Background/Purpose:

Methods:

Objectives: To describe the frequency and clinical characteristics of pulmonary hypertension (PH) in a cohort of Antisynthetase Syndrome (ASS) patients.

Methods: Patients from a single referral center diagnosed between 1994-2010 with a positive serologic test (immuno-blot) of antisynthetase antibodies, interstitial lung disease (ILD) and/or myositis were defined as ASS (N=90). All data were retrospectively collected from medical reports. A diameter of the Pulmonal artery >29 mm, measured by CT-scans, was defined as pathological. PH was suspected with an estimated pulmonary arterial pressure (PAP) of >45mmHg at transthoracal eccocardiography (TTE), and defined as a mean PAP >25 mmHg measured by right heart catheterization (RHC). The ratio between forced vital capacity and diffusion capacity of the lungs (FVC/DLCO) was considered pathological, indicating PH, at values > 1.6. All examinations were done within 3 months for each patient.

Fisher´s exact test was used to evaluate statistical significance (p<0.05)

Results: The cohort consisted of 75 anti-Jo1 positive, seven PL-7 positive and eight PL-12 positive patients with mean age at ASS diagnosis of 48 years (range 12-82 yrs) and median disease duration of seven years (range 0.25-34 yrs). Fourteen patients were referred to RHC due to clinical suspicion of PH. Ten out of 14 were diagnosed with PH, with a median PAP of 38 mmHg (range 26-55). Eight out of 10 patients had a pre-capillary type of PH, defined as pulmonal capillary wedge pressure (PCWP) < 15mmHg. Over all frequency of PH was 11 % (10/90). Twelve of 14 patients had available TTE-exams, all but one indicating PH with estimated PAP of >45mmHg. The Pulmonal artery was pathological enlarged in 9/10 patients with PH, median diameter 40 mm (range 33-45). Although not statistic significant (p<0.07), the result indicates a correlation between enlarged pulmonary artery and PH. Seven of nine patients with PH had pathological FVC/DLCO ratios. All 10 PH patients had manifest ILD at time of PH diagnosis. 2/10 patients with PH had co-existing rheumatic disease, both systemic sclerosis. The diagnosis of PH was made 36-336 months after ASS onset. Five of 10 patients with PH died during the observation-period.

     Clinical characteristics of ASS patients with performed RHC

PATIENT	SEX/AGE	ANTIBODY	SS-A	FVC/DLCO	DLCO	DIA A PULM	ECCO/ mmHg	RHC/ mmHg	PCWP/ mmHg	DEATH
PH
1	M 61	Jo-1	+	2,12	17	45	60	33	2	+
2	F 63	Jo-1		NA	NA	36	55	35	14
3	M 44	Jo-1	+	1,52	38	42	40	26	9
4	F 43	Jo-1	+	1,91	45	42	70	44	7
5	F 54	Jo-1	+	3	15	33	70	38	10	+
6	F 18	Jo-1	+	2,5	12	40	NA	50	8	+
7	M 33	Jo-1	+	2,23	26	38	60	38	16
8	F 21	Jo-1		1,62	17	NA	76	55	18	+
9	M 43	Jo-1	+	1,8	41	41	60	26	7
10	F 55	Jo-1	+	0,82	56	33	98	50	15	+
No PH
11	M 60	Jo-1	+	1,87	56	28	60	23	11
12	F 12	PL-12	+	1,17	47	33	45	18	2
13	F 19	Jo-1		1,45	33	27	NA	15	4
14	F 31	PL-12	+	2,09	31	33	70	24	6

Conclusion:

In this cohort of 90 ASS patients and with a median disease duration of seven years, a PH frequency of 11 % diagnosed by RHC was observed. The study indicated a correlation between enlarged Pulmonal artery and PH. In spite of small series, the results indicate PH as an important complication of ASS and should be given further notice.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/pulmonary-hypertension-in-the-antisynthetase-syndrome/