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Human COPA mutations affecting retrograde Golgi-to-endoplasmic reticulum (ER) protein transport cause diffuse alveolar haemorrhage (DAH) and ER stress ("COPA syndrome"). SLE patients also can develop DAH. C57BL/6 (B6) mice with pristane-induced lupus develop monocyte-dependent DAH indistinguishable from human DAH, whereas BALB/c mice are resistant. The study examined Copa and ER stress in pristane-induced lupus. Copa expression, ER stress, vascular injury, and apoptosis were assessed in mice, and COPA was quantified in blood from SLE patients. The pathogenesis of DAH in SLE and COPA syndrome may overlap.
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