An 87-year-old woman with Sjogren's syndrome diagnosed eight years ago was admitted to a nearby hospital for bilateral lower limb muscle weakness. She was palliatively treated following a diagnosis of spinal canal stenosis. However, she developed fever, wheezing, and bloody sputum, and a computed tomography scan of her chest showed bilateral consolidation. Despite antibiotic treatment, her condition became worse and she was transferred to our hospital on the 25th day of hospitalization. Unfortunately, cardiopulmonary arrest occurred immediately after arrival at our hospital and an autopsy was performed.
Severe pneumorrhagia was seen mainly in the upper lungs, and follicular bronchiolitis in the lower lobes without bleeding. Vasculitis with fibrinoid necrosis in whole body organs such as the right ventricle wall, spleen, digestive tract, liver, bladder, uterus, and both ovaries was observed. These findings suggested that vasculitis with inflammation of the small blood vessels accompanied Sjogren's syndrome.
Systemic vasculitis associated with Sjogren's syndrome resulting in fatal alveolar hemorrhage is extremely rare. Only a few cases of cryoglobulinemic vasculitis and idiopathic pulmonary hemosiderosis have been reported.