Analysis of clinical features of mixed connective tissue disease associated with pulmonary arterial hypertension

doi:10.11958/20231786

Abstract

Abstract:

Objective To investigate the clinical characteristics and risk factors of mixed connective tissue disease associated with pulmonary arterial hypertension (MCTD-PAH). Methods Twelve MCTD-PAH patients diagnosed by right heart catheterization (RHC) at Tianjin Medical University General Hospital were retrospectively included, and 36 MCTD patients without pulmonary arterial hypertension (MCTD-non-PAH) were randomly selected from the same period of hospitalization based on gender and age. The clinical features and auxiliary examination of the two groups were compared, and the survival status of the two groups was compared. Results The proportion of dyspnea after activity, myositis and pericardial effusion were higher in the MCTD-PAH group than those of the control group. Serum sedimentation rate and immunoglobulin G (IgG) levels were higher in the MCTD-PAH group. Multivariate Logistic regression analysis showed that dyspnea after activity and high level of IgG were risk factors for predicting the occurrence of PAH in MCTD. Three patients (16.7%) died in the MCTD-PAH group, and no patients died in the control group. Conclusion Pulmonary arterial hypertension is one of the serious complications of MCTD. MCTD patients have shortness of breath after activity and high level of IgG should be wary of concomitant PAH.

Key words: mixed connective tissue disease, pulmonary arterial hypertension, right heart catheterization, clinical features

CLC Number:

R544.16，R593.27

Figures/Tables 2

References 13

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组别	n		年龄/岁			女性			发热		手肿胀或手指硬化		关节炎			肌炎		皮疹		活动后气短			乏力		胸痛
对照组	36		45.69±12.04			33（91.7）			7（19.4）		10（27.8）		28（77.8）			6（16.7）		4（11.1）		10（27.8）			20（55.6）		1（2.8）
MCTD-PAH组	12		42.83±11.24			11（91.7）			4（33.3）		2（16.7）		10（83.3）			7（58.3）		1（8.3）		10（83.3）			6（50.0）		2（16.7）
t、Z或χ²			0.724			0.000			0.354		0.148		0.000			5.943^＊		0.000		11.429^＊＊			0.112		0.150^▲
组别		心悸			咳嗽			抗SSA抗体阳性				抗Ro52抗体阳性			抗心磷脂抗体阳性		类风湿因子阳性				IgG/（g/L）			CRP/（g/L）
对照组		2（5.6）			13（36.1）			4（11.1）				13（36.1）			1（2.8）		12（33.3）				20.96±7.90			0.51（0.17，1.29）
MCTD-PAH组		2（16.7）			1（8.3）			2（16.7）				5（41.7）			0		5（41.7）				30.67±9.01			0.29（0.18，0.42）
t、Z或χ²		0.364			2.151			0.000				0.000			1.000^▲		0.030				3.562^＊＊			1.404
组别				血沉/（mm/1 h）			心包积液			RA横径/mm				RV横径/mm					PASP/mmHg			射血分数/%
对照组				32.17±13.17			1（2.8）			34.0（33.0，35.0）				29.0（28.0，30.0）					25.0（21.3，27.8）			64.21±1.89
MCTD-PAH组				45.89±14.33			5（41.7）			41.5（38.0，47.8）				36.0（33.3，38.5）					70.0（53.8，79.5）			64.08±3.96
t、Z或χ²				2.749^＊＊			9.143^＊＊			4.454^＊＊				4.570^＊＊					4.479^＊＊			0.103

组别	n		年龄/岁			女性			发热		手肿胀或手指硬化		关节炎			肌炎		皮疹		活动后气短			乏力		胸痛
对照组	36		45.69±12.04			33（91.7）			7（19.4）		10（27.8）		28（77.8）			6（16.7）		4（11.1）		10（27.8）			20（55.6）		1（2.8）
MCTD-PAH组	12		42.83±11.24			11（91.7）			4（33.3）		2（16.7）		10（83.3）			7（58.3）		1（8.3）		10（83.3）			6（50.0）		2（16.7）
t、Z或χ²			0.724			0.000			0.354		0.148		0.000			5.943^＊		0.000		11.429^＊＊			0.112		0.150^▲
组别		心悸			咳嗽			抗SSA抗体阳性				抗Ro52抗体阳性			抗心磷脂抗体阳性		类风湿因子阳性				IgG/（g/L）			CRP/（g/L）
对照组		2（5.6）			13（36.1）			4（11.1）				13（36.1）			1（2.8）		12（33.3）				20.96±7.90			0.51（0.17，1.29）
MCTD-PAH组		2（16.7）			1（8.3）			2（16.7）				5（41.7）			0		5（41.7）				30.67±9.01			0.29（0.18，0.42）
t、Z或χ²		0.364			2.151			0.000				0.000			1.000^▲		0.030				3.562^＊＊			1.404
组别				血沉/（mm/1 h）			心包积液			RA横径/mm				RV横径/mm					PASP/mmHg			射血分数/%
对照组				32.17±13.17			1（2.8）			34.0（33.0，35.0）				29.0（28.0，30.0）					25.0（21.3，27.8）			64.21±1.89
MCTD-PAH组				45.89±14.33			5（41.7）			41.5（38.0，47.8）				36.0（33.3，38.5）					70.0（53.8，79.5）			64.08±3.96
t、Z或χ²				2.749^＊＊			9.143^＊＊			4.454^＊＊				4.570^＊＊					4.479^＊＊			0.103

变量	β	SE	Wald χ²	P	OR（OR 95%CI）
肌炎	1.116	1.218	0.839	0.360	3.052（0.280~33.247）
活动后气短	3.577	1.404	6.488	0.011	35.754（2.281~560.530）
IgG	0.001	0.001	3.872	0.049	1.001（1.000~1.003）
心包积液	2.989	1.746	2.931	0.087	19.872（0.649~608.731）
常数项	-6.934	2.162	10.281	0.001	0.001

变量	β	SE	Wald χ²	P	OR（OR 95%CI）
肌炎	1.116	1.218	0.839	0.360	3.052（0.280~33.247）
活动后气短	3.577	1.404	6.488	0.011	35.754（2.281~560.530）
IgG	0.001	0.001	3.872	0.049	1.001（1.000~1.003）
心包积液	2.989	1.746	2.931	0.087	19.872（0.649~608.731）
常数项	-6.934	2.162	10.281	0.001	0.001

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