A Case of Different Organ Responses to Immunosuppressive Therapy In a Microscopic Polyangiitis Patient with Interstitial Lung Disease.
- Author:
Jae Joon LEE
1
;
Jong Ho SHIN
;
Ki Tae BANG
;
Ji Wook CHOI
;
Hyun Jin MOON
;
Jae Woong JEON
;
Jin Uk JEONG
Author Information
- Publication Type:Case Report
- Keywords: Microscopic polyangiitis; Interstitial lung disease; Anti-neutrophil cytoplasmic antibody-associated vasculitis
- MeSH: Adrenal Cortex Hormones; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis; Autoantibodies; Autoimmune Diseases; Biopsy; Cough; Cytoplasm; Dyspnea; Hematuria; Humans; Immunosuppressive Agents; Lung Diseases, Interstitial*; Microscopic Polyangiitis*; Middle Aged; Prognosis; Renal Insufficiency; Serologic Tests; Sputum; Systemic Vasculitis
- From:Korean Journal of Medicine 2014;86(1):84-88
- CountryRepublic of Korea
- Language:English
- Abstract: Microscopic polyangiitis (MPA) is an idiopathic autoimmune disease characterized by systemic vasculitis associated with antineutrophil cytoplasmic autoantibodies. Interstitial lung disease is a less recognized manifestation of MPA and has a poor prognosis. A 61-year-old man presented with persistent cough, sputum and dyspnea. Laboratory examination revealed microscopic hematuria and renal insufficiency. Perinuclear anti-neutrophil cytoplasmic autoantibodies were positive according to serological testing. Computed tomography scans showed bibasilar reticulation and honeycombing in a peripheral distribution. Therefore, renal biopsy was performed, and MPA was diagnosed. After treating with corticosteroids and immunosuppressive agents, the patient had a complete renal response but progressive interstitial lung disease. We report a case of MPA presenting with interstitial lung disease in which the patient experienced different responses in each organ.