Skip to main content

Main menu

  • Home
  • Current issue
  • Early View
  • Archive
  • Authors/reviewers
    • Instructions for authors
    • Submit a manuscript
    • COVID-19 submission information
    • Institutional open access agreements
    • Peer reviewer login
  • Alerts
  • Subscriptions
  • ERS Publications
    • European Respiratory Journal
    • ERJ Open Research
    • European Respiratory Review
    • Breathe
    • ERS Books
    • ERS publications home

User menu

  • Log in
  • Subscribe
  • Contact Us
  • My Cart

Search

  • Advanced search
  • ERS Publications
    • European Respiratory Journal
    • ERJ Open Research
    • European Respiratory Review
    • Breathe
    • ERS Books
    • ERS publications home

Login

European Respiratory Society

Advanced Search

  • Home
  • Current issue
  • Early View
  • Archive
  • Authors/reviewers
    • Instructions for authors
    • Submit a manuscript
    • COVID-19 submission information
    • Institutional open access agreements
    • Peer reviewer login
  • Alerts
  • Subscriptions

Diffuse panniculitis in a teenage male with ZZ α1-antitrypsin deficiency

Spyros A. Papiris, Anthimos Parmaxidis, Sofia Theotokoglou, Zoe Tsakiraki, Martina Veith, Aikaterini Panagiotou, Vasiliki Pappa, Maria Kallieri, Jean-François Mornex, Alexander C. Katoulis, Dionysios Haritos, Ioannis G. Panayiotides, Effrosyni D. Manali
ERJ Open Research 2023 9: 00546-2022; DOI: 10.1183/23120541.00546-2022
Spyros A. Papiris
12nd Pulmonary Medicine Department, General University Hospital Attikon, Medical School, National and Kapodistrian University of Athens, Athens, Greece
8These authors contributed equally
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Anthimos Parmaxidis
22nd Propaedeutic Department of Internal Medicine, General University Hospital Attikon, Medical School, National and Kapodistrian University of Athens, Athens, Greece
8These authors contributed equally
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Sofia Theotokoglou
32nd Department of Dermatology and Venereology, General University Hospital Attikon, Medical School, National and Kapodistrian University of Athens, Athens, Greece
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Zoe Tsakiraki
42nd Second Department of Pathology, National and Kapodistrian University of Athens, Attikon University General Hospital, Athens, Greece
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Martina Veith
5Department of Medicine, Pulmonary and Critical Care Medicine, UKGM, Member of the German Center for Lung Research (DZL), Marburg, Germany
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Aikaterini Panagiotou
22nd Propaedeutic Department of Internal Medicine, General University Hospital Attikon, Medical School, National and Kapodistrian University of Athens, Athens, Greece
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Vasiliki Pappa
22nd Propaedeutic Department of Internal Medicine, General University Hospital Attikon, Medical School, National and Kapodistrian University of Athens, Athens, Greece
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Maria Kallieri
12nd Pulmonary Medicine Department, General University Hospital Attikon, Medical School, National and Kapodistrian University of Athens, Athens, Greece
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Jean-François Mornex
6Hospices Civils de Lyon, Groupement Hospitalier Est, Service de Pneumologie, Centre National de Référence des Maladies Pulmonaires Rares, INSERM CIC 1407, Lyon, France
7Université de Lyon, Université de Lyon, Université Lyon 1, UMR754 INRAE, IVPC, Lyon France
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Alexander C. Katoulis
32nd Department of Dermatology and Venereology, General University Hospital Attikon, Medical School, National and Kapodistrian University of Athens, Athens, Greece
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Dionysios Haritos
22nd Propaedeutic Department of Internal Medicine, General University Hospital Attikon, Medical School, National and Kapodistrian University of Athens, Athens, Greece
8These authors contributed equally
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Ioannis G. Panayiotides
42nd Second Department of Pathology, National and Kapodistrian University of Athens, Attikon University General Hospital, Athens, Greece
8These authors contributed equally
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Effrosyni D. Manali
12nd Pulmonary Medicine Department, General University Hospital Attikon, Medical School, National and Kapodistrian University of Athens, Athens, Greece
8These authors contributed equally
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • Article
  • Figures & Data
  • Info & Metrics
  • PDF
Loading

Abstract

Diffuse panniculitis is a rare manifestation of α1-ATD, albeit perhaps the most fulminant and life-threatening complication, associated usually with ZZ phenotype. Intravenous α1-AT treatment is lifesaving. https://bit.ly/3EDmCzT

To the Editor:

Diffuse panniculitis is an inflammatory condition of the subcutaneous fat associated with a multiplicity of aetiological factors and nosological conditions [1, 2]. Diffuse panniculitis commonly occurs spontaneously and presents with painful skin nodules occasionally evolving into skin-ulcerating lesions discharging oily, yellow exudate. Histology shows an inflammatory infiltrate with lobular, septal or combined distribution, depending on the subjacent entity and the timing of biopsy, consisting of neutrophils, lymphocytes, histiocytes or a combination thereof; moreover, foamy macrophages, multinuclear giant cells, granulomas, necrosis or vasculitis may be seen [3]. Lesions may heal spontaneously (albeit less commonly) or after appropriate treatment with atrophic scarring. Any part of the superficial body may be involved although upper and lower extremities are more commonly affected [3]. Occasionally, it presents as part of a systemic inflammatory syndrome involving several extra-skin tissues and organs, and associates with thrombosis, a life-threatening clinical scenario [2].

A 17-year-old patient, a current smoker with a body mass index of 33.2 kg·m−2, presented to the emergency department complaining of erythematous, painful, indurating skin nodular lesions and low-grade fever over the previous 2 weeks (figure 1a). His previous medical and family history were noncontributory. Lesions were located bilaterally in the axillary regions, at the right abdominal surface and in both glutei. Differential diagnosis included mostly erythema nodosum and autoimmune rheumatic disease. After dermatological evaluation, a diagnosis of diffuse panniculitis was proposed. From laboratory evaluation, abnormal values included high C-reactive protein (CRP) (65 mg·L−1), low folic acid (1 ng·mL−1), low vitamin B12 (138 pg·mL−1), low albumin (2.7 mg·dL−1), low total serum proteins (4.8 mg·dL−1) and high ferritin levels (447 ng·mL−1). Computed tomography (CT) of the thorax and abdomen disclosed only diffuse oedema of the subcutaneous fat in the affected areas. Empirical antimicrobial treatment was started initially with a β-lactam, then changed to combined meropenem and linezolid upon deterioration. Due to low vitamin and albumin levels, enteric malabsorption was suspected, but endoscopy of the upper and lower gastrointestinal tract and capsule endoscopy for the small intestine did not detect abnormalities. Surgical biopsy of subcutaneous fat showed septal panniculitis with many histiocytes (figure 1b–d). Serology for autoimmunity, testing for HIV, hepatitis B virus and hepatitis C virus, and Quantiferon test were all negative. While lesions located in the glutei and the left axillary region became ulcerated, leaking oily, yellow exudates (figure 1a), the right abdominal wall lesion subsided spontaneously. New painful lesions appeared in concomitance with excessive subcutaneous oedema on the arms, scrotum and thighs. The lesions in the right arm and thighs ulcerated. Exudate cultures for common and specific pathogens including fungi and mycobacteria proved sterile. The patient continued to be febrile and his general clinical condition deteriorated. Due to excessive oedema of the arms and high D-dimer values, CT pulmonary angiography was performed, excluding pulmonary embolism. In the next few days and while lesions on the right upper arm appeared to subside, an insidious subcutaneous emphysema developed.

FIGURE 1
  • Download figure
  • Open in new tab
  • Download powerpoint
FIGURE 1

a) Erythematous, painful, indurating skin nodular lesions, partly ulcerated and leaking oily, yellow exudate. b) Panniculitis with inflammation mainly centring on fibrous septa (septal type) (haematoxylin and eosin (H&E) stain, 40× magnification). c) Histiocytic predominance in the inflammatory infiltrate (H&E stain, 40× magnification). d) Immunostaining for CD68 (PGM-1 clone) confirming histiocytic predominance (40× magnification).

In the course of diagnostic work-up, serum α1-antitrypsin (α1-AT) levels were found to be extremely low (0.3 g·L−1, normal values 0.9–2.0 g·L−1) and the diagnosis of α1-antitrypsin deficiency (α1-ATD)-associated diffuse panniculitis as part of a systemic inflammatory syndrome was established; isoelectric focusing and genotyping confirmed the ZZ genotype/phenotype. Pulmonary function testing was normal except a diffusing capacity of the lung for carbon monoxide of 68% predicted (systemic inflammatory syndrome with pleural effusions and obesity). Dapsone not being available for use, doxycycline was initiated, a course of plasmapheresis was performed and sequentially, intravenous augmentation infusion with α1-AT was started at a dosage of 100 mg·kg−1 after authorisation was obtained. The day after the initiation of the augmentation therapy, the patient presented remarkable improvement of his general condition. Rapid remission of the inflammatory lesions and normalisation of the laboratory tests followed. The patient was discharged, smoking cessation was recommended and the next administration of augmentation treatment was scheduled. On the day of readmission (the 10th day), a mild reactivation of the disease was evident, rapidly responding to the next i.v. α1-AT administration.

Diffuse panniculitis associated with α1-ATD is an extremely rare, underdiagnosed systemic manifestation, this is potentially severe or even lethal, associated usually with ZZ genotype [4, 5]. α1-AT is the most abundant serum and tissue circulating antiprotease, produced mainly by liver hepatocytes [6]. α1-AT acts as a protease inhibitor preferentially targeting excess human neutrophil elastase and, by protecting lungs connective tissue, prevents early emphysema development. α1-ATD is one of the most common genetic conditions and the Z variant in the homozygous state accounts for the 1–2% of all pulmonary emphysemas. Low or absent plasma levels and/or dysfunctional α1-AT molecules, including mutant Z molecules in the form of polymers, increase the risk of developing early pulmonary emphysema, liver disease and, rarely, other systemic manifestations, including diffuse panniculitis and systemic vasculitis [7]. Cigarette smoking is considered the major additional risk factor for emphysema development [8]. Warter et al. [9], in 1972, first described the association between diffuse panniculitis and α1-ATD. Since then, >100 patients have been described, mostly associated with the ZZ phenotype [4].

α1-AT is an effective inhibitor of several serine proteinases in addition to neutrophil elastase (its main target), such as cathepsin G, trypsin, chymotrypsin, plasminogen activator and serine proteinase-3 [6]. In addition, α1-AT is a very potent systemic anti-inflammatory molecule able to regulate neutrophilic chemotaxis, activation and degranulation, and affecting immune response, autoimmunity and apoptosis through its interactions with interleukin 8, leukotriene B4 and tumour necrosis factor α [10]. Severe deficiency alleles, such as PiZ, PiSSiiyama, PiMMalton and PiKKings, present low serum α1-AT levels not by reducing synthesis in the liver hepatocytes but by its excessive degradation in the endoplasmic reticulum in a great proportion and by the intracellular formation of polymers of the mutant protein [7]. The accumulation of the above, because of their toxicity (gain of function), relates to neonatal hepatitis syndrome, early-life cirrhosis and hepatocellular carcinoma. Milder deficiency alleles such as the PiS, PiI and PiQueen's form polymers but at a slower rate [11]. Circulating polymers of the mutant protein not only lose any antiprotease and anti-inflammatory function but acquire a new and potent proinflammatory action at sensitive sites of the body (lung, liver, subcutis and vessels) to induce, sustain and increase inflammation, and provoke tissue damage [12]. This combined mechanism, loss of plasma antiprotease potential due to the serum α1-AT levels (loss of function) and increase of protease burden due to Z polymers’ action on neutrophilic local inflammation (gain of function) are considered the pathogenetic mechanism of tissue damage in diffuse panniculitis [13]. This putative mechanism is further confirmed by the prompt and excellent response that augmentation therapy with i.v. α1-AT provides in almost all patients; offering of a fresh pool of wild, highly anti-inflammatory molecules reduces local inflammation and restores tissue damage [4, 14]. In our patient, immediately after the confirmation of the diagnosis of diffuse panniculitis related to the α1-ATD ZZ phenotype, doxycycline was initiated mainly for its anti-inflammatory and immune-regulatory actions, plasmapheresis in an attempt to eliminate polymers of the Z mutant protein from blood and tissues, and augmentation therapy in order to offer targeted anti-inflammatory action.

To conclude, we describe a rare manifestation of α1-ATD, albeit perhaps the most fulminant and life-threatening complication of α1-ATD in adults with the ZZ and, much more rarely, with the SZ and MZ phenotype. Biopsy of the lesions, serum α1-AT levels with CRP and phenotyping/genotyping are indispensable to confirm the diagnosis. In the setting of a high CRP level associated with the inflammatory syndrome in diffuse panniculitis, sometimes the α1-AT levels may increase to higher levels, further emphasising the need for genotyping or phenotyping to make the diagnosis of α1-ATD. The recognised treatment options include dapsone, tetracyclines, intravenous α1-AT, plasmapheresis (low case numbers) and liver transplant (low case numbers) [4, 5]. So far, augmentation therapy is life-saving treatment [4]. In perspective, new drugs like fazirsiran (an RNA-interfering molecule that acts by degrading α1-AT and Z-α1-AT mRNA, thereby significantly reducing Z-α1-AT protein synthesis in hepatocytes and, therefore, leakage of Z polymers in plasma and tissues) may find a place in the treatment of α1-ATD-related diffuse panniculitis [15] in combination with contemporaneous treatment with intravenous α1-AT.

Acknowledgements

Maria Sfika, Vasiliki Apollonatou (both National and Kapodistrian University of Athens, Athens, Greece), Lykourgos Kolilekas (Chest Diseases Hospital of Athens Sotiria, Athens, Greece), Emmanouil Korakas, Dimitrios Lygkos and Prof. Konstantinos Triantafyllou (all three National and Kapodistrian University of Athens, Athens, Greece) for patient care and assistance.

Footnotes

  • Provenance: Submitted article, peer reviewed.

  • Author contributions: S.A. Papiris made major contributions to the concept and design of the study, and to the acquisition, analysis and interpretation of data, and wrote the manuscript; A. Parmaxidis made major contributions to the management of the patient, and to the acquisition, analysis and interpretation of data, and wrote part of the manuscript; S. Theotokoglu and Z. Tsakiri made major contributions to the documentation of the diagnosis, to the analysis and interpretation of data, and wrote part of the manuscript; M. Veith performed the genetic analysis for the patient, and made major contributions to the interpretation of data and revised critically this work for very important intellectual content; A. Parmaxidis, V. Pappa and M. Kallieri made major contributions to the management of the patient and interpretation of data, and revised critically this work for very important intellectual content; J-F. Mornex made major contributions to the interpretation of data and revised this work critically for very important intellectual content; A.C. Katoulis had major contribution to the documentation of the diagnosis, analysis and interpretation of data and revised critically this work for very important intellectual content; D. Haritos made major contributions to the management of the patient, and the acquisition, analysis and interpretation of data, and critically revised this work for very important intellectual content; I.G. Panayiotides made major contributions to the documentation of the diagnosis, analysis and interpretation of data, drafted part of the manuscript, and critically revised this work for very important intellectual content; E.D. Manali made major contributions to the concept and design of the study, to the acquisition, analysis and interpretation of data, drafted part of the manuscript, had access to all data, supervised the accuracy and integrity of any part of the work and revised critically this work for very important intellectual content. All authors read and approved of the final version of the submitted manuscript.

  • Conflict of interest: S.A. Papiris declares payment or honoraria from DEMO SA, in the 36 months prior to manuscript submission. J-F. Mornex declares consulting fees from CSL Behring, Grifols, LFB and Takeda; and payment or honoraria and support for attending meetings from CSL Behring, Grifols and LFB, all in the 36 months prior to manuscript submission; and that they are a member of the scientific board of ADAAT. E.D. Manali declares support for attending meetings from CSL Behring in the 36 months prior to manuscript submission. All other authors declare no competing interests.

  • Received October 17, 2022.
  • Accepted November 17, 2022.
  • Copyright ©The authors 2023
http://creativecommons.org/licenses/by-nc/4.0/

This version is distributed under the terms of the Creative Commons Attribution Non-Commercial Licence 4.0. For commercial reproduction rights and permissions contact permissions{at}ersnet.org

References

  1. ↵
    1. Anand NC,
    2. Takaichi M,
    3. Johnson EF, et al.
    Suggestions for a new clinical classification approach to panniculitis based on a Mayo Clinic experience of 207 cases. Am J Clin Dermatol 2022; 23: 739–746. doi: 10.1007/s40257-022-00709-9
    OpenUrl
  2. ↵
    1. Wick MR
    . Panniculitis: a summary. Semin Diagn Pathol 2017; 34: 261–272. doi:10.1053/j.semdp.2016.12.004
    OpenUrl
  3. ↵
    1. Caronje E,
    2. Brenn T,
    3. Lazar AJ, et al.
    1. Guzar B,
    2. Calonje E
    . Inflammatory diseases of the subcutaneous fat. In: Caronje E, Brenn T, Lazar AJ, et al., eds. McKee's Pathology of the skin with clinical correlations, 5th Edn. Elsevier Ltd, 2020; pp. 351–389.
  4. ↵
    1. Franciosi AN,
    2. Ralph J,
    3. O'Farrell NJ, et al.
    Alpha-1 antitrypsin deficiency-associated panniculitis. J Am Acad Dermatol 2022; 87: 825–832. doi:10.1016/j.jaad.2021.01.074
    OpenUrl
  5. ↵
    1. Johnson EF,
    2. Tolkachjov SN,
    3. Gibson LE
    . Alpha-1 antitrypsin deficiency panniculitis: clinical and pathologic characteristics of 10 cases. Int J Dermatol 2018; 57: 952–958. doi:10.1111/ijd.14012
    OpenUrl
  6. ↵
    1. Strnad P,
    2. McElvaney NG,
    3. Lomas DA
    . Alpha 1-antitrypsin deficiency. N Engl J Med 2020; 382: 1443–1455. doi:10.1056/NEJMra1910234
    OpenUrl
  7. ↵
    1. Lomas DA
    . Twenty years of polymers: a personal perspective on alpha-1 antitrypsin deficiency. COPD 2013; 10: Suppl. 1, 17–25. doi:10.3109/15412555.2013.764401
    OpenUrlCrossRef
  8. ↵
    1. Demeo DL,
    2. Sandhaus RA,
    3. Barker AF, et al.
    Determinants of airflow obstruction in severe alpha-1–antitrypsin deficiency. Thorax 2007; 62: 806–813. doi:10.1136/thx.2006.075846
    OpenUrlAbstract/FREE Full Text
  9. ↵
    1. Warter J,
    2. Storck D,
    3. Grosshans E, et al.
    Weber-Christian syndrome associated with an alpha-1 antitrypsin deficiency. Familial investigation. Ann Med Interne (Paris) 1972; 123: 877–882.
    OpenUrlPubMed
  10. ↵
    1. Strnad P,
    2. Brantly ML,
    3. Bals R
    1. Saltini C,
    2. Krotova K
    . Mechanisms of lung disease. In: Strnad P, Brantly ML, Bals R, eds. α1-Antitrypsin Deficiency (ERS Monograph). Sheffield, European Respiratory Society, 2019; pp. 52–63.
  11. ↵
    1. Fromme M,
    2. Schneider CV,
    3. Pereira V, et al.
    Hepatobiliary phenotypes of adults with alpha-1 antitrypsin deficiency. Gut 2022; 71: 415–423. doi:10.1136/gutjnl-2020-323729
    OpenUrlAbstract/FREE Full Text
  12. ↵
    1. Haq I,
    2. Irving JA,
    3. Saleh AD, et al.
    Deficiency mutations of alpha-1 antitrypsin. effects on folding, function, and polymerization. Am J Respir Cell Mol Biol 2016; 54: 71–80. doi:10.1165/rcmb.2015-0154OC
    OpenUrlCrossRefPubMed
  13. ↵
    1. Gross B,
    2. Grebe M,
    3. Wencker M, et al.
    New findings in PiZZ α1-antitrypsin deficiency-related panniculitis. Demonstration of skin polymers and high dosing requirements of intravenous augmentation therapy. Dermatology 2009; 218: 370–375. doi:10.1159/000202982
    OpenUrlCrossRefPubMed
  14. ↵
    1. Smith KC,
    2. Pittelkow MR,
    3. Su WP
    . Panniculitis associated with severe alpha 1 antitrypsin deficiency. Treatment and review of the literature. Arch Dermatol 1987; 123: 1655–1661. doi:10.1001/archderm.1987.01660360083017
    OpenUrlCrossRefPubMed
  15. ↵
    1. Strnad P,
    2. Mandorfer M,
    3. Choudhury G, et al.
    Fazirsiran for liver disease associated with alpha 1-antitrypsin deficiency. N Engl J Med 2022; 387: 514–524. doi:10.1056/NEJMoa2205416
    OpenUrl
PreviousNext
Back to top
Vol 9 Issue 2 Table of Contents
ERJ Open Research: 9 (2)
  • Table of Contents
  • Index by author
Email

Thank you for your interest in spreading the word on European Respiratory Society .

NOTE: We only request your email address so that the person you are recommending the page to knows that you wanted them to see it, and that it is not junk mail. We do not capture any email address.

Enter multiple addresses on separate lines or separate them with commas.
Diffuse panniculitis in a teenage male with ZZ α1-antitrypsin deficiency
(Your Name) has sent you a message from European Respiratory Society
(Your Name) thought you would like to see the European Respiratory Society web site.
CAPTCHA
This question is for testing whether or not you are a human visitor and to prevent automated spam submissions.
Print
Citation Tools
Diffuse panniculitis in a teenage male with ZZ α1-antitrypsin deficiency
Spyros A. Papiris, Anthimos Parmaxidis, Sofia Theotokoglou, Zoe Tsakiraki, Martina Veith, Aikaterini Panagiotou, Vasiliki Pappa, Maria Kallieri, Jean-François Mornex, Alexander C. Katoulis, Dionysios Haritos, Ioannis G. Panayiotides, Effrosyni D. Manali
ERJ Open Research Mar 2023, 9 (2) 00546-2022; DOI: 10.1183/23120541.00546-2022

Citation Manager Formats

  • BibTeX
  • Bookends
  • EasyBib
  • EndNote (tagged)
  • EndNote 8 (xml)
  • Medlars
  • Mendeley
  • Papers
  • RefWorks Tagged
  • Ref Manager
  • RIS
  • Zotero
Share
Diffuse panniculitis in a teenage male with ZZ α1-antitrypsin deficiency
Spyros A. Papiris, Anthimos Parmaxidis, Sofia Theotokoglou, Zoe Tsakiraki, Martina Veith, Aikaterini Panagiotou, Vasiliki Pappa, Maria Kallieri, Jean-François Mornex, Alexander C. Katoulis, Dionysios Haritos, Ioannis G. Panayiotides, Effrosyni D. Manali
ERJ Open Research Mar 2023, 9 (2) 00546-2022; DOI: 10.1183/23120541.00546-2022
Reddit logo Technorati logo Twitter logo Connotea logo Facebook logo Mendeley logo
Full Text (PDF)

Jump To

  • Article
    • Abstract
    • Acknowledgements
    • Footnotes
    • References
  • Figures & Data
  • Info & Metrics
  • PDF

Subjects

  • COPD and smoking
  • Tweet Widget
  • Facebook Like
  • Google Plus One

More in this TOC Section

  • A 3D-engineered silicone stent
  • Obesity does not modify effect of CPAP on insulin resistance
  • Broadening the scope for 3D-printed airway stents
Show more Research letters

Related Articles

Navigate

  • Home
  • Current issue
  • Archive

About ERJ Open Research

  • Editorial board
  • Journal information
  • Press
  • Permissions and reprints
  • Advertising

The European Respiratory Society

  • Society home
  • myERS
  • Privacy policy
  • Accessibility

ERS publications

  • European Respiratory Journal
  • ERJ Open Research
  • European Respiratory Review
  • Breathe
  • ERS books online
  • ERS Bookshop

Help

  • Feedback

For authors

  • Instructions for authors
  • Publication ethics and malpractice
  • Submit a manuscript

For readers

  • Alerts
  • Subjects
  • RSS

Subscriptions

  • Accessing the ERS publications

Contact us

European Respiratory Society
442 Glossop Road
Sheffield S10 2PX
United Kingdom
Tel: +44 114 2672860
Email: journals@ersnet.org

ISSN

Online ISSN: 2312-0541

Copyright © 2023 by the European Respiratory Society