Impact of Rheumatologic Consultations on Detecting Interstitial Lung Disease Associated with Connective Tissue Disease

Myung-Eun Song; Jisoo Lee; Sun-Hee Roh

doi:10.4078/jrd.2011.18.3.168

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Song, Lee, and Roh: Impact of Rheumatologic Consultations on Detecting Interstitial Lung Disease Associated with Connective Tissue Disease

Original Article

J Rheum Dis 2011;18(3):168-174.

Published online: 31 September 2011

DOI: https://doi.org/10.4078/jrd.2011.18.3.168

Impact of Rheumatologic Consultations on Detecting Interstitial Lung Disease Associated with Connective Tissue Disease

Myung-Eun Song, Jisoo Lee, Sun-Hee Roh

Division of Rheumatology, Ewha Womans University School of Medicine, Seoul, Korea

Received 15 November 20104 March 2011 Accepted 4 March 2011

Abstract

Objective

In patients with interstitial lung disease (ILD) associated with connective tissue disease (CTD), chronic ILD related symptoms may often dominate the clinical picture or precede systemic findings and thus often be seen by a non-rheumatologist. The purpose of this study was to evaluate the importance of rheumatologic consultation during ILD work up.

Methods

We retrospectively reviewed 64 patients with ILD from a single tertiary center for their clinical and laboratory characteristics, rheumatologic consultation status, and result of the consultation. American College of Rheumatology criteria for classification of each connective tissue disease were utilized. Undifferentiated connective tissue disease (UCTD) was classified by pre-specified criteria.

Results

A total of 23 (36%) of the ILD patients had associated CTD. Five (8%) patients had underlying CTD before the diagnosis of ILD, whereas 18 (28%) patients were diagnosed with CTD after the rheumatologic consultation. ILD patients with CTD were predominantly female, had significantly more frequent radiographic diagnoses of nonspecific interstitial pneumonia, increased frequencies of high titer antinuclear antibody positivity, and rheumatoid factor positivity. Rheumatologic consultation was referred in 36 (56%) patients. In 18 (50%) of the referred patients, CTD was diagnosed. In 61% of the patients diagnosed with CTD as a result of rheumatologic consultation, changes in therapy occurred.

Conclusion

A substantial proportion of patients with ILD are found to have an underlying CTD upon evaluation by a rheumatologist. Since ILD associated with CTD mimics idiopathic interstitial pneumonia, rheumatologic consultation may have a significant impact on the clinical care of ILD.

Keywords: Interstitial lung disease, Connective tissue diseases, Rheumatologic consultation

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Figure 1.

Connective tissue disease associations in interstitial lung disease patients studied. ∗Number (%) of patients. CTD, connective tissue disease, RA: rheumatoid arthritis, SSc: systemic sclerosis, DM: dermatomyositis, SLE: systemic lupus erythematosus, Vas: systemic vasculitis, AS: ankylosing spondylitis, UCTD: undifferentiated connective tissue disease.

Table 1.

Baseline characteristics of patients with interstitial lung disease

	n=64
Age, years	65.6 (12.4)
Male, n (%)	34 (53)
Radiographic ILD type, n (%)
UIP	37 (57.8)
NSIP	12 (18.8)
COP	12 (18.8)
DIP	2 (3.1)
AIP	1 (1.6)
ANA positivity, n (%)	37/56 (66.1)
RF positivity, n (%)	18/56 (32.1)
FVC, %	73.7 (19.2)
DL_CO, %	64.2 (23.1)
Rheumatologic consultation requested	36 (56.3%)

Data are presented as mean (SD) except where noted otherwise. ILD: interstitial lung disease, UIP: usual interstitial pneumonia, NSIP: nonspecific interstitial pneumonia, COP: cryptogenic organizing pneumonia, DIP: desquamative interstitial pneumonia, AIP: acute interstitial pneumonia, ANA: antinuclear antibody, RF: rheumatoid factor, FVC: forced vital capacity, DL _CO: carbon monoxide diffusion in the lung.

Table 2.

Characteristics of interstitial lung disease patients grouped according to their rheumatologic consultation status

	Not consulted (n=28)	Consulted (n=36)	p-value
Age, years	72.9 (8.8)	59.9 (11.8)	0.001
Male, n (%)	17 (60.7)	17 (47.2)	0.283
Radiographic ILD type, n (%)
UIP	21 (75)	16 (44.4)	0.014
NSIP	3 (10.7)	9 (25.0)	0.146
COP	4 (14.3)	8 (22.2)	0.420
DIP	0	2 (5.6)	0.205
AIP	0	1 (2.8)	0.374
ANA positivity, n (%)	8/20 (40.0)	19/36 (52.8)	0.359
1 : 40∼≤1 : 80	7/20 (35.0)	18/36 (50.0)	0.279
≥1 : 160	1/20 (5.0)	11/36 (30.6)	0.026
RF positivity, n (%)	6/20 (30.0)	24/36 (66.7)	0.008
ESR, mm/hr	27.9 (28.3)	27.2 (24.7)	0.922
FVC, %	71.6 (15.4)	77.0 (21.4)	0.312
DL _CO, %	64.5 (23.6)	64.6 (22.5)	0.986

Data are presented as mean (SD) except where noted otherwise. ILD: interstitial lung disease, UIP: usual interstitial pneumonia, NSIP: nonspecific interstitial pneumonia, COP: cryptogenic organizing pneumonia, DIP: desquamative interstitial pneumonia, AIP: acute interstitial pneumonia, ANA: antinuclear antibody, RF: rheumatoid factor, ESR: erythrocyte sedimentation rate, FVC: forced vital capacity, DL _CO: carbon monoxide diffusion in the lung.

Table 3.

Characteristics of patients with and without connective tissue disease^∗

	ILD with CTD (n=23)	ILD without CTD (n=41)	p
Age, years	62.3 (11.6)	67.4 (12.6)	0.109
Male, n (%)	8 (34.8)	26 (63.4)	0.028
Radiographic ILD type, n (%)
UIP	10 (43.5)	27 (65.9)	0.082
NSIP	8 (34.8)	4 (9.8)	0.014
COP	4 (17.4)	8 (19.5)	0.835
DIP	1 (4.3)	1 (2.4)	0.674
AIP	0	1 (2.4)	0.450
Biopsy, n (%)	12 (52.2)	13 (31.7)	0.107
Transbronchial lung biopsy	8 (34.8)	5 (12.2)	0.031
Open lung biopsy	4 (17.4)	8 (19.5)	0.835
FVC, %	69.9 (24.6)	75.4 (16.5)	0.371
DL _CO, %	60.1 (25.6)	65.9 (22.1)	0.431
ESR, mean± SD, mm/hr	28.2 (26.4)	27.1 (26.2)	0.882
ANA positivity, n (%)	18/23 (78.3)	19/33 (57.5)	0.108
1 : 40∼≤1 : 80	10 (43.5)	15 (45.5)	0.884
≥1 : 160	8 (34.8)	4 (12.1)	0.042
RF positivity, n (%)	12/23 (52.2)	7/33 (21.2)	0.016

Data are presented as mean (SD) except where noted otherwise. ILD: interstitial lung disease, UIP: usual interstitial pneumonia, NSIP: nonspecific interstitial pneumonia, COP: cryptogenic organizing pneumonia, DIP: desquamative interstitial pneumonia, AIP: acute interstitial pneumonia, ANA: antinuclear antibody, RF: rheumatoid factor, ESR: erythrocyte sedimentation rate, FVC: forced vital capacity DL _CO: carbon monoxide diffusion in the lung.

Table 4.

Changes in therapy in interstitial lung disease patients diagnosed with connective disease after rheumatologic consultation

Patient	Post-consultation diagnosis	Serology	Imaging pattern	Changes in treatment
73 F	SSc	+ANA, +SSA	UIP	CS, MTX
69 M	DM	+ANA	COP	High CS
74 M	UCTD	+ANA, +RF, +LA	UIP	CS, AZT
68 F	SSc	+ANA +SSB	COP	No change
59 F	RA	+ANA, +RF, +SSA, +ANCA, +CCP	COP	No change
71 M	UCTD	+RF, +ANCA	NSIP	No change
61 F	UCTD	+ANA, +ANCA	NSIP	No change
73 F	RA	+ANA, +ANCA	UIP	High CS
61 F	Vas	+RF, +ANCA	DIP	High CS, CY
61 M	RA	+ANA, +ANCA	NSIP	HCQ, SSZ
55 F	SLE	+ANA, +RF, +SSA	UIP	CS, HCQ, AZT
47 F	SLE	+ANA, +DNA +SSA	NSIP	CS, HCQ, AZT
61 M	DM	+ANA, +SSA	NSIP	High CS
54 F	UCTD	+ANA, +SSA	UIP	No change
61 M	AS	+ANA	UIP	No change
38 F	Vas	+ANA	UIP	High CS, CY
74 F	UCTD	+ANA, +RF	NSIP	No change
35 M	DM	Negative	NSIP	High CS, AZT

RA: rheumatoid arthritis, SSc: systemic sclerosis, DM: dermatomyositis, SLE: systemic lupus erythematosus, Vas: systemic vasculitis, AS: ankylosing spondylitis, UCTD: undifferentiated connective tissue disease, UIP: usual interstitial pneumonia, COP: cryptogenic organizing pneumonia, DIP: desquamative interstitial pneumonia, ANA: antinuclear antibody, SSA: anti-SSA, RF: rheumatoid factor, LA: lupus anticoagulant, SSB: anti-SSB, ANCA: antineutrophilic cytoplasmic antibody, CCP: anti-cyclic citrullinated peptide, DNA: anti-double strand DNA, NSIP: nonspecific interstitial pneumonia, CS: corticosteroid, High CS: ≥1 mg/kg/d corticosteroid, CY: cyclophosphamide, MTX: methotrexate, HCQ: hydroxychloroquine, SSZ: sulfasalazine, AZT: azathioprine.

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