Systemic sclerosis associated interstitial lung disease - individualized immunosuppressive therapy and course of lung function: results of the EUSTAR group

Adler, Sabine; Huscher, Dörte; Siegert, Elise; Allanore, Yannick; Czirják, László; DelGaldo, Francesco; Denton, Christopher P.; Distler, Oliver; Frerix, Marc; Matucci-Cerinic, Marco; Mueller-Ladner, Ulf; Tarner, Ingo-Helmut; Valentini, Gabriele; Walker, Ulrich A.; Villiger, Peter M.; Riemekasten, Gabriela

doi:10.1186/s13075-018-1517-z

Table 2 Characteristics of patients with dSSc or lSSc at the start of specific therapy

From: Systemic sclerosis associated interstitial lung disease - individualized immunosuppressive therapy and course of lung function: results of the EUSTAR group

	Never used IS		AZA		CYC		MMF		MTX		GC		AZA + GC		CYC + GC		MMF + GC		MTX + GC
	n	%	n	%	n	%	n	%	n	%	n	%	n	%	n	%	n	%	n	%
Sex
male	103	14.4	14	10.6	18	24.0	22	20.4	18	17.8	108	15.2	42	18.5	35	21.0	47	24.2	29	14.9
female	612	85.6	118	89.4	57	76.0	86	79.6	83	82.2	604	84.8	185	81.5	132	79.0	147	75.8	165	85.1
total	715	100.0	132	100.0	75	100.0	108	100.0	101	100.0	712	100.0	227	100.0	167	100.0	194	100.0	194	100.0
Extent of skin involvement
diffuse	278	38.9	74	56.1	46	61.3	79	73.1	71	70.3	389	54.6	132	58.1	96	57.5	137	70.6	119	61.3
limited	437	61.1	58	43.9	29	38.7	29	26.9	30	29.7	323	45.4	95	41.9	71	42.5	57	29.4	75	38.7
total	715	100.0	132	100.0	75	100.0	108	100.0	101	100.0	712	100.0	227	100.0	167	100.0	194	100.0	194	100.0
NYHA
I	200	53.2	63	50.0	21	30.9	43	45.3	47	52.2	260	39.4	80	37.6	45	27.8	64	36.4	82	46.6
II	128	34.0	50	39.7	26	38.2	34	35.8	34	37.8	254	38.5	82	38.5	69	42.6	66	37.5	78	44.3
III	40	10.6	10	7.9	19	27.9	17	17.9	9	10.0	118	17.9	39	18.3	42	25.9	38	21.6	15	8.5
IV	8	2.1	3	2.4	2	2.9	1	1.1	0	.0	28	4.2	12	5.6	6	3.7	8	4.5	1	.6
total	376	100.0	126	100.0	68	100.0	95	100.0	90	100.0	660	100.0	213	100.0	162	100.0	176	100.0	176	100.0
	of N	%	of N	%	of N	%	of N	%	of N	%	of N	%	of N	%	of N	%	of N	%	of n	%
ACA+	662	36.7	101	12.9	56	12.5	89	11.2	81	9.9	614	18.9	191	9.4	153	5.9	149	9.4	165	17.0
SCL70+	665	38.2	106	64.2	59	62.7	92	56.5	85	64.7	617	53.3	197	57.4	155	64.5	157	64.3	167	59.3
Pulmonary hypertension	657	22.5	111	13.5	55	34.5	93	23.7	83	14.5	588	23.1	195	25.1	151	29.1	156	28.8	166	16.3
Ground glass opacification	315	24.4	67	53.7	51	54.9	68	61.8	49	42.9	435	36.3	138	42.0	121	59.5	117	48.7	105	38.1
PFT restrictive defect	666	36.6	102	48.0	56	62.5	78	60.3	82	37.8	574	42.9	191	50.8	151	59.6	144	61.8	161	41.0
	mean	std	mean	std	Mean	std	mean	std	mean	std	mean	std	mean	std	mean	std	mean	std	mean	std
Age (years)	58.6	14.0	56.5	11.5	55.7	12.4	55.4	12.4	54.8	12.0	58.9	12.7	54.1	12.3	53.9	13.1	53.7	12.7	56.9	12.9
Body mass index	24.4	5.0	25.3	4.9	25.3	4.1	25.1	5.1	25.0	5.0	25.0	4.7	25.3	4.8	24.5	3.5	26.2	5.1	24.7	5.4
Disease duration (years)	10.4	8.5	10.4	7.3	9.4	6.6	10.6	7.3	8.2	4.8	11.9	7.6	10.1	6.8	8.6	6.2	9.8	7.0	10.0	7.4
mRSS	9.6	7.6	6.8	5.7	11.6	7.4	9.9	8.2	10.4	8.5	8.9	8.0	8.2	7.1	10.2	8.1	10.3	8.4	11.0	7.9
DLCO (% pred.)	68.0	19.7	59.8	17.2	46.3	18.8	55.7	16.7	64.1	17.8	57.3	19.8	55.5	19.6	49.6	18.2	50.7	16.4	60.0	18.2
FVC (% pred.)	95.7	20.6	86.6	18.9	73.6	20.4	81.8	20.9	89.9	19.6	85.0	22.6	79.9	19.4	77.3	20.3	77.0	22.1	85.8	20.2
FVC:DLCO ratio	1.5	.5	1.6	.5	1.8	.6	1.7	.7	1.4	.4	1.6	.5	1.6	.5	1.7	.6	1.7	.6	1.5	.5
FEV-1 (% pred.)	93.5	18.8	84.0	17.1	77.5	21.4	80.5	18.6	91.2	17.7	84.9	21.8	81.4	18.4	79.9	21.9	76.8	22.5	85.3	18.8
TLC (% pred.)	89.1	20.5	81.2	18.8	70.0	16.5	75.9	18.1	92.5	18.5	80.0	19.3	75.4	21.1	72.5	16.7	74.0	17.8	84.9	20.1

Only therapy regimens with frequencies of at least 5% are displayed (for patients on immunosuppressive therapy, only therapy episodes started during follow up were included to exclude possibly long-lasting therapy episodes documented at baseline). dsSSc diffuse cutaneous systemic sclerosis, lSSc limited systemic sclerosis, IS immunosuppression, AZA azathioprine, CYC cyclophosphamide, MMF mycophenolate mofetil, MTX methotrexate, GC glucocorticoid, NYHA New York Heart Association, ACA anti-centromere antibodies, SCL70 anti-topoisomerase I antibody, PFT pulmonary function test, mRSS modified Rodnan skin score, DLCO diffusing capacity of the lung for carbon monoxide, FVC forced vital capacity, FEV-1 forced expiratory volume in one second, TLC total lung capacity, pred. predicted, N number of patients within this specific group of medication

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