AAV in SSc is described from the point of view of MPA. Some of reported SSc cases with AAV are thought to exhibit the characteristic clinical manifestations of MPA, although ANCA positivity in SSc is uncommon. MPA is clinically characterized by a multisystemic disease such as RPGN, pulmonary hemorrhage, mononeuritis, and skin involvement, as well as other manifestations in conjunction with high levels of inflammatory activity such as high ESR or CRP. It is also characterized by a high frequency of MPO-ANCA, showing predominant pANCA by IIF. When rapid renal failure or RPGN with active urine sediments, pulmonary hemorrhage and/or systemic inflammatory manifestations are observed in patients with SSc having positive ANCA, the possibility of MPA should always be considered. If SSc patients with MPA have life-threatening visceral involvement such as the above clinical manifestations, the patients should be treated with induction therapy using cyclophosphamide, methotrexate, corticosteroids, or plasmapheresis, etc. according to the severity of the disease soon after the diagnosis of MPA. It is important not to overlook characteristic clinical manifestations of AAV during the course of the disease in SSc in order to diagnose MPA early.
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