β
Table1 Laboratoryfindingsonthefirstvisit Hematology WBC Neut. Lyn. Mon. Eos. Bas. RBC Hb Ht Plate 5,650 /μl 79.2 % 14.9 % 3.9 % 1.8 % 0.2 % 418 10 4 /μl 12.5g/dl 38.4 % 21.2 10 4 /μl Serology CRP IgG IgA IgM IgE ANA MPO-ANCA PR3-ANCA sil-2r 2.29mg/dl 1,865mg/dl 444mg/dl 127mg/dl 630mg/dl 10.9 <1.3EU < 1.3EU 783U/ml Bloodchemistry TP Alb UA BUN Cr T-cho FBS T-Bil AST ALT γ-gtp LDH CK ALP Na K Cl Ca ACE CEA ProGRP SCC 7.4g/dl 3.7g/dl 8.6mg/dl 19.8mg/dl 1.2mg/dl 170mg/dl 92mg/dl 0.6mg/dl 16IU/l 10IU/l 25IU/l 181IU/l 95IU/l 252IU/l 142mEq/l 3.9mEq/l 103mEq/l 9.2mEq/l 20.7IU/l 2.3ng/ml 28.9pg/ml 1.7n/ml coldaggulutination Mycoplasmap.ab Chlamydiap.ab β-d glucan Urinalysis PH gravity PH protein glucose keton occultblood Bloodgasanalysis PH PaCO2 PaO2 HCO3 B.E Respiratoryfunction %VC FEV1.0% RV/TLC %DLCO < 32 < 40 4.8pg/ml 6.0 1.014 5.0 7.426 36.8torr 113.9torr 23.7mmol/l -0.3 105.7 % 91.0 % 28.4 % 73.3 % PPD 8 8 mm 15 15 ECG RBBB
Fig.1 SeriesofchestX-raysbeforehisvisit. BilateralhilarlymphadenopachywaspersistentonalX-rays.Reticularandsmalnodularshadowsonboth lungfieldsweregradualyincreasing. Fig.2 ChestX-rayonhisfirstvisitdemonstratedan infiltrationshadow withacavityontherightupper lobe.difusereticularnodularshadowsandbilateral hilarlymphadenopathywerealsoseen. Fig.3 (A)Chestcomputedtomographyshowedacavitylesionwithathickwalandinfiltration.(B)A low density areawasseen in thecentralareaofthe cavity.mediastinallymphnodeswereswolen.
Fig.4 (A)Galium scintigraphyshowedabnormalaccumulationintherightupperlungfieldandweakaccumulationinbothlungs.(b)histopathologyofthetransbronchiallungbiopsyspecimenobtainedfrom thecavitywalintherights 2 demonstratedanoncaseatingepitheloidcelgranuloma. Fig.5 ChestX-raysaftercorticotherapy.(A)Rightupperlobeshadow wasafterone monthoftherapy.(b)afterthreemonthsoftherapy,theshadow anddifuseshadowsin bothlungswerefurtherdecreased,althoughthehilarlymphnodeswelingremained.
Fig.6 (A)Chestcomputed tomography afterthree monthsoftherapyshowedthecavitylesion withathinwal.(b)thesamelesionwassmalerafterafurther3months.
Table2 CasesofprimarypulmonarycavitarysarcoidosisreportedinJapan(1975-2005) # Year Author Age/Sex Location Periodfrom onset Extrapulmonarylesion Therapy Prognosis 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 1975 1977 1983 1984 1984 1988 1988 1988 1989 1991 1992 1992 1994 1995 1995 1995 1996 1997 1997 1998 1999 1999 2000 2000 2000 2003 2003 2004 2004 Mikami Terai Kondou Okada Akiyama1 Akiyama2 Doi Yamaguchi Shibata Ishihara Shimomoto Zaizenn Yoshi Takeyabu Yamaguchi Iwanaga Katayama Akuzawa Nakayama Hamada Iida Ueda Nagata Yoshida Asai1 Asai2 Asai3 Ohmichi Inomata Yoshioka Misawa Hosono 21/M 30/M 20/M 26/M 25/M 38/M 34/M 22/M 26/M 28/M 20/M 32/F 36/M 27/M 22/F 21/F 20/M 26/M 44/M 30/F 37/F 27/F unilateral(s2,s6) bilateralupperlobes muliple 3inrightlowerlobeand1in left rights4,s8 multiple upperlobes rights1,lefts1+2,s10 rights8,lefts1+2 rights1,s2,s3lefts1+2 rights2 rights1,lefts1+2 rights3 rights1,s2,lefts8 rights1,lefts1+2 lefts3,s8 rights2,lefts4 rights9 rights2,lefts1+2 rights2 within6months within1year (medexa) 2months lachrymal& parotid glands 12years 14months no 13months within1year 7months within6months no 8years 13months 5months 8months 5months 8years no skin,skinandbrain 14years 6months 7month skin(lupuspernio) andskin pulstherapy inhaled (medexa:medicalexamination)