Shrinking lung syndrome presenting as an initial pulmonary manifestation of SLE

Pillai, Sreevidya; Mehta, Jay B.; Levin, Terry L.; Muzumdar, Hiren; Nandalike, Kiran

doi:10.1177/0961203314536248

Cited by 21 publications

(15 citation statements)

References 13 publications

(15 reference statements)

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“…Although it is much more frequent in advanced stages of the disease, rarely, SLS may be the presenting manifestation of SLE. [43,48,57,66,69,76] This complication is more common in SLE patients of female sex, with a female-to-male ratio of 17:1.…”

Section: Discussionmentioning

confidence: 99%

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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Section: Discussionmentioning

confidence: 99%

Shrinking lung syndrome in systemic lupus erythematosus

et al. 2016

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“…Treatment remains empirical and includes steroids and/or immunosuppressants, intravenous immunoglobulins, biological factors, and rituximab [16]. Diaphragmatic muscle involvement and related weakness may present as ‘shrinking lungs and high sluggish diaphragm with clear lung fields' in lupus occurring mainly in patients without evidence of systemic myopathy [144,145,146,147,148]. In such a clinical context, hypercapnic respiratory failure has occasionally been reported [149].…”

Section: Acute Hypercapnic Respiratory Failure Related To Acute Ventimentioning

confidence: 99%

“…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

Respiration

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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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“…Currently, fewer than 100 cases of SLS in adults have been reported in the English literature 17. The lack of clinical suspicion and the disincentive to report milder cases may explain the scarcity of these cases.…”

Section: Discussionmentioning

confidence: 99%

Severe cognitive dysfunction and shrinking lung syndrome in systemic lupus erythematous

Barbosa¹,

Cardozo²,

Ferraz³

et al. 2014

ACR

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Systemic lupus erythematosus (SLE) is an autoimmune disease that can affect any organ or system. Neuropsychiatric and pulmonary involvement can occur in 40 and 50% of patients respectively, and may occur in several different clinical forms. While the main neuropsychiatric manifestations are represented by cognitive impairment, organic cerebral syndromes, delirium, psychosis, seizures, and peripheral neuropathies, the main forms of pulmonary involvement are pleurisy with or without pleural effusion, pneumonitis, interstitial disease, pulmonary hypertension, and alveolar hemorrhage. The authors report the case of a 49-year-old woman whose first manifestation of SLE was represented by two rare manifestations: rapidly progressive cognitive impairment, which was associated with respiratory failure caused by the shrinking lung syndrome. The authors call attention to the under-diagnosis of lupus pulmonary complications and its association with severe cognitive impairment that often necessitates aggressive treatment.

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Shrinking lung syndrome presenting as an initial pulmonary manifestation of SLE

Cited by 21 publications

References 13 publications

Shrinking lung syndrome in systemic lupus erythematosus

Shrinking lung syndrome in systemic lupus erythematosus

Acute Respiratory Events in Connective Tissue Disorders

Severe cognitive dysfunction and shrinking lung syndrome in systemic lupus erythematous

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