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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.