Journal List > J Rheum Dis > v.18(3) > 1063906

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

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.

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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.
jrd-18-168f1.tif
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)
DLCO, % 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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