Rituximab in the Treatment of Shrinking Lung Syndrome in Systemic Lupus Erythematosus

Toribio, Patricia Peñacoba; Albani, María Emilia Córica; Pérez, Mercedes; Serna, Arturo Rodríguez de la

doi:10.1016/j.reumae.2013.09.003

Cited by 6 publications

(6 citation statements)

References 12 publications

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“…Most of the patients were treated with biological agents because the symptoms and PFT parameters did not improve after treatment with glucocorticoids and immunosuppressants (12/15). Two patients experienced relapse after glucocorticoid reduction [5,6], and one patient was treated with rituximab due to grade III glomerulonephritis [7]. Rituximab was most widely used drug in the treatment of SLE with SLS (12/15); except one patient in whom rituximab efficacy was poor because she developed anti-rituximab antibody [8] and one who had severe allergic rash after rituximab use [9], the other patients (10/12) experienced a significant curative effect with rituximab.…”

Section: Literature Reviewmentioning

confidence: 99%

"Tofacitinib Treatment in Refractory Systemic Lupus Erythematosus with Shrinking Lung Syndrome: A Case-Based Review"

Han

2024

BJSTR

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Shrinking Lung Syndrome (SLS) is a rare pulmonary complication of Systemic Lupus Erythematosus (SLE), which mainly manifests as dyspnea, pleural chest pain, and restrictive ventilation dysfunction, with or without an elevated hemi-diaphragm. Currently, there is no unified diagnostic criteria for SLS. SLS is a diagnosis of exclusion and can be diagnosed only in the absence of interstitial, alveolar and pulmonary vascular disease. The treatment is based on high-dose glucocorticoids and immunosuppressants. Here, we report a case of a 35-year-old woman who was diagnosed with SLE in 2008. Four years later, she was diagnosed with SLS due to chest pain, dyspnea, cough, and restrictive ventilatory dysfunction. Her condition improved after treatment with glucocorticoids and immunosuppressants but relapsed many times. She was treated with tofacitinib after relapse in May 2019, and then her condition improved and remains in remission with a four-year followup. We searched for and summarized 15 cases of patients with refractory SLE combined with SLS. They were all treated with biological agents. Tofacitinib is a new treatment choice for patients with refractory SLE with SLS.

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Section: Literature Reviewmentioning

confidence: 99%

"Tofacitinib Treatment in Refractory Systemic Lupus Erythematosus with Shrinking Lung Syndrome: A Case-Based Review"

Han

2024

BJSTR

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“…Theophylline and beta-agonists can help with diaphragmatic weakness [129][130][131]. Rituximab has also been used successfully as a monotherapy or with cyclophosphamide and beta-agonists, especially in steroidrefractory cases [81,132,133]. Other drugs have been used successfully after steroid failure; however, in a retrospective study by Robles-Perez et al, 6/18 patients reported adverse effects of rituximab [134].…”

Section: Treatmentmentioning

confidence: 99%

Systemic Lupus Erythematosus and Lung Involvement: A Comprehensive Review

Smıth

Lee

Park

et al. 2022

JCM

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Systemic lupus erythematosus (SLE) is a complex autoimmune disease with multiorgan manifestations, including pleuropulmonary involvement (20–90%). The precise mechanism of pleuropulmonary involvement in SLE is not well-understood; however, systemic type 1 interferons, circulating immune complexes, and neutrophils seem to play essential roles. There are eight types of pleuropulmonary involvement: lupus pleuritis, pleural effusion, acute lupus pneumonitis, shrinking lung syndrome, interstitial lung disease, diffuse alveolar hemorrhage (DAH), pulmonary arterial hypertension, and pulmonary embolism. DAH has a high mortality rate (68–75%). The diagnostic tools for pleuropulmonary involvement in SLE include chest X-ray (CXR), computed tomography (CT), pulmonary function tests (PFT), bronchoalveolar lavage, biopsy, technetium-99m hexamethylprophylene amine oxime perfusion scan, and (18)F-fluorodeoxyglucose positron emission tomography. An approach for detecting pleuropulmonary involvement in SLE includes high-resolution CT, CXR, and PFT. Little is known about specific therapies for pleuropulmonary involvement in SLE. However, immunosuppressive therapies such as corticosteroids and cyclophosphamide are generally used. Rituximab has also been successfully used in three of the eight pleuropulmonary involvement forms: lupus pleuritis, acute lupus pneumonitis, and shrinking lung syndrome. Pleuropulmonary manifestations are part of the clinical criteria for SLE diagnosis. However, no review article has focused on the involvement of pleuropulmonary disease in SLE. Therefore, this article summarizes the literature on the epidemiology, pathogenesis, diagnosis, and management of pleuropulmonary involvement in SLE.

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“…The most widely used drugs were azathioprine and cyclophosphamide, with variable success (10) . Further, RTX was used in severe or refractory SLS (9 patients), with effectiveness and good safety profile in all cases (14,15) . Based on this preliminary experience, RTX might emerge as the first-choice immunosuppressant, particularly in patients with severe or refractory disease, although larger-cohort studies must be performed in this field to confirm the efficacy of RTX in SLS.…”

Section: Case Presentationmentioning

confidence: 99%

“…Based on this preliminary experience, RTX might emerge as the first-choice immunosuppressant, particularly in patients with severe or refractory disease, although larger-cohort studies must be performed in this field to confirm the efficacy of RTX in SLS. Theophylline and betaagonists, alone or in combination with glucocorticoids, have also been suggested with the intent to increase diaphragmatic strength, but evidence of beneficial effects is lacking (15) . Recently, one case report showed good response to belimumab (16) .…”

Section: Case Presentationmentioning

confidence: 99%

Shrinking Lung Syndrome in Systemic Lupus Erythematosus

Osman,

Omar,

Elameen

et al. 2022

Minia Journal of Medical Research

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Shrinking lung syndrome (SLS) is a rare complication of systemic lupus erythematosus (SLE), however it was also reported with other connective tissue diseases (CTDs). It is characterized by its low prevalence with higher incidence in females patients. Although it is much more frequent in advanced stages of the disease, rarely, SLS may be the presenting manifestation of SLE. It was found that it mostly occurs in patients without previous or concomitant major organ involvement, moreover in inactive stages of the disease. Progressive exertional dyspnea accompanied by pleuritic chest pain are considered the dominant symptoms in SLS. Pulmonary function tests (PFTs) with reduced lung volumes and radiographic finding of elevated diaphragmatic copula are the characteristic features in SLS. There are no standardized guidelines for the treatment of SLS in SLE. However, the majority of patients showed good response on medium to high doses of glucocorticoids. Moreover, an immunosuppressive agent should be added if the patient fails to improve on steroids alone.

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Rituximab in the Treatment of Shrinking Lung Syndrome in Systemic Lupus Erythematosus

Cited by 6 publications

References 12 publications

"Tofacitinib Treatment in Refractory Systemic Lupus Erythematosus with Shrinking Lung Syndrome: A Case-Based Review"

"Tofacitinib Treatment in Refractory Systemic Lupus Erythematosus with Shrinking Lung Syndrome: A Case-Based Review"

Systemic Lupus Erythematosus and Lung Involvement: A Comprehensive Review

Shrinking Lung Syndrome in Systemic Lupus Erythematosus

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