Gianluca Sambataro1, Francesco Ferro2, Martina Orlandi3, Domenico Sambataro4, Sebastiano Emanuele Torrisi5, Luca Quartuccio6, Carlo Vancheri5, Chiara Baldini2, Marco Matucci Cerinic3. 1. 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. 2. Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, 56126, Italy. 3. Department of Experimental and Clinical Medicine, Division of Rheumatology AOUC, University of Florence, Italy. 4. 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. 5. Regional Referral Centre for Rare Lung Diseases, A. O. U. "Policlinico-Vittorio Emanuele" Dept. of Clinical and Experimental Medicine, University of Catania, Italy. 6. Department of Medical and Biological Sciences, Rheumatology Clinic, University of Udine, Italy.
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.
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 ILDpatients 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: Domenico Sambataro; Gianluca Sambataro; Francesca Pignataro; Giovanni Zanframundo; Veronica Codullo; Evelina Fagone; Emanuele Martorana; Francesco Ferro; Martina Orlandi; Nicoletta Del Papa; Lorenzo Cavagna; Lorenzo Malatino; Michele Colaci; Carlo Vancheri Journal: Diagnostics (Basel) Date: 2020-04-09
Authors: Luca Quartuccio; Ginevra De Marchi; Simone Longhino; Valeria Manfrè; Maria Teresa Rizzo; Saviana Gandolfo; Alberto Tommasini; Salvatore De Vita; Robert Fox Journal: Front Immunol Date: 2021-07-12 Impact factor: 7.561