Diagnosis and management of lung involvement in systemic lupus erythematosus and Sjögren’s syndrome: a literature review:
Roberto Depascale,Giulia Del Frate,Michela Gasparotto,Valeria Manfrè,Mariele Gatto,Luca Iaccarino,Luca Quartuccio,Salvatore De Vita,Andrea Doria +8 more
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TLDR
In this article, a comprehensive overview of lung manifestations in systemic lupus erythematosus (SLE) and primary Sjogren's syndrome (pSS) has been outlined with a multiplicity of different manifestations.Abstract:
Lung involvement in systemic lupus erythematosus (SLE) and primary Sjogren's syndrome (pSS) has extensively been outlined with a multiplicity of different manifestations. In SLE, the most frequent finding is pleural effusion, while in pSS, airway disease and parenchymal disorders prevail. In both cases, there is an increased risk of pre-capillary and post-capillary pulmonary arterial hypertension (PAH) and pulmonary venous thromboembolism (VTE). The risk of VTE is in part due to an increased thrombophilic status secondary to systemic inflammation or to the well-established association with antiphospholipid antibody syndrome (APS). The lung can also be the site of an organ-specific complication due to the aberrant pathologic immune-hyperactivation as occurs in the development of lymphoma or amyloidosis in pSS. Respiratory infections are a major issue to be addressed when approaching the differential diagnosis, and their exclusion is required to safely start an immunosuppressive therapy. Treatment strategy is mainly based on glucocorticoids (GCs) and immunosuppressants, with a variable response according to the primary pathologic process. Anticoagulation is recommended in case of VTE and multi-targeted treatment regimens including different drugs are the mainstay for PAH management. Antibiotics and respiratory physiotherapy can be considered relevant complement therapeutic measures. In this article, we reviewed lung manifestations in SLE and pSS with the aim to provide a comprehensive overview of their diagnosis and management to physicians taking care of patients with connective tissue diseases.read more
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Thoracic Involvement in Systemic Autoimmune Rheumatic Diseases: Pathogenesis and Management
Elena De Zorzi,Paolo Spagnolo,Elisabetta Cocconcelli,Elisabetta Balestro,Luca Iaccarino,Mariele Gatto,Francesco Benvenuti,Nicol Bernardinello,Andrea Doria,Toby M. Maher,Elisabetta Zanatta +10 more
TL;DR: In this article , the authors summarized and critically discussed the current knowledge on thoracic involvement in ARDs, with emphasis on disease pathogenesis and management, and provided a thorough, multidisciplinary assessment to determine disease activity and degree of impairment is required to optimize patient management.
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Chest high-resolution computed tomography in primary Sjögren's syndrome: an up-to-date primer for rheumatologists.
Lorenzo Cereser,Ivan Giovannini,Guido Caronia,Alen Zabotti,Salvatore De Vita,Chiara Zuiani,Luca Quartuccio,Rossano Girometti +7 more
TL;DR: This review aims to provide a primer for rheumatologists on chest HRCT, illustrating the up-to-date technique, imaging findings, and clinical indications in pSS and highlighting the importance ofrheumatologist-radiologist constructive collaboration in the clinical management of such patients.
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Dépistage des manifestations pulmonaires des connectivites
Pierre-Antoine Juge,Raphael Borie,Marie-Pierre Debray,Aurélie Cazes,Catherine Bancal,Bruno Crestani,Philippe Dieudé +6 more
TL;DR: In this paper , the authors give the recent data that help to discuss and argue the necessity of screening, notably in high-risk patients, in high risk patients, for pulmonary manifestations associated with connective tissue diseases.
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Systemic Lupus Erythematosus and Pulmonary Hypertension
Konstantinos Parperis,Nikolaos Velidakis,Elina Khattab,Evangelia Gkougkoudi,Nikolaos P.E. Kadoglou +4 more
TL;DR: In this paper , the authors proposed an early diagnosis of Systemic Lupus Erythematosus (SLE) related pulmonary hypertension and identification of the underlying pathogenetic mechanisms, in order to prevent irreversible pulmonary vascular damage.
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Presentation of Sjogren Syndrome as Nodular Pulmonary Amyloidosis
TL;DR: Pulmonary nodular amyloidosis is presented as a rare presentation of Sjogren syndrome in the setting of relatively well-controlled symptoms and should prompt evaluation of associated conditions such as malignancy and autoimmune disorders to guide further management.
References
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2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT).
Nazzareno Galiè,Marc Humbert,Jean-Luc Vachiery,Simon Gibbs,Irene Lang,Adam Torbicki,Gérald Simonneau,Andrew Peacock,Anton Vonk Noordegraaf,Maurice Beghetti,Ardeschir Ghofrani,Miguel Angel Gomez Sanchez,Georg Hansmann,Walter Klepetko,Patrizio Lancellotti,Marco Matucci,Theresa McDonagh,Luc Pierard,Pedro T. Trindade,Maurizio Zompatori,Marius M. Hoeper +20 more
TL;DR: In this paper, the authors defined the following terms: ALAT, alanine aminotransferase, ASAT, aspartate AMINOTE, and APAH, associated pulmonary arterial hypertension.
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Nintedanib in Progressive Fibrosing Interstitial Lung Diseases
TL;DR: In patients with progressive fibrosing interstitial lung diseases, the annual rate of decline in the FVC was significantly lower among patients who received nintedanib than among those who received placebo.
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2019 update of the EULAR recommendations for the management of systemic lupus erythematosus
Antonis Fanouriakis,Myrto Kostopoulou,Alessia Alunno,Martin Aringer,Ingeborg M. Bajema,John Boletis,Ricard Cervera,Andrea Doria,Caroline Gordon,Marcello Govoni,Frédéric Houssiau,David Jayne,Marios Kouloumas,Annegret Kuhn,Janni Lisander Larsen,Kirsten Lerstrøm,Gabriella Moroni,Marta Mosca,Matthias Schneider,Josef S Smolen,Elisabet Svenungsson,Vladimir Tesar,Angela Tincani,Anne Troldborg,Ronald F van Vollenhoven,Jörg Wenzel,George Bertsias,Dimitrios T. Boumpas +27 more
TL;DR: The updated recommendations provide physicians and patients with updated consensus guidance on the management of SLE, combining evidence-base and expert-opinion, based on emerging new evidence.
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Increased Risk of Lymphoma in Sicca Syndrome
Stuart S. Kassan,Terry L. Thomas,Haralampos M. Moutsopoulos,Robert Hoover,Robert P. Kimberly,Daniel R. Budman,Jose Costa,John L. Decker,Thomas M. Chused +8 more
TL;DR: Patients with a history of parotid enlargement, splenomegaly, and lymphadenopahy had an increased risk of lymphoma and clinical conditions did not appear to be early manifestations of undiagnosed lymphoma but rather seemed to identify a subgroup of patients with sicca syndrome with marked lymphoid reactivity, who had a particularly high risk of subsequently developing lymphoma.
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Initial Use of Ambrisentan plus Tadalafil in Pulmonary Arterial Hypertension
Nazzareno Galiè,Joan Albert Barberà,Adaani E. Frost,Hossein Ardeschir Ghofrani,Marius M. Hoeper,Vallerie V. McLaughlin,Andrew J. Peacock,Gérald Simonneau,Jean-Luc Vachiery,Ekkehard Grünig,Ronald J. Oudiz,Anton Vonk-Noordegraaf,Rj James White,Christiana Blair,Hunter Gillies,Karen L. Miller,Julia Harris,Jonathan Langley,Lewis J. Rubin +18 more
TL;DR: Among participants with pulmonary arterial hypertension who had not received previous treatment, initial combination therapy with ambrisentan and tadalafil resulted in a significantly lower risk of clinical-failure events than the risk with am debrisentan or tadalAFil monotherapy.