Shrinking lung syndrome and systemic lupus erythematosus: a case series and literature review

Smyth, H.; Flood, Rachael; Kane, David; Donnelly, Seamas C.; Mullan, Ronan

doi:10.1093/qjmed/hcx204

Cited by 17 publications

(19 citation statements)

References 19 publications

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“…Majority of the cases are associated with dyspnea, decrease in lung volume with restrictive pattern, and elevation of hemidiaphragm. The greatest challenge in the diagnosis of SLS is the absence of other pulmonary pathologies such as interstitial, alveolar, or pulmonary disease [1,6]. Our patient had the long-standing history of SLE with multiple co-morbidities including medical noncompliance and poor follow up.…”

Section: Discussionmentioning

confidence: 98%

“…Shrinking lung syndrome (SLS) is a rare pulmonary complication of systemic lupus erythematosus (SLE). It is characterized by progressive dyspnea, elevation of diaphragm, pleuritic chest pain, decreased lung volumes on imaging, and restrictive pattern seen in the pulmonary function tests (PFTs) [1]. Prevalence of SLS is estimated to be between 0.5% and 1.1% among patients with SLE [2].…”

Section: Introductionmentioning

confidence: 99%

“…Its pathophysiology remains largely unclear; however, hypotheses have been suggested ranging from microatelectatic changes due to lack of surfactant and increased surface tension, diaphragm fibrosis, and phrenic nerve palsy [3]. There is no standardized treatment of SLS, though most of these patients are treated with medium to high dose steroids [1]. Monoclonal antibodies to B-lymphocyte antigen CD (cluster of differentiation) 20 are also being studied as a possible treatment [4].…”

Section: Introductionmentioning

confidence: 99%

“…Monoclonal antibodies to B-lymphocyte antigen CD (cluster of differentiation) 20 are also being studied as a possible treatment [4]. Despite lack of standardized and available therapy, the overall mortality seems to be low [1].…”

Section: Introductionmentioning

confidence: 99%

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Shrinking Lung Syndrome: A Rare Manifestation of Systemic Lupus Erythematosus

Choudhury¹,

Ramos²,

Anjum³

et al. 2020

Cureus

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Shrinking lung syndrome (SLS) is a pulmonary complication of systemic lupus erythematosus (SLE) characterized by dyspnea, pleuritic chest pain, and progressive decrease in lung volumes with no evidence of pleural or interstitial disease on chest CT. We present a 51-year-old female with a 14-year history of SLE with symptoms of progressive shortness of breath, pleuritic chest pains, low grade fevers, and productive cough which was unresponsive to multiple courses of antibiotics. After careful review of her course of SLE and timeline of symptoms, she was diagnosed with SLS. Even though rare, clinicians should have a high suspicion of SLS in patients with a long-term history of SLE and worsening dyspnea. Early treatment can be initiated to help reduce long-term morbidity and mortality and maintain the quality of life.

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Section: Discussionmentioning

confidence: 98%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Shrinking Lung Syndrome: A Rare Manifestation of Systemic Lupus Erythematosus

Choudhury¹,

Ramos²,

Anjum³

et al. 2020

Cureus

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“…Shrinking lung syndrome (SLS) is an uncommon but well-recognized complication of SLE and other autoimmune diseases 97 such as SSc, pSS, and rheumatoid arthritis (RA). 98 SLS affects from 0.5% to 1.53% of all lupus patients, 99 in most cases 4 to 5 years after the initial diagnosis.…”

Section: Pulmonary Involvement In Slementioning

confidence: 99%

Diagnosis and management of lung involvement in systemic lupus erythematosus and Sjögren’s syndrome: a literature review

Depascale

Frate

Gasparotto

et al. 2021

Therapeutic Advances in Musculoskeletal

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Lung involvement in systemic lupus erythematosus (SLE) and primary Sjögren’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.

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Lupus Erythematosus and Antiphospholipid Syndrome

Dutz,

Khosravi‐Hafshejani

2024

Rook's Textbook of Dermatology

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Lupus erythematosus is an autoimmune disease that commonly affects the skin, and may present with cutaneous only disease. This chapter reviews the forms of cutaneous lupus‐specific skin disease (discoid lupus, subacute cutaneous lupus, lupus tumidus and acute cutaneous lupus), their clinical features, pathogenesis and treatment. A review of systemic lupus erythematosus is also provided with a discussion of diagnosis, clinical features, cutaneous aspects and treatment, germane for the dermatologist. Clinical features of neonatal lupus erythematosus and the antiphospholipid antibody syndrome, more commonly seen in systemic lupus erythematosus kindreds, are also discussed.

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Shrinking lung syndrome and systemic lupus erythematosus: a case series and literature review

Cited by 17 publications

References 19 publications

Shrinking Lung Syndrome: A Rare Manifestation of Systemic Lupus Erythematosus

Shrinking Lung Syndrome: A Rare Manifestation of Systemic Lupus Erythematosus

Diagnosis and management of lung involvement in systemic lupus erythematosus and Sjögren’s syndrome: a literature review

Lupus Erythematosus and Antiphospholipid Syndrome

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