Shrinking Lung in Primary Sjogrën Syndrome Successfully Treated With Rituximab

Pérez, José Jesús Blanco; Pérez-González, Alexandre; Vales, José Luis Guerra; González, Rosendo Bugarín; Pego-Reigosa, José María

doi:10.1016/j.arbr.2014.11.027

Cited by 9 publications

(19 citation statements)

References 5 publications

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“…While SLS has mainly been described in SLE, it has also been reported in association with other connective tissue diseases such as Sjo¨gren's syndrome, scleroderma and rheumatoid arthritis. [14][15][16][17] Its prevalence in lupus patients ranges from 0.5% to 1.1%, 18,19 lower than our estimation of 1.53%. The reasons behind this discrepancy are not clear; however, it is possible that SLS is misdiagnosed in SLE.…”

Section: Discussioncontrasting

confidence: 68%

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Shrinking lung syndrome in systemic lupus erythematosus: a single-centre experience

Deeb

Tselios

Gladman

et al. 2017

Lupus

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Introduction Shrinking lung syndrome (SLS) is a rare manifestation of systemic lupus erythematosus (SLE), characterized by decreased lung volumes and extra-pulmonary restriction. The aim of this study was to describe the characteristics of SLS in our lupus cohort with emphasis on prevalence, presentation, treatment and outcomes. Patients and methods Patients attending the Toronto Lupus Clinic since 1980 ( n = 1439) and who had pulmonary function tests (PFTs) performed during follow-up were enrolled ( n = 278). PFT records were reviewed to characterize the pattern of pulmonary disease. SLS definition was based on a restrictive ventilatory defect with normal or slightly reduced corrected diffusing lung capacity for carbon monoxide (DLCO) in the presence of suggestive clinical (dyspnea, chest pain) and radiological (elevated diaphragm) manifestations. Data on clinical symptoms, functional abnormalities, imaging, treatment and outcomes were extracted in a dedicated data retrieval form. Results Twenty-two patients (20 females) were identified with SLS for a prevalence of 1.53%. Their mean age was 29.5 ± 13.3 years at SLE and 35.7 ± 14.6 years at SLS diagnosis. Main clinical manifestations included dyspnea (21/22, 95.5%) and pleuritic chest pain (20/22, 90.9%). PFTs were available in 20 patients; 16 (80%) had decreased maximal inspiratory (MIP) and/or expiratory pressure (MEP). Elevated hemidiaphragm was demonstrated in 12 patients (60%). Treatment with prednisone and/or immunosuppressives led to clinical improvement in 19/20 cases (95%), while spirometrical improvement was observed in 14/16 patients and was mostly partial. Conclusions SLS prevalence in SLE was 1.53%. Treatment with glucocorticosteroids and immunosuppressives was generally effective. However, a chronic restrictive ventilatory defect usually persisted.

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Section: Discussioncontrasting

confidence: 68%

“…While SLS has mainly been described in SLE, it has also been reported in association with other connective tissue diseases such as Sjögren’s syndrome, scleroderma and rheumatoid arthritis. 14–17…”

Section: Discussionmentioning

confidence: 99%

Shrinking lung syndrome in systemic lupus erythematosus: a single-centre experience

Deeb

Tselios

Gladman

et al. 2017

Lupus

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“…pSS patients with shrink lung syndrome respond well to large doses of glucocorticoids combined with cyclophosphamide or azathioprine or rituximab therapy according to previous studies. [3][4][5][6][7][8] In our case, the patient's initial and major symptom was dyspnea.…”

Section: Discussionmentioning

confidence: 58%

Primary Sjögren's syndrome with type II respiratory failure caused by myotonic dystrophy type 1: A case report and literature review

Jiang

Zhou

et al. 2022

Int J of Rheum Dis

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Primary Sjögren's syndrome (pSS) is an autoimmune disease characterized by the involvement of exocrine glands with dryness of mouth and eyes as typical clinical manifestations, and may also have extra‐glandular organ involvement. Myotonic dystrophy type 1 (DM1) is the most common type of adult‐onset muscular dystrophy, which can lead to progressive muscle weakness and myotonia. The coexistence of pSS with DM1 was rarely reported. We here report a Chinese female pSS patient with progressive type II respiratory failure as a major manifestation and finally diagnosed with DM1 by genetic testing.

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“…Among the therapy, azathioprine [79,216] and mycophenolate mofetil [217,218] have been reported. Biologic therapy, such as rituximab, a humanized monoclonal antibody targeting mature B-cells (CD20+), was tried in case series and reports with promising results [219], in reparatory cases [220] and also associated with lymphoproliferative diseases [221][222][223], in special cases, such as in shrinking lung syndrome associated with SS [224]. Off-the-label cases reported other biologicals, such as tocilizumab [225] and abatacept [226].…”

Section: Brief Historymentioning

confidence: 99%

Perspective Chapter: Pulmonary System and Sjogren’s Syndrome

Ehtesham¹,

Tiwari²,

George³

et al. 2022

Idiopathic Pulmonary Fibrosis

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Sjogren’s syndrome (SS) is a connective tissue disease targeting the exocrine glands with subsequent sicca symptoms mainly in eyes and mouth. Respiratory symptoms may be the most frequent extraglandular manifestation following fatigue and pain. Mucosal dysfunction may affect the upper and lower airways, being the small airways more frequently involved. Parenchymal disease carries most of the morbidity and mortality. Nonspecific interstitial pneumonia (NSIP) is the most common radiographic feature, whereas the fibrotic NSIP type is the most reported finding in biopsies. Pulmonary lymphoma may arise from bronchial-associated lymphoid tissue lesions, and although rare, it is prevalent in SS. Chronic hypertrophic bronchial wall changes may ascribe to the various cystic lesions. Under their presence, possible lymphocytic interstitial pneumonia, amyloidosis, and lymphoma should be explored. Pulmonary arterial hypertension may present as frequently as in lupus, especially in Asian populations. Advanced knowledge in the pathogenesis has helped in understanding the various presentations within the respiratory system, contrasting with the scarce therapeutic options to treat both the airway and parenchymal disease. Anti-fibrotic parenchymal lung therapy offers promising outcomes. The pulmonary involvement in SS may associate with a decline in quality of life and reduced life expectancy. Subsequently, clinicians should know these facts for a timely intervention.

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Shrinking Lung in Primary Sjogrën Syndrome Successfully Treated With Rituximab

Cited by 9 publications

References 5 publications

Shrinking lung syndrome in systemic lupus erythematosus: a single-centre experience

Shrinking lung syndrome in systemic lupus erythematosus: a single-centre experience

Primary Sjögren's syndrome with type II respiratory failure caused by myotonic dystrophy type 1: A case report and literature review

Perspective Chapter: Pulmonary System and Sjogren’s Syndrome

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