Major Scleroderma Emergencies

Although scleroderma (systemic sclerosis; SSc) is often associated with major internal organ involvement, acute emergencies may occur either independently or in association with progression of existing organ involvement. Two case examples illustrate the complexity of acute management of these emergencies. The first case with cold reactive (anti-C3d) antibody with type 1 cryoglobulin associated with dysproteinaemic state with IgM paraprotein illustrates an uncommon important cause for a common manifestation of SSc that requires specific intervention in addition to vascular support. The second case illustrates the complexity of multiorgan disease contributing to progressive dyspnoea and the identification of dominant pathological process considered to be the critical driver of the current symptomatology with an appreciation that failure to act in a timely manner will lead to precipitous deterioration of potentially reversible component(s) of the disease process in addition to meticulous monitoring of response over time.