Korean J Med.  2014 Jan;86(1):84-88. 10.3904/kjm.2014.86.1.84.

A Case of Different Organ Responses to Immunosuppressive Therapy In a Microscopic Polyangiitis Patient with Interstitial Lung Disease

• Lee JJ
• Shin JH
• Bang KT
• Choi JW
• Moon HJ
• Jeon JW
• Jeong JU

• KMID: 1867323
• DOI: http://doi.org/10.3904/kjm.2014.86.1.84

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.

Keyword

Microscopic polyangiitis; Interstitial lung disease; Anti-neutrophil cytoplasmic antibody-associated vasculitis

MeSH Terms

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
Adrenal Cortex Hormones
Autoantibodies
Immunosuppressive Agents