Rituximab en el tratamiento del síndrome del pulmón encogido del lupus eritematoso sistémico

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

doi:10.1016/j.reuma.2013.09.003

Cited by 12 publications

(4 citation statements)

References 12 publications

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“…Rituximab (RTX) is increasingly being used in severe or refractory cases of SLS (9 patients), with effectiveness and good safety profile. [22,52,54,59] …”

Section: Resultsmentioning

confidence: 99%

“…[43–77] Further, RTX is used in severe or refractory SLS (9 patients), with effectiveness and good safety profile in all cases. [22,52,54,59] 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 beta-agonists, alone or in combination with glucocorticoids, have also been suggested with the intent to increase diaphragmatic strength, but evidence of beneficial effects is lacking.…”

Section: Discussionmentioning

confidence: 99%

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Shrinking lung syndrome in systemic lupus erythematosus

et al. 2016

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Shrinking lung syndrome (SLS) is a rare and less known complication mainly associated with systemic lupus erythematosus (SLE). In this study, we analyze the clinical features, investigation findings, approaches to management, and outcome in a case series of 9 adult patients with SLE and SLS diagnosed during a 35-year period in 3 referral tertiary care hospitals in Spain. Additionally, we reviewed 80 additional cases previously reported (PubMed 1965–2015). These 80 cases, together with our 9 patients, form the basis of the present analysis.The overall SLS prevalence in our SLE population was 1.1% (9/829). SLS may complicate SLE at any time over its course, and it usually occurs in patients without previous or concomitant major organ involvement. More than half of the patients had inactive lupus according to SELENA-systemic lupus erythematosus disease activity index (SLEDAI) scores. Typically, it presents with progressive exertional dyspnea of variable severity, accompanied by pleuritic chest pain in 76% of the cases.An important diagnostic delay is common. The diagnostic tools that showed better yield for SLS detection are the imaging techniques (chest x-ray and high-resolution computed tomography) along with pulmonary and diaphragmatic function tests. Evaluation of diaphragm dome motion by M-mode ultrasonography and phrenic nerve conduction studies are less useful.There are no standardized guidelines for the treatment of SLS in SLE. The majority of patients were treated with medium or high doses of glucocorticoids. Several immunosuppressive agents have been used in conjunction with steroids either if the patient fails to improve or since the beginning of the treatment. Theophylline and beta-agonists, alone or in combination with glucocorticoids, have been suggested with the intent to increase diaphragmatic strength.The overall long-term prognosis was good. The great majority of patients had significant clinical improvement and stabilization, or mild to moderate improvement on pulmonary function tests. The mortality rate was very low.

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“…Rituximab (RTX) is increasingly being used in severe or refractory cases of SLS (9 patients), with effectiveness and good safety profile. [22,52,54,59] …”

Section: Resultsmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Shrinking lung syndrome in systemic lupus erythematosus

et al. 2016

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“…The etiology remains unclear, although in some patients a diaphragmatic inflammatory myopathy has been documented histologically through biopsy [150,151,152]. The therapeutic approach includes steroids and immunosuppressants [146,147,153]. …”

Section: Acute Hypercapnic Respiratory Failure Related To Acute Ventimentioning

confidence: 99%

Acute Respiratory Events in Connective Tissue Disorders

Papiris¹,

Manali²,

Kolilekas³

et al. 2016

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Subacute-acute, hyperacute, or even catastrophic and fulminant respiratory events occur in almost all classic connective tissue disorders (CTDs); they may share systemic life-threatening manifestations, may precipitously lead to respiratory failure requiring ventilatory support as well as a combination of specific therapeutic measures, and in most affected patients constitute the devastating end-of-life event. In CTDs, acute respiratory events may be related to any respiratory compartment including the airways, lung parenchyma, alveolar capillaries, lung vessels, pleura, and ventilatory muscles. Acute respiratory events may also precipitate disease-specific extrapulmonary organ involvement such as aspiration pneumonia and lead to digestive tract involvement and heart-related respiratory events. Finally, antirheumatic drug-related acute respiratory toxicity as well as lung infections related to the rheumatic disease and/or to immunosuppression complete the spectrum of acute respiratory events. Overall, in CTDs the lungs significantly contribute to morbidity and mortality, since they constitute a common site of disease involvement; a major site of infections related to the ‘mater' disease; a major site of drug-related toxicity, and a common site of treatment-related infectious complications. The extreme spectrum of the abovementioned events, as well as the ‘vicious' coexistence of most of the aforementioned manifestations, requires skills, specific diagnostic and therapeutic means, and most of all a multidisciplinary approach of adequately prepared and expert scientists. Avoiding lung disease might represent a major concern for future advancements in the treatment of autoimmune disorders.

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“…β 2 -Agonists, theophylline and even a surgical approach have also been used to increase diaphragmatic strength. In refractory cases, treatment with rituximab has shown promising results [76,77,78,79]. …”

Section: Systemic Lupus Erythematosusmentioning

confidence: 99%

Treatment Considerations of Lung Involvement in Rheumatologic Disease

Robles-Pérez

Molina-Molina²

2015

Respiration

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Lung involvement in rheumatologic diseases has a broad spectrum of clinical and radiological presentations, from acute and inflammatory predominance to chronic and fibrotic lung disorders. Therapy has to be guided by the type of rheumatologic disease, the kind of lung involvement and the risk associated with the clinical state of the patient and prognostic factors. Although several therapeutic approaches have been used, the best treatment is still not certain; clinical trials are mandatory, which would require a better knowledge of the pulmonary pathogenesis of immunologic diseases. The following review is focused on the therapeutic options for those rheumatologic disorders that could present as a predominant interstitial lung disease which may confer a bad or life-threatening prognosis.

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Rituximab en el tratamiento del síndrome del pulmón encogido del lupus eritematoso sistémico

Cited by 12 publications

References 12 publications

Shrinking lung syndrome in systemic lupus erythematosus

Shrinking lung syndrome in systemic lupus erythematosus

Acute Respiratory Events in Connective Tissue Disorders

Treatment Considerations of Lung Involvement in Rheumatologic Disease

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