Thrombotic thrombocytopenic purpura in the setting of systemic sclerosis

TTP in association with SSc has been reported rarely. The diagnosis of TTP, and the distinction from SRC, may be challenging, as these cases may resemble SRC. However, the correct diagnosis is critical because treatment differs substantially for each of these life-threatening conditions. Currently, the assessment of von Willebrand factor cleaving protease activity may assist in making this distinction.