Abstract

Takayasu arteritis is a rare systemic large vessel vasculitis affecting the aorta and its branches. Sarcoidosis, too, is an inflammatory disease. Both entities are granulomatous conditions with a question of possible linkquestionable association in their etiopathogenesis. Only a few cases of their coexistence have been reported in the literature. To our knowledge, no such cases have been reported from Nepal. We report a Nepalese woman who presented with non-productive cough, progressive shortness of breath and chest tightness of 3 years duration. She had a history of recurrent bilateral granulomatous uveitis over the previous 3 years. Examination revealed clubbing of digits, absent pulses over the left radial, ulnar and brachial arteries, and a weak pulse over the right arm including the bilateral carotid arteries. Pulmonary function test showed restrictive pattern, a high-resolution computed tomography (HRCT) scan of the chest revealed findings suggestive of pulmonary sarcoidosis. A CT aortogram suggested large vessel vasculitis. Bronchoscopy with biopsy revealed granulomatous inflammation, negative for malignancy and tuberculosis. She was hence, diagnosed with co-existing Takayasu arteritis and sarcoidosis, and treated with Prednisolone 60 mg once daily with dramatic improvement over 4 days and was discharged stable on domiciliary oxygen. She is currently on azathioprine 50 mg, prednisolone 10 mg without the need for supplemental oxygen. This case report highlights the importance of a proper physical examination as a guide to the use of modern technology in making a correct diagnosis. Furthermore, in countries where tuberculosis is endemic, it should always come as the most important differential diagnosis of granulomatous inflammation.

Highlights

  • Takayasu arteritis is a rare systemic large vessel vasculitis affecting the aorta and its branches

  • We report a patient from Nepal who was diagnosed with sarcoidosis and Takayasu arteritis (TA) concomitantly, and aim to contribute to literature regarding patients with these coexisting diseases

  • With the diagnosis of sarcoidosis with coexisting Takayasu arteritis, she was treated with prednisolone 60 mg once daily (1 mg/kg/day) along with supportive treatment including oxygen, chest physiotherapy, bone protection with calcium and vitamin D, and thromboembolism prophylaxis with daily 60 mg of subcutaneous enoxaparin

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Summary

17 Apr 2020

The diagnosis of sarcoidosis (pulmonary and ocular) was made On examination, she was afebrile with a blood pressure of 130/80 mmHg in the right arm and heart rate was 80/minute. Though images not available for the report, there was stenosis of right axillary, brachial artery and its branches, with sparing of abdominal aorta up to the lower extremities This was suggestive of large vessel vasculitis in the upper extremities, Takayasu arteritis (Type I). With the diagnosis of sarcoidosis with coexisting Takayasu arteritis, she was treated with prednisolone 60 mg once daily (1 mg/kg/day) along with supportive treatment including oxygen, chest physiotherapy, bone protection with calcium and vitamin D, and thromboembolism prophylaxis with daily 60 mg of subcutaneous enoxaparin. Ophthalmology examination showed bilateral granulomatous uveitis and was treated with topical prednisolone drops She dramatically improved over 3–4 days and required only 1 liter/minute of oxygen support. She is on regular follow up every 3 months with a pulmonologist

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