TAP CHi NGHIEN CU'U Y HOC •
DAC DIEM LAM SANG VA CAN LAM SANG BENH Md LIEN K^T HON HOP
Nguydn Thj Mai Hu'O'ng, N g u y i n Van Do^n Truong Dai hoc Y Ha Ndi Nghien cuv nham md ta dac diem lam sang va can lam sang b$nh mo lien ket hon hop. Nghidn ciru mo ta cat ngang tren 45 benh nhan dwgc chan doan lin dau b$nh md lien kit hdn hcyp tif thang 7/2010 dh thang 7/2012 tai trung tam Di img • Miin dich lam sang benh vi$n Bach Mai theo bd tieu chuan chan dohn ciia Alarcon • Segovia (1987) Kit qua nghien ciru cho ihiy ton thuong lam sang da d^ng, thuong g^p nhibt la dau khdp (100%), hdi chimg Raynaud (93,3%); dau ca (86,7%,), mit nep nhan da (82,2%,); kho thotOc nguc (88,9%o) Ton thuong co tren di^n co chiim 71,1%: ty le khang the ANA (khang the khang nhin- Antinuclear antibodies) duong tinh la 97,8%,; Anti - RNP70 (Anti Ribonucleotidprotein 70) dwang tinh IA 100%; ton thuong phoi ke va PHA (tang ap li/c ddng m^ch phoi - Pulmonary Hypertension Artery) g^p d nhiiu benh nhan. MCTD (B$nh md lien kit hdn hap - Mixed Connective Tissue Disease) cd tneu chimg chong cheo cua cac benh md lien kit khac v&i ty le khang thi khang nhan, khang the khang RNP - 70 duong tinh cao Tyle ton thuong phoi ke la 64,5% va PHA chiim 89,3% so benh nhan duoc sieu am tim.
TCF khoa; benh md lien k i t hdn ho-p, khang the khang RNP70, PHA
I. DAT VAN DE
Benh md lien k i t hdn hop la mdt bdnh ta m i l n dieh, d a o c md.ta lan d i u tien bdi Sharp vao nam 1972 Benh gay ton t h a o n g tai n h i l u eo quan vdi bieu hien iam sang chdng cheo eao dae diem fam sang cua bdnh Lupu= ban do he thdng (Systemic lupus erythematosus - SLE), xo cifng bi he thdng (Systemic sclerosis - SSc), viem da c a (Polymyositis - PM) Benh md lien k i t hdn hop cOng nha cac b?nh md hen k i t khac edn n h i l u d i l u ehaa biet v l co c h i smh bdnh hpc [1]
Tuy nhien, vdi s a phat tnen cua xet nghiem m i l n dich hoc, bdnti md lien k i t hdn hop daoc edng nhan la mdt benh cd dac tinh iam sang hdn hpp dac trang ciia benh md lien k i t , cd ta khang the khang U l - RNP ( U l -
Bia chi lien h€ Nguyen Thi tvlai Huong, Truong Dai hoc Y Ha Noi
Email. maihuong62@gmail com Ngay nhan: 02/8/2013 Ngdy duoc chip thuan 30/10/2013
Ribonucleotidprotein) va khang the khang nhan vdi hiSu gia eao [2], Bdnh md lien k^t hdn hap v i n cd dac t r a n g rieng, cac bieu hi$n iam sang khdng x u l t hien ciing mdt thdi diem ma nd thay doi theo thai gian, cung nha cac . xet nghiem m i l n dich khac eua ho khang the khang nhan nha khang the khang chudi kSp (Ds - DNA) cd the d a a n g tinh thoang qua trong mpt giai doan cua benh Bdnh md lifin k i t hon hop cd ton t h a o n g phoi chiem 75%, nd t h a d n g khdng cd tneu chifng trong giai doan d i u va tang ap ddng mach phoi IS nguyen nhan hang d i u gay t i i vong [3; 4]
Nhd sa phat trien ciia xdt nghiem miin djeh hpc ma chung ta mdi b i l t d i n bdnh m6 hen k i t hdn hap t i n tai la mdt benh d$c lap vdi cae dac tinh lam sang chdng eheo nhi^u benh md hen k i t nha SLE, SSc, PM va a$c tinh h u y l t thanh hoc cd khang the khang Ul - RNP d a o n g tinh vdi hieu gia cao. Cho d^n nay chaa cd cdng trinh nao nghien cifU v^
benh md lien k i t hdn hop tgi Vidt Nam Vi vSV chung tdi hen hanh d l tai vdi mue tieu:
TCNCYH 85 (5) - 2013
Md ta dac diem iam sang va can iam sang benh md li^n k i t hdn hap.
II. D 6 | TU'O'NG VA PHU'aNG PHAP
1. Ddi tu'crng
Nghien cifu dape t i l n hanh tren 45 benh nhan daoc chan doan l l n d i u benh md lien k i t hdn hap tif thang 7/ 010 - 7/2012 tai trung tam Di dng - M i l n dich iam sang, bdnh vien Bgch Mai theo bd tieu chuan chan doan cua Aiareon ~ Segovia (1987)
1.1. Huyet thanh hgc
Hieu gia khang the khang RNP cao (> 1:1600)
1.2. Lam sang
• PhLJ cac ngdn tay - Vidm mang hoat djeh
- Vidm eo (md benh hpe ho^c sin.h hoc).
- Hifen tapng Raynaud
- Xo cifng da viing ngon chi, cd hoac khdng kdm theo xa cifng toan the.
* Chan doan bdnh md hen k i t hdn hop khi cd ti6u chuan v l h u y l t thanh hoc va it n h i t 3 tifiu chuan iam sang, trong do au tien tieu chuan vifem eo hoac viem mang hoat djch,
Tradng hop 3 tieu chuan lam sang la sang ngdn tay, xo cdng d i u chi va hidn taong Raunaud can phai ed them tieu chuan khac de phan bi$t vdi bpnh xa cdng bi
Tieu chuan loai trif:
Bdnh nhan khdng hpp tac tham gia nghian cifu.
2. Phu'O'ng phap
Phaong phap nghidn cifu md ta e i t ngang.
Thu thap so lieu theo m l u banh dn nghien c d u
TAP CHI NGHIEN Ct>U Y HOC
Benh nhan d a p c danh gia tran iam sang va cac xdt nghiem co ban. Cdng thirc mau, smh hda mau, smh hda n a d c tieu, X-quang tim phoi t h i n g , sieu dm d bung, dien co.
Banh nhan ed ton thaong tim phoi daoc iam thdm cac xet nghiem dien tim, khi mau ddng maeh, tham dd chde nang thdng khi phdi, xet nghiem m i l n dich b l n g phaong phap ELISA gian t i l p (Enzyme Linked Immunosorbent Assay)
3. X a ly s d lieu: eae sd lipu daac xd ly b l n g toan thdng ke y hoe, s d dung phan mem SPSS 16.0
4. Dao du'c nghien c i r u
Nghien cdu da d a p c thdng qua hpi ddng dao difc, benh nhan c h i p thuan tham gia nghien cicu, la nghien eifu md ta nen khdng anh hadng tdi tien trinh d i l u tri eua benh nhan
III. K^T QUA
Dac diem lam sang va can lam sang benh md hen k i t hdn hop
1. Dac diem chung cua benh nhan TUOI trung binh ciia benh nhan la 37,42 ± 12.82 vdi ty le nam/ nd la 1/10.
2. Cac b i l u hien lam sang du^oc md ta trong bang 1
Bieu hien iam sang da dang vdi cac tneu chifng ciia cac bdnh md hen k i t khac nha SLE, SSc, PM Ty le benh nhan gap dau khdp la nhilu nhit vdi 100% benh nhan, hdi ehdng Raynaud (93,3%), dau ea (86,7%), m i t n i p nhan da (82.2%), kho thd tdc ngac (88,9%) la cac trieu chifng thadng xuyen gap n h i t (bang 1)
TAP CHI NGHIEN CLFU Y HOC •
Bang 1. Cac dau hieu lam sang dac tru'ng
STT 1 2 3 4 5 6 ' 7 8 9 10 11 12 13 14 15 16 17 18 19 20
Ddu hieu lam sang Sot
Dau kh6p Cling khffp Bidn dang khap HOI chLHng Raynaud Su'ng phu ban tay Ban do M ^ t n 6 p ntian Rung toe N6t san Gottron Teo da, loet da Loet niSm mac Dau CO, yeu c a goe ctii D§u hieu ghS dau Giam nhu ddng thuc quan Khd tho, tLFC nguc Giam thong khi phoi Viem mang phoi mang tim Tneu Chung th^n kmh Cac trieu chung khac
n 19 45 2 3 . 16 42 14 17 37 35 4 20 16 39 2 4 ' 15 40 27 25 12 24
%
42,2 100 51,1 35,6 93,3 31.1 37,8 82,2 77,8 8,9 44,4 35,6 86,7 63,3 33,3 88,9 60,0 55,6 26,7 53,3
•
3. Cac chi sd can lam sang
c a e gia th trung binh c^c chi sd h u y l t hpc (hdng c l u , hemoglobin, h i u c l u ) cd giam han so vdi gia tn ly thuylt cd y nghTa vdi p < 0,001, Cac chi s6 men co, men gan (OK, GOT) tang hon so vdi gia trj binh thadng c6 y nghTa t h i n g ke vdi p < 0,05. Cac ehi s6 hdng c l u , bach c l u , protein nadc t i l u tang han so vdi gia tri binh thadng cd y nghTa thdng ke vdi p < 0.001 Ty le benh nhSn cd ton thaong ca t h i y tren dien co c h i l m 7 1 , 1 % . Ty le d a o n g tinh cao vdi khang the ANA (97,8%) va Anti - RNP70 (100%), mdt so benh nhan cd d a o n g hnh vdi khang the khae Ds - DNA (42,2%), Sei - 70 (8,9%)
•48 TCNCYH 85 (5) - 2013
• TJ5P CHl NGHIEN C l f U Y HOC 4. T6n thircng phAi t r i n Xquang
Bang 2. Ty 16 t6n thircng phoi tren Xquang
Xquang tim ph6i thSng CT nguc
Tong so
45 31
Viem n.
24 20
phoi ke
%
53,3 64,6
Tran djch
"2 12 9
mang phoi
%
26,7 29,0
Trdn Xquang tim phoi t h i n g va CT scanner ngac ton thaong phoi ke c h i l m ty Id cao 53,3% va 4,5%, ton thaang tran dich mang phoi c h i l m ty le t h i p hon la 26,7% va 29,0%
5. Tdn thu'O'ng tim tren sieu am doppler tim
Bang 3. Ty le tdn thu'O'ng tim tren sieu am doppler tim
_.j .: Tang ap Itfc „ . ,. - .. Tang du'dng kinh ^^ . , Tong so , , \...'-. D'ch mang tim ^ ^ j . J:. EF giam
dpng mach phoi ^ that phai ^ n % n % n % n %
Ty le tang ap lac dpng maeh phoi la 89,3%, tran dich mang tim 53,3%, tang dadng kinh t h i t phai la 32,1% so bpnh nhan, phan sd tdng mau EF giam gap 21,4% tradng hap
Bang 4. Mdi Men quan giO'a du'd'ng kinh that phai
Du'dng kinh Tang ap lac ddng mach phdi P that phai Binh thu'ang Nhe VO-a Nang
Binh thadng 3(15,8%) 9 (47,4%) 7 (36,8%) 0 ( 0 % ) < 0,05 Tang 0 ( 0 % ) 1(11,1%) 3(33,3%) 5(55,6%)
Tong 3 10 10 5 28 Cd sa hdn quan ciia tang ap lac ddng mach phoi den tang dadng kinh t h i t phat cua benh nhan vdi p < 0,05
6. Chi sd tham dd chu-c nang thdng bjnh thadng. Chi sd FEV1 (the tich thd ra t6i khi phoi da trong giay d i u tien - Forced expiratory
Chi s6 lien quan FVC (Dung tich sdng thd volume in first second) gap d mifc dp giam mgnh - Forced vital capacity) gap d mdc dd nhieu la 2 6 , 1 % , giam nhe la 52,2% chi sd d giSm nhe la 47,8% va giam nhieu la 52,2% , mifc binh thadng la 21,7%. Chi sd FEVl/FVC khdng cd tradng hop nao cd ehi so a gidi hgn d mCrc binh thadng c h i l m 91,3%
TAP CHi NGHIEN CLPU Y HOC •
Ty le chi s o t h d n g k h i phoi
= ^
D > 8 0 % D 60 - 80'!
• < 6 0 %
Hinh 1. Ty le cac chi s d t h o n g k h i p h o i
Nghien edu eho t h i y cac ehi sd p C 0 2 (Phan dp rieng p h l n C 0 2 trong mau ddng maeh - Partial pressure of cacbon dioxide in artenal blood), H C 0 3 - tang hon so vdi gia tn binh thadng ed y nghTa vdi p < 0,01. Chi sd p 0 2 (Phan ap rieng p h l n 0 2 trong mdu ddng mach - Partial pressure of oxygen in arterial blood) giam so vdi gia tn binh t h a d n g cd ^i-nghTa vdi p < 0,001.
Ty le thay ddi cac chi sd khi mau dpng mach
26.9
11 5 | 19 2
D T a n g a Binh thii'ang D Gtam
pH pC02 p 0 2 HC03-
Hinh 2. Ty le cac chi s d khi mau d p n g m a c h
IV. BAN LUAN
' Nghien eifu cua chiing tdi, do tuoi thadng gap phii hap vdi cae benh nhan benh md hen ket khac, thdi ky smh de la thdi ky cac benh md hen k i t boat ddng manh n h i t Ty le gidi tinh nam' na la 1,10 hpan toan phu hop vdi mpt sd nghien c d u n a d c ngoai nha PJW Venables ty le nam, na la 1 9 [5]. c a c ton thaang tren lam sang va can lam sang cho
t h i y tdn t h a o n g cac c o quan da dang noi bSt len ia d i u hieu dau khdp 100%, hdi chirng Raynaud 93,3%, xo cdng m i t n i p nhan da 82,2% va dau c o 86,7%, ton thaong ca trfen dien c a la 7 1 , 1 % . Oac bidt cd x6t nghiem khang the khang nhan d a o n g tinh tdi 97.8%
va khang the khang RNP70 la 100% c6 y nghTa vd ciing quan trong trong c h i n doSn benh [ 1 ; 3]
TCNCYH 85 (5) - 2013
• TAP CHi NGHIEN C l K l Y HOC Ton thaong phoi chiem ty le cao, trdn iam
sang ty lp gap khd t h d tire ngac la 88,9%, giam thdng khi phoi qua danh gia lam sang la 60% va tren Xquang phdi thadng cd ty le tdn thaang phoi ke la 53,3%, ton thaong tran djeh mang phoi la 26,7%, trSn CT Scanner ngac cd ty le ton thaong phoi ke la 64,5%. Tuy nhien, day la nghien cdu t i n thaang chung, chiing tdi chaa cd d i l u kidn chyp CT scanner hang l09t de danh gia ca nhti'ng benh nhan khdng cd tdn thaong iam sang hay trdn Xquang thadng. Hau h i t cac benh nhan cd tdn thaong phoi ke d i u da cd giam the tich phoi, c6 lidn quan d i n hdi ehieng rdi loan thdng khi han c h i Dii do dieu kidn chung ta ehaa iam dapc xet nghiem TLC (dung tich khi toan phoi Total Lung Capacity) de danh gia chinh xac hpi chifng rdi loan thdng khi han c h i nhang k i t qua chiing tdi thu d a o c cung cd gia tn tien laang eho bdnh nhan. Tang pC02 chdng td cd giam thdng khi p h i nang, giam dao thai COz iam if dong C O j trong co the gay toan hd hap, hau qua cua ton thaong phoi man tinh [4, 6]
Trong nghien ciru ciJa chung tdi, tang ap li/c dOng mach p h i i c h i l m 89,3% trong so 28 b^nh nhan, k i t qua cd sa khae biet vdi cac nghian cdu ciia Josephine Swanton (2005) tang ap lac ddng mgch phoi tang ttf 9 - 23%
trong 5 nam, [7], gSp ty 1$ kha cao la d i l u d l hiiu bdi nhifng bdnh nhan d a o c laa chon d i u la nhdng benh nhan cd ton thaong tim phoi trdn iam sang va Xquang Cd lien quan chat che giLfa tang ap lyc dpng mach phoi va tang dadng kinh t h i t phai vdi p < 0,05. Sidu am doppler tim la mdt bidn phap khdng xam nhap cd dd nhgy va dd dac hi$u cao d l chan doan sdm tang ap lye dpng mach phoi d b^nh nhan b$nh md lifen k i t hdn hpp cd tSng ap lac ddng mgch phoi hoac nghi n g d tang ap lae ddng mgch p h i i [4]
V. K^T LUAN
Ton thaong tren iam sang va can iam sang da dang vdi trieu chdng lam sang chong cheo cua cac bdnh md lien k i t khac vdi cac bieu hien dac trang cua benh; hieu gia khang the khdng nhan va khang RNP - 70 daong tinh ty le cao dac trang cho benh.
Ton thaong phoi c h i l m ty le cao trong cac ton thaong ndi tang, cd s y hen quan giCfa tang ap lyc ddng maeh phoi va dadng kinh that phai Phat hidn sdm cac ton thaong phdi va tang ap lac ddng maeh phoi va tien hanh d i l u tn sdm cho benh nhan la vd ciing quan trong
Lo"! cam en
Chung tdi xin tran trong cam an cac bac sT, ky thuat vien va y ta tai Trung tam Di dng - M i l n dich iam sang benh vien Bach Mai da giup d d chiing toi trong qua trinh thae hien nghien eifu nay,
TAI LIEU THAM K H A O
1. Sharp GC, May CM, Holman HR et al (1976). Association of antibodies to nbonucleopretein and Sm antigens with mixed connective - tissue disease, systemic lupus Erythematosus and other rheumatic diseases.
New England Journal of f/ledicine, 295, 1 1 4 9 - 1 1 5 4
^. Sharp GC, Tan EM, Gould RG, et al.
(1972). Mixed connective tissue disease-an apparently distinct rheumatic disease syndrome associated vi/ith a specific antibody to an extractable nuclear antigen American Journal of Medicine, 52, 148 - 1 5 9
o. Gary SF, Edward DH, lain BM et al (2008). MIXED CONNECTIVE TISSUE DISEASE, Kelley's Textbook of Rheumatology, 12, 1 0 5 4 - 1 0 6 0
4. Haroon N, Chandran V, Bharadwaj A (2005}. Pulmonary hypertension not a major 51
TAP CHl NGHIEN CLPU Y HOC •
feature of early mixed connective tissue An overview of clinical manifestations, disease: A prospective clmicoserological diagnosis and treatment Best Practice &
study. Original Article, 2 1 , 104 - 1 0 7 . Research Clinical Rheumatology, 26, 61 - 72.
• Venables P (2006). Mixed connective 7. Josephine Swanton B. David Isenberg tissue disease /upus, 15, 1 3 2 - 1 3 7 . «* ^ ' <2005). Mixed Connective Tissue
Disease: Sbll Crazy After All These Years, 6. Oscar - Danilo Ortega-Hernandez YS pi^^^^^^c Disease Clinical North America.
et al (2012). Mixed connective tissue disease « . .^^ . 436
Summary
CLINICAL AND LABORATORY CHARACTERISTICS OF MIXED CONNECTIVE TISSUE DISEASE
Our objective was to study the clinical and laboratory charaeterishes pf mixed connective tis- sue disease (MCTD). it was a cross-sectional descriptive study There were 45 patients who were diagnosed as having mixed connective tissue disease from 7/2010 - 7/2012 at the Allergy and Clinical Immunology Center in Baehmai Hospital, MCTD was diagnosised by using Alarcon- Segovia (1987) criteria Arthralgia was the most obvious symptom that was seen, 100% patients, following by Raynaud syndrome, 93.3% The most common symptoms were muscle pain (86 7%), loss of skin wrinkles (82.2%), difficulty breathing and chest tightness (88, 9%) and muscle dam- age, 71 1 % In our study, the high concentration of autoimmune antibodies was seen in most pa- tients. ANA (Antinuclear antibodies) was 97 8%: anti-RNP70 (Anti Ribonucleotidprotein 70) was 100% Interstitial pneumonia and pulmonary artery pressure was seen in many patients In conclusion, MCTD had overlapping clinical lesions of other autoimmune diseases, had high titer ANA, had high titer anti - RNP70 There are 64 5%i patients with interstitial lung injury and PHA 89 3% of patients received an echocardiogram.
Key word: mixed Connective Tissue Disease, Anti U l RNP-70, PHA
TCNCYH 85 (5) - 2013
