Journal Information
Share
Share
Download PDF
More article options
Letter to the Editor
DOI: 10.1016/j.pulmoe.2020.05.005
Open Access
Available online 27 September 2020
Serial autoantibody detection in interstitial lung diseases: should they be repeated at follow-up?
Visits
...
P. Camelia, D. Platarotia, G.D. Ranab, L. Bergantinia,
Corresponding author
laurabergantini@gmail.com

Corresponding author.
, M. d'Alessandroa, E. Bargaglia
a Respiratory Diseases Unit, Department of Clinical Medicine and Immunological Sciences, Siena University, Siena, Italy
b Internal Medicine Unit, Department of Internal Medicine, Geriatrics and Rare Diseases Unit, Bari University
Received 16 March 2020
Article information
Full Text
Bibliography
Download PDF
Statistics
Full Text

Interstitial lung diseases (ILDs) are a heterogeneous group characterized by progressive thickening of the lungs; they include a wide variety of lung pathologies of known and unknown cause. Idiopathic pulmonary fibrosis (IPF) and non-specific idiopathic pneumonia (NSIP) are the most common idiopathic ILDs.1 The 2018 ATS/ERS international guidelines for the diagnosis of IPF recommend analysis of autoantibodies to exclude ILDs associated with connective tissue lung diseases (CTD-ILD).1 The diagnosis of IPF requires exclusion of other ILDs and serological evaluation of C-reactive protein, erythrocyte sedimentation rate, antinuclear antibodies, rheumatoid factor, myositis panel, and anti cyclic citrullinated peptide in newly diagnosed ILDs. Many biomarkers have been proposed in the literature for differential diagnosis but they are not yet approved for clinical use.2 The guidelines also underline that there is no clear agreement on the serological tests to perform in the initial screening.1 Autoantibody determination is mainly required in cases with atypical IPF features, including women under 60 years of age, where interstitial lung involvement may be the first manifestations of CTD-ILD.3

We therefore wondered whether autoantibody determination should be repeated regularly under specific conditions in patients with UIP pattern and negative serological test at onset, in order to verify the initial diagnosis of IPF, since some patients develop CTD manifestations months or years after the initial diagnosis.3 The question mainly concerns UIP and NSIP radiological patterns, i.e. the most frequent radiological features associated with rheumatoid arthritis, systemic sclerosis and Sjogren syndrome.4

We retrospectively evaluated a cohort of 91 patients (71 males, age 68.46 ± 7.70 years) diagnosed with IPF and treated with pirfenidone between August 2011 and January 2019: we selected patients who had undergone assay of serum autoantibodies. Five of them (2 males, age 69,8 ± 13,1 years) developed clinically evident CTD (2 Sjogren syndrome and 3 rheumatoid arthritis), that was confirmed by rheumatological tests and clinical evaluation. Concerning systemic manifestations of CTD, two patients reported sicca syndrome, one patient showed rheumatic nodules of the elbow and two patients complained of diffuse joint pain and stiffness. Mean time to CTD onset after diagnosis of IPF was 25,8 ± 33,7 months. The three patients with RA-UIP died 60 ± 31.74 months after diagnosis of ILD, showing a prognosis similar to that of IPF patients treated with pirfenidone, as already reported in the literature.5

Our results are in line with the first paper reporting this subtype of ILD patient.5 Homma et al. observed a mean latency of 24 months between IPF diagnosis and CTD, which is considerably shorter than our figure.6 This discrepancy may be due to inclusion of patients with various radiological and ILD patterns, not focused on UIP features as in our study. In confirmation of these assumptions, Kono et al. reported similar results to ours in a cohort of 111 patients diagnosed with IPF, where 9% of patients developed CTD.7

Here we reported our cases, initially diagnosed with IPF, who subsequently developed CTD. This subgroup is estimated to make up 8–10% of all IPF patients, raising the question of follow-up and sustaining the utility of taking a working diagnosis approach to ILD. We first provided survival data on these patients treated with pirfenidone; it could be worth extracting the population with UIP prior to diagnosis of CTD in order to investigate the effect of antifibrotic treatment in this cohort and the usefulness of serial determination of autoantibodies.

Conflict of interest

The present research was performed at Siena University without funding sponsors. The authors have no conflict of interest to declare.

References
[1]
G. Raghu, M. Remy-Jardin, J.L. Myers, L. Richeldi, C.J. Ryerson, D.J. Lederer, et al.
Diagnosis of Idiopathic Pulmonary Fibrosis. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline.
Am J Respir Crit Care Med, 198 (2018), pp. e44-68
[2]
M. d’Alessandro, A. Carleo, P. Cameli, L. Bergantini, A. Perrone, L. Vietri, et al.
BAL biomarkers’ panel for differential diagnosis of interstitial lung diseases.
Clin Exp Med, 20 (2020), pp. 207-216
[3]
A. Fischer, K.M. Antoniou, K.K. Brown, J. Cadranel, T.J. Corte, R.M. du Bois, et al.
An official European Respiratory Society/American Thoracic Society research statement: interstitial pneumonia with autoimmune features.
Eur Respir J, 46 (2015), pp. 976-987
[4]
N. Tanaka, J.D. Newell, K.K. Brown, C.D. Cool, D.A. Lynch.
Collagen vascular disease-related lung disease: high-resolution computed tomography findings based on the pathologic classification.
J Comput Assist Tomogr, 28 (2004), pp. 351-360
[5]
L. Vietri, P. Cameli, M. Perruzza, B. Cekorja, L. Bergantini, M. d’Alessandro, et al.
Pirfenidone in idiopathic pulmonary fibrosis: real-life experience in the referral centre of Siena.
Ther Adv Respir Dis, 14 (2020),
[6]
Y. Homma, Y. Ohtsuka, K. Tanimura, H. Kusaka, M. Munakata, Y. Kawakami, et al.
Can interstitial pneumonia as the sole presentation of collagen vascular diseases be differentiated from idiopathic interstitial pneumonia?.
Respiration, 62 (1995), pp. 248-251
[7]
M. Kono, Y. Nakamura, N. Enomoto, D. Hashimoto, T. Fujisawa, N. Inui, et al.
Usual interstitial pneumonia preceding collagen vascular disease: a retrospective case control study of patients initially diagnosed with idiopathic pulmonary fibrosis.
Copyright © 2020. Sociedade Portuguesa de Pneumologia
Pulmonology

Subscribe to our newsletter

Article options
Tools

Are you a health professional able to prescribe or dispense drugs?

Cookies policy
To improve our services and products, we use cookies (own or third parties authorized) to show advertising related to client preferences through the analyses of navigation customer behavior. Continuing navigation will be considered as acceptance of this use. You can change the settings or obtain more information by clicking here.