Prompt plasmapheresis successfully rescue pulmonary-renal syndrome caused by ANCA-negative microscopic polyangiitis.
Pulmonary-renal syndrome (PRS) associated with antineutrophil cytoplasmic antibodies (ANCA)-negative microscopic polyangiitis (MPA) is relatively rare, and the effects of plasmapheresis on these patients remain unclear. Here, we report the case of a 66-year-old man who presented with fever, acute renal failure, thrombocytopenia, and sudden onset of diffuse pulmonary hemorrhage. Prompt plasmapheresis and concurrent pulse therapy with methylprednisolone effectively rescued his pulmonary-renal syndrome. The patient was then diagnosed with MPA on the basis of typical histological findings and the absence of surrogate markers of Wegener's granulomatosis and Churg-Strauss syndrome. This case demonstrates the therapeutic effects of plasmapheresis on ANCA-negative MPA and highlights the necessity of prompt plasmapheresis for not only resolving pulmonary hemorrhage but also increasing the likelihood of renal function restoration in patients with PRS.
Wang
Chih-Chiang
CC
Division of Nephrology, Department of Internal Medicine, Tri-Service General Hospital, Number 325, Section 2, Cheng-Kung Road, Neihu 114, Taipei, Taiwan, Republic of China.
Shiang
Jeng-Chuan
JC
Tsai
Ming-Kai
MK
Chen
I-Hung
IH
Lin
Shih-Hua
SH
Chu
Pauling
P
Wu
Chia-Chao
CC
D000284:Administration, Oral; D000368:Aged; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007674:Kidney Diseases / Q000209:etiology / Q000628:therapy*; D008171:Lung Diseases / Q000000981:diagnostic imaging / Q000209:etiology / Q000628:therapy*; D008297:Male; D055953:Microscopic Polyangiitis / Q000097:blood / Q000150:complications / Q000628:therapy*; D009173:Mycophenolic Acid / Q000031:analogs & derivatives / Q000627:therapeutic use; D010956:Plasmapheresis; D011239:Prednisolone / Q000627:therapeutic use; D020551:Pulse Therapy, Drug; D013902:Radiography, Thoracic; D013577:Syndrome; D016896:Treatment Outcome
D000894:Anti-Inflammatory Agents, Non-Steroidal; D019268:Antibodies, Antineutrophil Cytoplasmic; D005938:Glucocorticoids; D011239:Prednisolone; D009173:Mycophenolic Acid