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Clinical, morphological features and prognostic factors associated with interstitial lung disease in primary Sjӧgren's syndrome: A systematic review from the Italian Society of Rheumatology.
Autoimmun Rev. 2020 Feb; 19(2):102447.AR

Abstract

OBJECTIVE

To evaluate the prevalence, clinical presentation, serological and morphological features of, and therapeutic options for Interstitial Lung Disease (ILD) in primary Sjögren's Syndrome (pSS).

METHODS

Pubmed was searched between February 1996 and December 2018 using a combination of MESH terms related to pSS and ILD. Selected works were subjected to blind evaluation by two authors and a senior author in case of disagreement. The work followed PRISMA guidelines and was registered on PROSPERO (CRD42018118669).

RESULTS

About 20% of pSS patients have ILD, with a 5-y survival of 84% and a need for supplemental oxygen in the 11-33% range. A significant proportion of ILD patients are seronegative without sicca syndrome. ILD seems to be associated with higher levels of Lactic Dehydrogenases and positivity for Anti-Ro52k. The prevalent pattern in High Resolution Computed Tomography is Nonspecific Interstitial Pneumonia (NSIP), but all other patterns can be present. No difference in mortality was found between patients with NSIP and Usual Interstitial Pneumonia patterns. Amyloidosis and primary lung lymphoma can be observed in about 10% of pSS patients.

CONCLUSION

The recognition of pSS underlying an ILD can be challenging in seronegative patients with no or mild sicca symptoms. A complete diagnostic assessment, including minor salivary glands and, in some cases, lung biopsy, should be performed on all patients at risk. A better recognition of the clinical or serological markers of ILD progression in these patients is warranted to drive the physicians to an early diagnosis and an effective treatment.

Authors+Show Affiliations

Artroreuma S.R.L., Rheumatology Outpatient Clinic Accredited with the Italian National Health System, Corso S. Vito 53, 95030 Mascalucia, (CT), Italy; Regional Referral Centre for Rare Lung Diseases, A. O. U. "Policlinico-Vittorio Emanuele" Dept. of Clinical and Experimental Medicine, University of Catania, Italy. Electronic address: dottorsambataro@gmail.com.Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, 56126, Italy.Department of Experimental and Clinical Medicine, Division of Rheumatology AOUC, University of Florence, Italy.Artroreuma S.R.L., Rheumatology Outpatient Clinic Accredited with the Italian National Health System, Corso S. Vito 53, 95030 Mascalucia, (CT), Italy; Department of Clinical and Experimental Medicine, Rheumatology Unit, Cannizzaro Hospital, Via Messina 200, Catania 95100, Italy.Regional Referral Centre for Rare Lung Diseases, A. O. U. "Policlinico-Vittorio Emanuele" Dept. of Clinical and Experimental Medicine, University of Catania, Italy.Department of Medical and Biological Sciences, Rheumatology Clinic, University of Udine, Italy.Regional Referral Centre for Rare Lung Diseases, A. O. U. "Policlinico-Vittorio Emanuele" Dept. of Clinical and Experimental Medicine, University of Catania, Italy.Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, 56126, Italy.Department of Experimental and Clinical Medicine, Division of Rheumatology AOUC, University of Florence, Italy.

Pub Type(s)

Letter
Systematic Review

Language

eng

PubMed ID

31843713

Citation

Sambataro, Gianluca, et al. "Clinical, Morphological Features and Prognostic Factors Associated With Interstitial Lung Disease in Primary Sjӧgren's Syndrome: a Systematic Review From the Italian Society of Rheumatology." Autoimmunity Reviews, vol. 19, no. 2, 2020, p. 102447.
Sambataro G, Ferro F, Orlandi M, et al. Clinical, morphological features and prognostic factors associated with interstitial lung disease in primary Sjӧgren's syndrome: A systematic review from the Italian Society of Rheumatology. Autoimmun Rev. 2020;19(2):102447.
Sambataro, G., Ferro, F., Orlandi, M., Sambataro, D., Torrisi, S. E., Quartuccio, L., Vancheri, C., Baldini, C., & Matucci Cerinic, M. (2020). Clinical, morphological features and prognostic factors associated with interstitial lung disease in primary Sjӧgren's syndrome: A systematic review from the Italian Society of Rheumatology. Autoimmunity Reviews, 19(2), 102447. https://doi.org/10.1016/j.autrev.2019.102447
Sambataro G, et al. Clinical, Morphological Features and Prognostic Factors Associated With Interstitial Lung Disease in Primary Sjӧgren's Syndrome: a Systematic Review From the Italian Society of Rheumatology. Autoimmun Rev. 2020;19(2):102447. PubMed PMID: 31843713.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Clinical, morphological features and prognostic factors associated with interstitial lung disease in primary Sjӧgren's syndrome: A systematic review from the Italian Society of Rheumatology. AU - Sambataro,Gianluca, AU - Ferro,Francesco, AU - Orlandi,Martina, AU - Sambataro,Domenico, AU - Torrisi,Sebastiano Emanuele, AU - Quartuccio,Luca, AU - Vancheri,Carlo, AU - Baldini,Chiara, AU - Matucci Cerinic,Marco, Y1 - 2019/12/14/ PY - 2019/09/07/received PY - 2019/09/13/accepted PY - 2019/12/18/pubmed PY - 2020/2/29/medline PY - 2019/12/18/entrez KW - Anti-Ro KW - Connective tissue disease KW - High resolution computed tomography KW - Interstitial lung disease KW - Interstitial pneumonia with autoimmune features KW - Mortality KW - Prognostic factors KW - Sicca syndrome KW - Sjögren's syndrome KW - Systematic review SP - 102447 EP - 102447 JF - Autoimmunity reviews JO - Autoimmun Rev VL - 19 IS - 2 N2 - OBJECTIVE: To evaluate the prevalence, clinical presentation, serological and morphological features of, and therapeutic options for Interstitial Lung Disease (ILD) in primary Sjögren's Syndrome (pSS). METHODS: Pubmed was searched between February 1996 and December 2018 using a combination of MESH terms related to pSS and ILD. Selected works were subjected to blind evaluation by two authors and a senior author in case of disagreement. The work followed PRISMA guidelines and was registered on PROSPERO (CRD42018118669). RESULTS: About 20% of pSS patients have ILD, with a 5-y survival of 84% and a need for supplemental oxygen in the 11-33% range. A significant proportion of ILD patients are seronegative without sicca syndrome. ILD seems to be associated with higher levels of Lactic Dehydrogenases and positivity for Anti-Ro52k. The prevalent pattern in High Resolution Computed Tomography is Nonspecific Interstitial Pneumonia (NSIP), but all other patterns can be present. No difference in mortality was found between patients with NSIP and Usual Interstitial Pneumonia patterns. Amyloidosis and primary lung lymphoma can be observed in about 10% of pSS patients. CONCLUSION: The recognition of pSS underlying an ILD can be challenging in seronegative patients with no or mild sicca symptoms. A complete diagnostic assessment, including minor salivary glands and, in some cases, lung biopsy, should be performed on all patients at risk. A better recognition of the clinical or serological markers of ILD progression in these patients is warranted to drive the physicians to an early diagnosis and an effective treatment. SN - 1873-0183 UR - https://www.unboundmedicine.com/medline/citation/31843713/Clinical_morphological_features_and_prognostic_factors_associated_with_interstitial_lung_disease_in_primary_Sjӧgren's_syndrome:_A_systematic_review_from_the_Italian_Society_of_Rheumatology_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S1568-9972(19)30260-5 DB - PRIME DP - Unbound Medicine ER -