Teaching Case

Section

Chest imaging

Case Type

Clinical Cases

Authors

Shreyas Reddy K¹, Anna Rachel Menezes², Jayanti Musib¹

1. Department of Radiology, St. John’s Medical College, Bangalore, India

2. Resident Medical Officer, St. Ann’s Hospital, Elathagiri, India

Patient

48 years, female

Categories

Area of Interest Thorax ; Imaging Technique CT, CT-High Resolution, Ultrasound, Ultrasound-Colour Doppler ;

No Procedure ; No Special Focus ;

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Clinical History

A 48-year-old female, on irregular follow-up for systemic lupus erythematosus (SLE), came to the pulmonary medicine outpatient department with a 2-year history of easy fatiguability and exertional dyspnea progressing to current dyspnea at rest. She had no history of orthopnea or paroxysmal nocturnal dyspnea.

Differential Diagnosis List

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Shrinking lung syndrome

Phrenic nerve palsy

Diaphragmatic eventration

Pulmonary hypoplasia

Subpulmonic effusion

Neuromuscular disorders involving the chest wall

Imaging Findings

Chest X-Ray (CXR) in posteroanterior view revealed an elevated right hemidiaphragm with reduced lung volume on the right. No obvious lung parenchymal, cardiac, and mediastinal abnormalities were noted.

On further evaluation with high-resolution computed tomography (HRCT) of the chest, conclusive evidence of an elevated right hemidiaphragm and significant volume loss in the right middle and lower lobes with subsegmental atelectasis were seen. No features of parenchymal/pleural/vascular disease were noted.

Additionally, an M mode ultrasound of the chest was performed, demonstrating a paradoxical movement of the right hemidiaphragm on inspiration.

Discussion

Shrinking Lung Syndrome (SLS) is a rare diagnosis of exclusion, usually occurring in female patients, after a mean duration of 4-6.5 years from the diagnosis of SLE.[1,2] It was first described by Hoffman and Beck in 1965 and is characterized by unexplained dyspnea and a decrease in lung volume, with or without a hemidiaphragm elevation, in the absence of implicit alveolar or interstitial disease. [3] Currently, a combination of pathophysiological mechanisms including pleural inflammation and chronic hypoventilation due to SLE-associated neuro-myopathy have been attributed to SLS. [4–6]

As with our case, SLS often presents as exertional dyspnea progressing over months (98.87%) [2,7] and pleuritic chest pain (76%).[2] It may also rarely present as fever (19%) or dry cough (8%).[2] Features of SLS occur independently of the course, severity, and biomarkers of SLE and are non-collateral with features of other major organ involvement.[8] Examination usually reveals tachypnea with the use of accessory respiratory muscles and bilateral shrinking of lung borders without characteristic auscultatory findings apart from rare basal crepitations.[9] Sequential pulmonary function testing shows gradually decreasing lung volumes in a restrictive pattern.[2]

The moderately slow progression of SLS not only increases its morbidity but also contributes to a delay in diagnosis, (11 +/- 20 months) leading to possible drastic complications including respiratory arrest.[2,10] Findings of an elevated hemidiaphragm on Chest X-ray and/or CT findings of diaphragmatic elevation, pleuritis +/- basal laminar atelectasis without features of interstitial pneumonitis in a female patient with chronic SLE, should prompt a radiologist to report a possible diagnosis of SLS.[4,7,11,12] Additional evidence of paradoxical diaphragmatic movement can also be confirmed on M-mode ultrasonography.[13]

In our case report, a middle-aged female patient, presented with gradual onset, progressive dyspnea for the past 2 years, 8 years after a diagnosis of SLE. Her pulmonary function tests showed a restrictive pattern. CXR revealed an elevated right hemidiaphragm with reduced lung volume on the right when compared to a previous CXR performed 2 years before her presentation. The finding was further confirmed on CT, along with establishing evidence of significant volume loss in the right middle and lower lobes with subsegmental atelectasis without features suggestive of pulmonary/pleural/vascular disease. M mode ultrasound also demonstrated paradoxical movement, clinching the diagnosis of shrinking lung syndrome.

Fortunately, despite the scarcity of clinical trials, available literature on isolated cases shows a near-complete resolution of clinical and pulmonary function test results with moderate to high-dose corticosteroids in the range of 0.5-1.0mg/kg/day.[9] The addition of immunosuppressants such as Methotrexate, Rituximab, and Colchicine not only improves imaging parameters but also prevents the recurrence of SLS features while tapering corticosteroids. [9,14–16]

Written informed patient consent for publication has been obtained.

References

[1] Smyth H, Flood R, Kane D, Donnelly S, Mullan RH. Shrinking lung syndrome and systemic lupus erythematosus: a case series and literature review. QJM: An International Journal of Medicine. 2018 Dec 1;111(12):839–843.

[2] Borrell H, Narváez J, Alegre JJ, Castellví I, Mitjavila F, Aparicio M, Armengol E, Molina-Molina M, Nolla JM. Shrinking lung syndrome in systemic lupus erythematosus. Medicine (Baltimore). 2016 Aug 19;95(33):e4626. PMCID: PMC5370827

[3] Hoffbrand BI, Beck ER. “Unexplained” Dyspnoea and Shrinking Lungs in Systemic Lupus Erythematosus. Br Med J. 1965 May 15;1(5445):1273–1277. PMCID: PMC2166064

[4] Henderson LA, Loring SH, Gill RR, Liao KP, Ishizawar R, Kim S, Perlmutter-Goldenson R, Rothman D, Son MBF, Stoll ML, Zemel LS, Sandborg C, Dellaripa PF, Nigrovic PA. Shrinking Lung Syndrome as a Manifestation of Pleuritis: A New Model Based on Pulmonary Physiological Studies. The Journal of Rheumatology. The Journal of Rheumatology; 2013 Mar 1;40(3):273–281. PMID: 23378468

[5] Martens J, Demedts M, Vanmeenen MT, Dequeker J. Respiratory Muscle Dysfunction in Systemic Lupus Erythematosus. CHEST. Elsevier; 1983 Aug 1;84(2):170–175.

[6] Wilcox PG, Stein HB, Clarke SD, Paré PD, Pardy RL. Phrenic Nerve Function in Patients with Diaphragmatic Weakness and Systemic Lupus Erythematosus. Chest. 1988 Feb 1;93(2):352–358.

[7] Choudhury S, Ramos M, Anjum H, Ali M, Surani S. Shrinking Lung Syndrome: A Rare Manifestation of Systemic Lupus Erythematosus. Cureus [Internet]. Cureus; 2020 May 21 [cited 2022 May 26];12(5). Available from: https://www.cureus.com/articles/32163-shrinking-lung-syndrome-a-rare-manifestation-of-systemic-lupus-erythematosus

[8] Almajed MR, Obri MS, Mahmood S, Demertzis ZD. Shrinking Lung Syndrome in a Young Female: A Rare Pulmonary Manifestation of Systemic Lupus Erythematosus. Cureus [Internet]. Cureus; 2022 Apr 20 [cited 2022 May 26];14(4). Available from: https://www.cureus.com/articles/95040-shrinking-lung-syndrome-in-a-young-female-a-rare-pulmonary-manifestation-of-systemic-lupus-erythematosus

[9] Duron L, Cohen-Aubart F, Diot E, Borie R, Abad S, Richez C, Banse C, Vittecoq O, Saadoun D, Haroche J, Amoura Z. Shrinking lung syndrome associated with systemic lupus erythematosus: A multicenter collaborative study of 15 new cases and a review of the 155 cases in the literature focusing on treatment response and long-term outcomes. Autoimmun Rev. 2016 Oct;15(10):994–1000. PMID: 27481038

[10] Omdal R, Roos P, Wildhagen K, Gunnarsson R. Respiratory arrest in systemic lupus erythematosus due to phrenic nerve neuropathy. Lupus. SAGE Publications Ltd STM; 2004 Oct 1;13(10):817–819.

[11] Singh R, Huang W, Menon Y, Espinoza LR. Shrinking lung syndrome in systemic lupus erythematosus and Sjogren’s syndrome. J Clin Rheumatol. 2002 Dec;8(6):340–345. PMID: 17041405

[12] Neto FB de A, Lyra TCB, Mello RKVG de, Valois VM, Albano TB, Paiva AFL de, Chate RC, Nomura CH. Shrinking Lung Detected on Computed Tomography: Pictorial Essay of the Main Findings of the Image. Open Journal of Medical Imaging. Scientific Research Publishing; 2018 Jun 14;8(2):17–24.

[13] Rajasurya V, Bhavsar S, Gudivada SD, Sharma V. M-Mode Ultrasound in the Diagnosis and Follow-up of Diaphragmatic Dysfunction in the Setting of Shrinking Lung Syndrome: A Case Report. CHEST. Elsevier; 2014 Oct 1;146(4):930A.

[14] Karim MY, Miranda LC, Tench CM, Gordon PA, D’Cruz DP, Khamashta MA, Hughes GRV. Presentation and prognosis of the shrinking lung syndrome in systemic lupus erythematosus. Seminars in Arthritis and Rheumatism. 2002 Apr 1;31(5):289–298.

[15] Peñacoba Toribio P, Córica Albani ME, Mayos Pérez M, Rodríguez de la Serna A. Rituximab in the Treatment of Shrinking Lung Syndrome in Systemic Lupus Erythematosus. Reumatología Clínica (English Edition). 2014 Sep 1;10(5):325–327.

[16] Pillai S, Mehta J, Levin T, Muzumdar H, Nandalike K. Shrinking lung syndrome presenting as an initial pulmonary manifestation of SLE. Lupus. SAGE Publications Ltd STM; 2014 Oct 1;23(11):1201–1203.

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