<p>Sjögren’s disease (SjD) is a chronic systemic autoimmune disorder primarily affecting the exocrine glands, while Takayasu arteritis (TAK) is a large-vessel vasculitis involving the aorta and its major branches. Although SjD frequently overlaps with other autoimmune diseases, its coexistence with TAK has been reported only rarely. A 47-year-old Turkish woman with seropositive SjD presented with constitutional symptoms, upper extremity claudication, and asymmetric peripheral pulses. Laboratory evaluation revealed elevated inflammatory markers while antiphospholipid antibodies were negative. Computed tomography angiography demonstrated stenotic lesions and vessel wall thickening involving the left subclavian, axillary, carotid, and brachiocephalic arteries. Additional positron emission tomography/computed tomography showed increased vascular uptake consistent with active large-vessel inflammation. Based on clinical, laboratory, and imaging findings, the patient fulfilled the criteria for TAK. Treatment resulted in marked clinical improvement and normalization of inflammatory markers, with sustained remission during a 2-year follow-up period. The coexistence of SjD and TAK is exceptionally rare, with only a limited number of cases reported to date. This case represents the first reported Turkish patient and adds to the growing evidence that large-vessel vasculitis should be considered in SjD patients who develop unexplained vascular or systemic inflammatory features.</p>

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Coexistence of Sjögren’s disease and Takayasu arteritis in a woman: a case-based review

  • Dilara Bulut Gökten,
  • Fatma Yümün Kavak,
  • Ömer Atakan Soğur,
  • Ridvan Mercan

摘要

Sjögren’s disease (SjD) is a chronic systemic autoimmune disorder primarily affecting the exocrine glands, while Takayasu arteritis (TAK) is a large-vessel vasculitis involving the aorta and its major branches. Although SjD frequently overlaps with other autoimmune diseases, its coexistence with TAK has been reported only rarely. A 47-year-old Turkish woman with seropositive SjD presented with constitutional symptoms, upper extremity claudication, and asymmetric peripheral pulses. Laboratory evaluation revealed elevated inflammatory markers while antiphospholipid antibodies were negative. Computed tomography angiography demonstrated stenotic lesions and vessel wall thickening involving the left subclavian, axillary, carotid, and brachiocephalic arteries. Additional positron emission tomography/computed tomography showed increased vascular uptake consistent with active large-vessel inflammation. Based on clinical, laboratory, and imaging findings, the patient fulfilled the criteria for TAK. Treatment resulted in marked clinical improvement and normalization of inflammatory markers, with sustained remission during a 2-year follow-up period. The coexistence of SjD and TAK is exceptionally rare, with only a limited number of cases reported to date. This case represents the first reported Turkish patient and adds to the growing evidence that large-vessel vasculitis should be considered in SjD patients who develop unexplained vascular or systemic inflammatory features.