissue disease was elicited either in past or present. No history suggestive of recurrent oral and genital ulceration She was found to be hypertensive with unequal blood pressure in both arms and absent lower limb pulses femoral onwards. There was also a wide pulse pressure in both upper limbs. Cardiovascular examination was suggestive of thrill in parasternal, aortic, pulmonary, and bilateral supraclavicular areas. A diastolic decrescendo murmur was observed which was best heard in the aortic area. Bruit were present over bilateral renal angle, over the subclavian, bilateral carotid and over bilateral interscapular areas. Suspecting a large vessel vasculitis, we went ahead with the investigations. <strong>Diagnostic Testing:</strong> A hemogram was suggestive of normocytic normochromic anaemia, with thrombocytosis and very high erythrocyte sedimentation rate (ESR) of 115 at the end of first hour by wintergreen method. In retrospect, when we looked into the previous records, a very high ESR with thrombocytosis was also present in her initial presentation six years prior. Radiographs of hands and knees were almost normal given the long history of arthritis. Echocardiogram revealed a left ventricular ejection fraction of 66%, mild aortic regurgitation and mild mitral regurgitation. Computerised tomography (CT) angiography of aorta and its branches revealed significant involvement of the aorta and its branches. There was prominent arch of aorta with narrowing of right brachiocephalic at its origin (Figure 1A). There was long segment narrowing with stenosis of descending thoracic aorta in pre ductal region to supra renal region (Figure 1B). The abdominal aorta was reported to have abrupt narrowing with non-opacification extending from infra renal region to the common iliac artery with opacification of bilateral common iliac artery filled by extensive collaterals (Figure 1C). Anti-nuclear antibody (ANA) test done by indirect immunofluorescence (IIF) was negative. <strong>Differential &amp; Final Diagnosis:</strong> To summarize we have a young girl with history of arthritis and on successful treatment with methotrexate, who subsequent to tapering and stopping methotrexate presented with features suggestive of large vessel vasculitis. Our first differential was Takayasu’s arteritis. The patient however never complained of typical symptoms of claudication seen in such extensive disease. A possibility of systemic lupus erythematosus (SLE) with the rare feature of large vessel vasculitis was ruled out by the absence of any other clinical or immunologic criteria. Infectious causes of aortitis like tuberculosis were ruled out by a normal chest radiograph, negative Mantoux and a negative quantiferon gold test. She had no history suggestive of recurrent oro-genital ulceration in her entire disease duration of almost ten years making it unlikely to be Behcet disease. Given her young age of onset and characteristic pulse and blood pressure inequality along with characteristic angiographic evidence, our patient fulfilled the American college of rheumatology (ACR) 1990 criteria for Takayasu arteritis.<sup>1</sup>However, the long history of peripheral arthritis preceding the obvious features of vasculitis was very odd in this patient. Joint involvement as a presenting feature of Takayasu arteritis is very rare found only in 6-10% of patients.<sup>2 </sup>Whether the use of methotrexate in this patient masked her physical findings of large vessel vasculitis for the past 5-6 years remains unknown. She however never received high dose steroids as recommended for Takayasu arteritis.<sup>3</sup> The grossly wide pulse pressure of in absence of significant aortic regurgitation proven by investigations, may be explained by reduced elasticity of aorta and widespread collaterals. <strong>Discussion of Management</strong>: Our patient was on methotrexate for her arthritis and had developed severe intolerance towards it. She was from poor financial background and without any health insurance because of which she could not afford either tocilizumab or anti- tumor necrosis factor inhibitors as conventionally recommended.<sup>3</sup> So we started her on 1mg/kg steroids and pulse cyclophosphamide after counselling and discussion with family members. She was also started on anti-hypertensives and a cardiology consultation was also sought for possible intervention. <strong>Conclusions:</strong> A patient presenting with one immunologic feature as arthritis may in distant future evolve into a dangerous and life -threatening disease as vasculitis. Although we may have read of literatures quoting atypical features of diseases, they never strike us unless we encounter them for ourselves. Lastly, the importance of a simple clinical examination as looking for pulse and blood pressure inequality goes a long way in detecting such an atypical presentation. <strong>Disclosures:</strong> None