Severe Raynaud's phenomenon and Sjögren's syndrome with ferocious gangrene change and auto-amputation
Sjögren’s syndrome is a chronic autoimmune exocrinopathy that can attack multiple organs, such as the pulmonary, renal, neurological and vascular systems.1 Raynaud’s phenomenon is a clinical manifestation in which artery spasms cause reduced blood flow with paleness, cyanosis and even gangrenous change.2
A 47-year-old female non-smoker presented with rapid and progressive interchangeable paleness and cyanosis over the toes (Fig 1a and b) for 3 weeks. She also suffered from dry eye, xerophthalmia, numbness, and pinprick sensation over the bilateral lower limbs. Laboratory studies were positive for antinuclear antibodies (1:5120, speckle pattern) and a high titre of rheumatoid factor at 114 IU/mL. Anti-citrullinated protein antibody, anti-Scl-70-antibody, anti-Smith-antibody, anti-ribonucleoprotein-antibody and anti-ds-DNA-antibody tests were all negative. After further investigation, Sjögren’s syndrome was impressed with positive anti-Ro-antibodies (more than 240 U/mL), lacrimal gland function was reduced by Schirmer’s test and sialoscintigraphy showed dysfunction of salivary secretion.1 Tests for hepatitis C virus, human immunodeficiency virus, antiphospholipid antibodies and cryoglobulin were all normal. Oral nifedipine, sildenafil, cilostazol, clopidogrel, intravenous prostaglandin injection, hyperbaric oxygen therapy and epidural lumbar sympathetic nerve block (medical sympathectomy) halted this severe ischemic process related to progressive toes’ gangrene change. After aggressive treatment, only the right 5th toe was partially auto-amputated (Fig 1c).
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- © Royal College of Physicians 2019. All rights reserved.
References
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- Shiboski CH
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- Wigley FM
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