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A case of amyopathic dermatomyositis with acute interstitial pneumonia (DAD pattern)

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A case of amyopathic dermatomyositis with acute interstitial pneumonia (DAD pattern)

Kenzo KAJIÞ1, Atsuyuki IGARASHIÞ1, Yasuhito HAMAGUCHIÞ2, Shinichi SATOÞ2

Þ1Department of Dermatology, Kanto Medical Center NTT EC

Þ2Department of Dermatology, Kanazawa University Graduate School of Medical Science (Received March 22, 2004)

summary

A 61­year­old man was admitted to our hospital because of edematous erythema on his upper eyelids and dry cough. No subjective nor objective ˆndings suggestive of skeletal muscle involvement, such as muscle weakness and elevated levels of aldolase and creatine phosphokinase were noted. Chest high­resolution computed tomography rev­

ealed a ground glass opacity and consolidation of his lower lung. Skin biopsy ˆndings were compatible with der­

matomyositis. Therefore, he was diagnosed as amyopathic dermatomyositis(ADM)with acute interstitial pneumonia and treatment with steroid pulse therapy was started. Since histological evaluation showed diŠuse alveolar damage dur­

ing the initial treatment, the treatment was changed into the combination therapy of prednisolone and cyclosporine.

However, his acute interstitial pneumonia did not respond to this treatment and passed away by aggravation of a breathing state and concurrence of disseminated intravascular coagulation. Japanese patients with ADM have been shown to be more frequently associated with intractable acute interstitial pneumonia than Caucasian patients, suggest­

ing that the racial diŠerence in‰uences the occurrence of acute interstitial pneumonia in ADM. Since autoantibodies speciˆc for ADM have not been detected, we performed immunoprecipitcation analysis using35S methionine­labeled K562 cells to identify them. His sera immunoprecipated a polypeptide of 140 kDa. The 140 kDa polypeptide might be one of autoantibodies speciˆc for ADM with acute interstitial pneumonia, although future analysis using a larger num­

ber of patients with ADM will be required to conˆrm this result.

Key words\amyopathic dermatomyositis; interstitial pneumonia; 140 kDa polypeptide; immunoprecipitcation

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RBC 439œ/ul

Hb 13.0 g/dl

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Plts 31.1œ/ul

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TP 7.6 g/dl

Alb 3.9 g/dl

ZTT 4.7 U

TTT 1.3 U

GOT 22 IU/l

GPT 16 IU/l

LDH 387 IU/l

g_GTP 31 IU/l

ChE 207 IU/l

CPK 96 IU/l

ALD 2.8 U/l

BUN 18 mg/dl

Cre 1.1 mg/dl

Na 4.4 mmol/l

K 4.4 mmol/l

Cl 99 mmol/l

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1) Pearson CM : Polymyositis and dermatomyosi­

tis, In McCarty DJ(ed). Arthritis, 9th ed. Lea and Febiger, 1979.

2) |´aFCK¼³²C{nÇ÷CÙ©FJ‰[

ÅÝéVEP´aCffÆ¡ÃÌÅV|C“

gDffÆ¡ÃÐCpp194_201, 2002.

3) ã½»êÙ©F²•ÉÄ°˜»µ­Çã1–Ž

ʼnbµ½}«Ô¿«xŠ‡¹Ìamyopathic dermatomyositis ÌêáDàÈêåãï24

(5)

\2 P´aɺ¤Ô¿«xŠÌagDªÞÆÕ°œE©ÈRÌ

UIP BOOP DAD LIP NSIP

aŠ© xEÇÌ×EZ

CüÛ«ìúC I|x

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UIP, BOOP, DAD Ì¢¸êÉàªÞ

³êÈ¢

aúª Ù ÚÏê

P ´ a DM/PM, SSc,

RA, SLE, MCTD

DM/PM, RA, SLE, SjS

DM, RA, SLE SjS DM/PM, RA,

MCTD

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×E”ª

Š“p…“¨

D†…“¨`ª CD4/CD8sè (UIP_pattern)

×E”ª

Š“p…“ªª D†…“¨`ª CD4/CD8(ƒ1.0) (BOOP_pattern)

×E”ª

Š“p…“ªª D†…“¨`ª CD4/CD8(ƒ1.0) (BOOP_pattern) ÖA·é©ÈRÌ RARSRÌCR

g|C\‰[[

IRÌ

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ž«

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ÇD sÇCžÉ¶¶ è ärIÇDCÜê É««Š“pîÖ ÌÚs

ÇDCÜêÉsÇ

UIP: usual interstitial pneumonia, BOOP: bronchiolitis obliterans organizing pneumonia, DAD: diŠuse alveolar damage, LIP: lymphoid interstitial pneumo­

nia, NSIP: nonspeciˆc interstitial pneumonia, ARS: aminoacyl tRNA synthetase

(1): 35_38, 2002.

4) _ì`VÙ©F}¬is«ÌÔ¿«xŠð‡¹ µ€Sµ ½ Amyopathic dermatomyositisÌ 1 áDç†Õ°C38: 2019_2022, 1996.

5) ¡cOÙ©Fv€IÈÔ¿«xŠð‡¹µ½ Amyopathic dermatomyositisÌ 1 áDç†Õ

°C37: 463_466, 1995.

6) Rokea A.el­Azhary and Soheil Y.Pakzad : Amyopathic dermatomyositis: Retrospective

review of 37 cases.J AM ACAD DERMATOL, 46(4): 560_565, 2002.

7) OXo¢FP´aɨ¯é©ÈR̤†Ìi àDú{Õ°ÆuwïïC25(1): 23_27, 2002.

8) Shinji Sato : clinical and Immunological Fea­

tures of Autoantibodies to 140 kDa polypeptide in Patients with Amyopathic dermatomyositis, Arthritis & Rheumatism46(9), S398, 2002.

参照

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