"Intrathoracic Manifestations of Connective Tissue Diseases on High Resolution Computed Tomography"

Diego Baenas¹, Maira Orozco², María Eugenia Olmos³, Luis Lasca⁴, Paula Riba⁵, Patricio Muszinsky⁵, Juan Pablo Pirola⁶, Verónica Saurit⁷, Alejandro Alvarellos⁷, Ana C. Alvarez⁸, Soledad Retamozo^9,10, Nadia Riscanevo^7,11, Janet Flores¹², Ariel Blua³, Ana María López¹³, Gustavo Muiño¹⁴, Santiago Orozco¹⁵ and Francisco Caeiro¹⁶, ¹Rheumatology Unit, Hospital Privado Universitario de Córdoba, Postgraduate Career of Rheumatology Catholic University of Córdoba., Cordoba, Argentina, ²Radiology, Radiology Unit, Hospital Privado Universitario de Córdoba, Cordoba, Argentina, ³Pulmonary, Pulmonary Unit, Hospital Privado Universitario de Córdoba, Cordoba, Argentina, ⁴Radiology Unit, Oulton Institute, Cordoba, Argentina, ⁵Radiology, Radiology Unit, Oulton Institute, Cordoba, Argentina, ⁶Rheumatology, Rheumatology Unit, Hospital Privado Universitario de Córdoba., Cordoba, Argentina, ⁷Rheumatology, Rheumatology Unit, Hospital Privado Universitario de Córdoba, Cordoba, Argentina, ⁸Rheumatology, Rheumatology Unit, Hospital Privado Universitario de Córdoba, Córdoba, Argentina, ⁹Instituto de Investigaciones en Ciencias de la Salud, Universidad Nacional de Córdoba, Consejo Nacional de Investigaciones Científicas y Técnicas (INICSA-UNC-CONICET), Cordoba, Argentina, ¹⁰Hospital Privado Centro Médico de Córdoba, Cordoba, Argentina, ¹¹Rheumatology Unit, Hospital Privado Universitario de Córdoba, Cordoba, Argentina, ¹²Rheumatology Unit, Rheumatology Unit, Hospital Privado Universitario de Córdoba, Cordoba, Argentina, ¹³Pulmonary Unit, Pulmonary Unit, Hospital Privado Universitario de Córdoba., Cordoba, Argentina, ¹⁴Radiology, Radiology Unit,Hospital Privado Universitario de Córdoba., Cordoba, Argentina, ¹⁵Radiology, Radiology Unit, Hospital Privado Universitario de Córdoba., Cordoba, Argentina, ¹⁶Rheumatology, Hospital Privado Centro Médico de Córdoba, Córdoba, Argentina

Meeting: 2017 ACR/ARHP Annual Meeting

Date of first publication: September 18, 2017

Keywords: interstitial lung disease, Pulmonary Involvement, Rheumatoid arthritis (RA), systemic lupus erythematosus (SLE) and systemic sclerosis

Session Information

Date: Tuesday, November 7, 2017

Session Title: Imaging of Rheumatic Diseases Poster II

Session Type: ACR Poster Session C

Session Time: 9:00AM-11:00AM

Background/Purpose:

Connective tissue diseases (CTD) can cause intrathoracic involvement, increasing patients morbidity and mortality. High-resolution computed tomography (HRCT) is a key method for evaluation of these manifestations. The aim of this study is to describe clinical features and to evaluate intrathoracic HRCT findings in patients with CTD.

Methods:

Retrospective descriptive study, from January 2010 to December 2016.We evaluated HRCT findings in patients with CTD. We excluded patients under 18 years of age, pregnant, diagnosis of vasculitis, respiratory infections, drug toxicity, and previous thoracic surgery or radiotherapy. Data were analyzed using SPSS 17.0 software.

Results:

Out of 199 patients, 96 had rheumatoid arthritis (RA), 29 systemic lupus erythematosus (SLE), 26 systemic sclerosis (SSc), 23 inflammatory myopathies (IM), 22 primary Sjögren’s syndrome (pSS) and 3 mixed connective tissue disease (MCTD). The baseline characteristics of patients are shown in table 1.

Table 1. Baselines features.

Features

(n=96)

SLE

(n=29)

SSc

(n=26)

(n=23)

pSS

(n=22)

MCTD

(n=3)

Age (x years ± SD),

Sex (female), n (%)

Disease duration (x years ± SD)

62.4 ±13.1

68 (70.8)

11.2 ± 7.5

45±15.3

27 (93.1)

8 ± 8.5

50.5±16.9

23 (88.5)

7.3 ± 6.5

58.3±14.9

17 (73.9)

6.4 ± 7

64± 9.4

20 (90.9)

8.2 ± 5.5

43.3± 7.4

3 (100)

5 ± 3

Tabaquism, n (%)

Smokers

Former smokers

Never smokers

24 (25)

30 (31.3)

42 (43.7)

9 (31)

4 (13.8)

16 (55.2)

5 (19.2)

16 (61.5)

5 (21.7)

1 (4.3)

17 (73.9)

2 (9.1)

4 (18.2)

16 (72.7)

1 (33.3)

–

Pleuropulmonary involvement as first manifestation of CTD, n (%)

6 (6.3)

2 (6.9)

4 (15.4)

2 (8.7)

5 (22.7)

–

Symptoms, n (%)

Dyspnoea

Cough

Sputum production

Chest pain

Fever

Raynaud

38 (39.6)

36 (37.5)

12 (12.5)

8 (8.3)

12 (12.5)

–

8(27.6)

12 (41.4)

1 (3.4)

4 (13.8)

7 (24.1)

10 (34.5)

14 (53.8)

13 (50)

1 (3.8)

–

1 (3.8)

23 (88.5)*

8 (34.8)

9 (39.1)

1 (4.3)

–

1 (4.3)

4 (17.4)

9 (40.9)

13 (59.1)

2 (9.1)

1 (4.5)

2 (9.1)

1 (33.3)

–

2 (66.7)

Laboratory

ESR (x mm/h ± SD)

RCP (x ± SD)

Hypocomplementemia, n (%)

41±31.5

2.3±3.7

3(3.1)

39.6±28.5

2.3±2.7

20 (69)*

50.5±16.9

1.7± 2.8

–

38.4±30

4.2±7.5

–

32.8±30

3.7±5.7

38±25.5

1.2±0.8

–

HRCT findings, n (%)

Bronchiectasis

Bronchiolitis

Pleural effusion

Pleural thickening

Pericardial effusion

Lymphadenopathy

Esophageal dilatation

Diaphragmatic elevation

PAH

28 (29.2)*

18 (18.8)

8 (8.3)

9 9.4)

8 (8.3)

2 (2.1)

–

12 (12.5)

1 (3.4)

2 (6.9)

10 (34.5)*

4 (13.8)

5 (17.3)

3 (10.3)

6 (20.7)

–

8 (27.6)

4 (15.4)

–

2 (7.7)

4 (15.4)

1 (3.8)

18 (69.2)*

–

12 (46.2)*

4 (17.4)

2 (8.7)

1 (4.3)

2 (8.7)

3 (13)

1 (4.3)

2 (8.7)

–

4 (17.4)

4 (18.2)

3 (13.6)

1 (4.5)

–

2 (9.1)

1 (4.5)

5 (22.7)

1 (4.5)

2 (9.1)

1 (33.3)

–

2 (66.7)

–

2 (66.7)

Autoantibodies, n (%)

ANA (título >1/80)

Anti-DNA

Anti-Sm

Anti-Ro (SS-A)

Anti-La (SS-B)

Anti-centromere

Anti-Scl-70

Anti-Jo1

Anti-RNP

83 (86.5)*

5 (5.2)

–

5 (5.2)

1 (1)

–

7 (24)

29 (100)*

12 (41.4)*

6 (20.7)*

13 (44.8)

7 (24.1)

–

7 (24.1)

2 (7.7)

26 (100)*

–

1 (3.8)

14 (53.8)*

7 (26.9)*

–

1 (3.8)

3 (13)

13 (56.5)

–

5 (21.7)

2 (8.7)

–

4 (17.4)*

–

7 (31.8)

11 (50)

–

8 (36.4)

2 (9.1)

–

3 (100)*

sSS, n (%)

7 (7.3)

4 (13.8)

1 (3.8)

–

Pulmonary Function Abnormalities, n (%)

Restrictive

Obstructive

post-bronchodilator FVC

post-bronchodilator FEV1

31/57(54.4)

18 (58)

13 (42)

50 (87.7)

49 (86)

7/7 (100)

6 (85.7)

1 (14.3)

7 (100)

15/20 (75)

12 (80)

3 (20)

19 (95)

6/15 (40)

4 (66.7)

2 (33.3)

11 (73.3)

11/16(68.8)

10 (90.9)

1 (9.1)

11 (100)

2/3 (66.7)

1 (50)

3 (100)

Abnormal 6MWT, n (%)

Abnormal DLCO, n (%)

*p<0.05.

7/21 (33.3)

19/22(86.4)

1/2 (50)

6/6 (100)

8/15 (53.3)

13/15 (86.6)

2/7 (28.6)

11/13 (84.6)

3/4 (75)

2/3 (66.7)

The mean age was 58 years (range 18-84, SD ± 15.5) with a female predominance in all groups. In 10% of cases pleuropulmonary involvement was the first manifestation of CTD. HRCT findings and ILD patterns are shown in table 1 and 2 respectively.

Table 2. HRCT findings in CTD.

Diseases (n) HRCT findings n, (%)	RA (42)	SLE (18)	SSc (14)	IM (19)	pSS (16)	MCTD (1)	TOTAL
NSIP	17 (40.5)	11 (61.1)	9 (64.3)	15 (78.9)	10 (62.4)	1 (100)	63
UIP	22 (52.4)	—	5 (35.7)	2 (10.5)	3 (18.8)	—	32
COP	3 (5.8)	4 (22.2)	—	1 (5.3)	—	—	8
LIP	—	—	—	1 (5.3)	3 (18.8)	—	4
DAD	—	3 (16.7)	—	—	—	—	3
Patterns frequency DAD: Diffuse alveolar damage; LIP: Lymphocytic interstitial pneumonia.	UIP>NSIP>COP	NSIP>COP>DAD	NSIP>UIP	NSIP>UIP>COP=LIP	NSIP > UIP =LIP	NSIP

Interstitial lung disease (ILD) was observed in 55.3% of patients.

There was a higher frequency of bronchiectasis in RA, esophageal dilation in SSc and pleural effusion in SLE (p <0.05).

Pleural involvement was the most frequent manifestation in SLE (48.3%) and esophageal involvement in SSc (69.2%).

In RA we observed bronchial involvement in 47.9% of patients and pleural in 17.7%. 43.8% of the patients with RA presented ILD; usual interstitial pneumonia (UIP) was the most frequent pattern in 52.4% followed by non-specific interstitial pneumonia (NSIP) in 40.5% and cryptogenic Organizing Pneumonia (COP) in 7.1%. All patients with RA and ILD in our sample had positive rheumatoid factor (RF). Only 2 patients with RA had rheumatoid nodules.

NSIP was the most frequent pattern of ILD in the remaining CTD (37.9% in SLE, 34.6% in SSc, 65.2% in IM, 45.5% pSS and 33.3% in MCTD).

Tomographic signs of pulmonary arterial hypertension (PAH) were evidenced in 40 patients, 30% with RA and 30% SSc. A statistically significant association was found between PAH and SSc (p = 0.0) and among the findings of PAH in HRCT and SD pattern in capillaroscopy (p = 0.01).

Conclusion:

Intrathoracic manifestations are common in patients with CTD. A comprehensive knowledge of the pleuropulmonary involvement in CTD is important, because the prognosis and optimal therapy differ for each presentation.

Disclosure: D. Baenas, None; M. Orozco, None; M. E. Olmos, None; L. Lasca, None; P. Riba, None; P. Muszinsky, None; J. P. Pirola, None; V. Saurit, None; A. Alvarellos, None; A. C. Alvarez, None; S. Retamozo, None; N. Riscanevo, None; J. Flores, None; A. Blua, None; A. M. López, None; G. Muiño, None; S. Orozco, None; F. Caeiro, None.

To cite this abstract in AMA style:

Baenas D, Orozco M, Olmos ME, Lasca L, Riba P, Muszinsky P, Pirola JP, Saurit V, Alvarellos A, Alvarez AC, Retamozo S, Riscanevo N, Flores J, Blua A, López AM, Muiño G, Orozco S, Caeiro F. “Intrathoracic Manifestations of Connective Tissue Diseases on High Resolution Computed Tomography” [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/intrathoracic-manifestations-of-connective-tissue-diseases-on-high-resolution-computed-tomography/. Accessed February 23, 2019.

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