Wegener's granulomatosis H1960
1. Á¤ÀÇ: upper & lower respiratory tractÀÇ granulomatous vasculitis + GN
±× ¿Ü, small arteries & veinsÀÇ disseminated vasculitis
2. ºóµµ: ³²³àµ¿ÀÏ, ¾î´À ³ªÀÌ¿¡µµ »ý±æ¼ö ÀÖÀ¸³ª û¼Ò³â ÀÌÀü¿¡´Â µå¹°´Ù(Æò±Õ 40¼¼).
3. º´Å»ý¸® ¹× º´ÀÎ
1) histologic hallmarks
necrotizing vasculitis of small arteries & veins + granuloma formation
(intravascular or extravascular)
2) Fig317-2 lung biopsy: necrotizing vasculitis with granuloma formation
Fig317-3 chest CT: multiple, bilateral, nodular cavitary infiltrates
endobronchial disease : active form or fibrous scarring -> obstruction with atelectasis
¾ß±â
i) upper airway lesion(ƯÈ÷, sinus, nasopharynx)
inflammation, necrosis, and granuloma formation c/s vasculitis
ii) renal involvement: focal & segmental glomerulitis -> RPGNÀ¸·Î ÁøÇà
granuloma´Â µå¹°´Ù.
iii) classic triads(upper & lower respiratory tracts + kidneys)¿Ü¿¡µµ
½ÇÁ¦ ¸ðµç Àå±â°¡ ħ¹ü°¡´ÉÇÏ´Ù.
immunopathogenesis´Â ºÒºÐ¸íÇÏÁö¸¸ aberrant hypersensitivity response°¡ ÃßÁ¤µÈ´Ù.
chronic nasal carriage of S. aureus°¡ higher replase rate¿Í °ü·ÃµÈ´Ù.
±×·¯³ª º´Àο¡ °ü¿©ÇÑ´Ù´Â Áõ°Å´Â ¾ø´Ù.
3) PB mononuclear cells¿¡¼ IFN-¥ã secretionÀÌ Áõ°¡µÈ´Ù.
IL-4, 5, 10ÀÇ Áõ°¡´Â ¾ø´Ù.
IL-10¿¡ ÀÌÇØ IFN-¥ã´Â ¾ïÁ¦µÈ´Ù.
±× ¿Ü TNF-¥á ¹× CD4+ T cell, IL-12°¡ Áõ°¡µÈ´Ù.
ÀÌ·¯ÇÑ °ÍµéÀº Th1 type T cell cytokine patternÀÌ´Ù.
<Á¤¸®> Áõ°¡: IL-12, IFN-¥ã, TNF-¥á, CD4+ T cell
°¨¼Ò: IL-4, 5, 10
±× ¿Ü, c-ANCA¡è: ANCA°¡ º´Àο¡ 1Â÷Àû ¿ªÇÒÀ» ÇÑ´Ù´Â Áõ°Å´Â ¾ø´Ù.
4. ÀÓ»ó ¹× °Ë»ç½Ç ¼Ò°ß(Tab 317-4)
severe upper respiratory tract: PNS pain & purulent or bloody discharge
c/s nasal mucosal ulceration
-> nasal septal perforation, saddle nose deformity
serous otitis media
pulmonary involvement: asymptomatic infiltratesºÎÅÍ cough, hemoptysis, dyspnea,
chest discomfort(85-90%)
eye(52%), skin(46%), cardiac(8%), renal(77%)
* fever : underlying disease activity¸¦ ÀǹÌÇÏÁö¸¸ º¸´Ù ÈçÇϱâ´Â secondary infectionÀ»
ÀǹÌÇÑ´Ù(ÈçÈ÷ upper airway)
ESR¡è, mild anemia & leukocytosis, hypergammaglobulinemia(ƯÈ÷ IgA),
mild RA¡è, thrombocytosis
c-ANCA(90%): renal disease°¡ ¾ø´Ù¸é sensitivity´Â 70%·Î ¶³¾îÁø´Ù.
5. Áø´Ü
biopsy¿¡¼ necrotizing granulomatous vasculitis¸¦ Áõ¸íÇÑ´Ù.
i) lung : open thoracotomy = granulomatous vasculitis
ii) upper airway tissue : granulomatous inflammation with necrosis(vasculitis´Â ¾ø´Ù)
iii) renal biopsy: GN
c-ANCA´Â ƯÀ̵µ°¡ ¸Å¿ì ³ô´Ù. ƯÈ÷ acute GNÀÌ ÀÖ´Ù¸é ´õ¿í ±×·¸´Ù.
±×·¯³ª c-ANCA´Â Áø´Ü¿¡ º¸Á¶ÀûÀÌ¸ç ¸Å¿ì µå¹® ¿¹¸¦ Á¦¿ÜÇϰí´Â tissue diagnosis¸¦
´ë½ÅÇØ¼´Â ¾ÈµÈ´Ù.
6. Ä¡·á
°ú°Å¿£ ¼ö°³¿ù³» »ç¸Á
¨ç glucocorticoid(1mg/kg/d) some symptomatic improvement
º´ÀÇ °æ°ú¿¡ ¿µÇâÀ» ¸ø¹ÌÄ£´Ù.
¨è cyclophosphamide: most effective treatment
2mg/kg/d + glucocorticoid
WBC count¸¦ º¸¸é¼ »ç¿ëÇϴµ¥ 3000/uLÀ̻󿡼 ½ÃÀÛÇÏ¿© 1500/uL¸¦ À¯ÁöÇÑ´Ù.
CRÈÄ 1³â´õ »ç¿ëÇÏ°í ±×ÈÄ taperingÇÑ´Ù.
µÎ°¡Áö ¾àÁ¦ÀÇ »ç¿ëÀ¸·Î ¿¹ÈÄ´Â excellent
90%À̻󿡼 marked improvement
75%¿¡¼ CR
CRÈÄ 50%¿¡¼ Àç¹ßÇϳª reinduction¿¡ ¹ÝÀÀÇÑ´Ù.