Shrinking lung syndrome in autoimmune inflammatory diseases: A case series and review of literature

doi:10.5847/wjem.j.1920-8642.2022.041

World Journal of Emergency Medicine ›› 2022, Vol. 13 ›› Issue (3): 254-256.doi: 10.5847/wjem.j.1920-8642.2022.041

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Shrinking lung syndrome in autoimmune inflammatory diseases: A case series and review of literature

Xiao-chan Chen¹, Shan He², Jing Xue¹()

¹Department of Rheumatology, the Second Affiliated Hospital of Zhejiang University School of Medicine, Hangzhou 310009, China
²Department of Rheumatology and Immunology, the Affiliated Jinhua Hospital of Zhejiang University School of Medicine, Jinhua 321000, China

Received:2021-11-16 Accepted:2022-03-10 Online:2022-05-13 Published:2022-05-01
Contact: Jing Xue E-mail:jingxue@zju.edu.cn

1. Supplementary Table 1. Shrinking lung syndrome in non-SLE patients from the literature.docx(22KB)

Abstract

Xiao-chan Chen, Shan He, Jing Xue. Shrinking lung syndrome in autoimmune inflammatory diseases: A case series and review of literature[J]. World Journal of Emergency Medicine, 2022, 13(3): 254-256.

Figures/Tables 3

Table 1.

Figure 1.

Table 2.

References 10

1	Deeb M, Tselios K, Gladman DD, Su J, Urowitz MB. Shrinking lung syndrome in systemic lupus erythematosus: a single-centre experience. Lupus. 2018; 27(3):365-71. doi: 10.1177/0961203317722411 pmid: 28758573
2	Uslu S, Köken Avşar A, Erez Y, Sarı İ. Shrinking lung syndrome in primary Sjögren syndrome. Balkan Med J. 2020; 37(6):351-2.
3	Baenas DF, Retamozo S, Pirola JP, Caeiro F. Shrinking lung syndrome and pleural effusion as an initial manifestation of primary Sjögren's syndrome. Reumatol Clin (Engl Ed). 2020; 16(1):65-8.
4	Scirè CA, Caporali R, Zanierato M, Mojoli F, Braschi A, Montecucco C. Shrinking lung syndrome in systemic sclerosis. Arthritis Rheum. 2003; 48(10):2999-3000. doi: 10.1002/art.11393
5	Ahmed S, Herrick A, O'Driscoll BR. Shrinking lung syndrome in patients without systemic lupus erythematosus: comment on the concise communication by Tavoni et al. Arthritis Rheum. 2001; 44(1):243-5.
6	Henderson LA, Loring SH, Gill RR, Liao KP, Ishizawar R, Kim S, et al. Shrinking lung syndrome as a manifestation of pleuritis: a new model based on pulmonary physiological studies. J Rheumatol. 2013; 40(3):273-81. doi: 10.3899/jrheum.121048 pmid: 23378468
7	Duron L, Cohen-Aubart F, Diot E, Borie R, Abad S, Richez C, et al. 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; 15(10):994-1000. doi: 10.1016/j.autrev.2016.07.021 pmid: 27481038
8	Desai SS, Allen E, Deodhar A. Miller Fisher syndrome in adult onset Still's disease: case report and review of the literature of other neurological manifestations. Rheumatol Oxf Engl. 2002; 41(2):216-22.
9	Smyth H, Flood R, Kane D, Donnelly S, Mullan RH. Shrinking lung syndrome and systemic lupus erythematosus: a case series and literature review. QJM. 2018; 111(12):839-43. doi: 10.1093/qjmed/hcx204 pmid: 29088421
10	Ciaffi J, Gegenava M, Ninaber MK, Huizinga TWJ. Shrinking lung syndrome: diagnostic and therapeutic challenges in 3 patients with systemic lupus erythematosus. J Clin Rheumatol. 20211; 27(8S):S525-9. doi: 10.1097/RHU.0000000000001132

Variables		Case 1	Case 2	Case 3
Age, years		26	53	68
Sex		Female	Female	Female
Primary disease		SLE	SLE	AOSD
Duration of the primary disease, years		12.0	2.0	0.4
Treatment before the diagnosis of SLS		Prednisone, HCQ, TAC	Prednisone, HCQ	Prednisone
Presenting features of SLS		Dyspnea, cough	Dyspnea, chest pain	Dyspnea
Other clinical manifestations		Mucocutaneous, arthritis, fever, nephritis	Mucocutaneous, arthritis, nephritis, cardiac valvular dysfunction	Fever, mucocutaneous
Autoantibodies		ANA, Smith, RNP, SSA, Ro-52, SSB, ARPA	ANA, dsDNA, SSA, Ro-52, SSB,	None
Chest CT		Aatelectasis, diaphragm elevation, pleural effusion, pleural thickening	Aatelectasis, pleural effusion	Atelectasis, diaphragm elevation
SLEDAI		10	20	/
Pulmonary function test
	Forced vital capacity, %	62.5	59.1	72.4
	Total lung capacity, %	69.9	72.2	66.9
	FEV1, %	67.3	65.0	74.8
	DLCO, %	42.4	55.2	50.4
Treatment		MP 60 mg/d+CYC+TAC	MP 500 mg/d+CYC+IVIG	MP 160 mg/d+CYC
Follow-up time, months		36	8	6
Clinical outcome		Improved	Improved	Improved
PFT outcome		Improved	Improved	Not improved
HRCT outcome		Improved	Improved	Not improved

Variables	SLE (n=225)	Non-SLE (n=10)
General characteristics
Age, years, median (range)	32 (11-69)	48 (15-68)
Sex (female/male)	208/17	9/1
Duration of the primary disease, years, median (range)	5.4 (0-33.0)	2.8 (0-10.0)
Clinical manifestations, n (%)
Dyspnea	201/203 (99.0)	10/10 (100.0)
Pleuritic chest pain	127/160 (79.4)	6/10 (60.0)
Cough	38/118 (32.2)	2/10 (20.0)
Image, PFT, and EMG findings, n (%)
Diaphragm elevation	186/204 (91.2)	9/10 (90.0)
Pleural effusion	71/204 (34.8)	5/10 (50.0)
Atelectasis	31/204 (15.2)	7/10 (70.0)
Pleural thickening	24/204 (11.7)	2/10 (20.0)
PFT restrictive pattern	198/205 (96.6)	10/10 (100.0)
Phrenic nerve desfunction	3/16 (18.8)	3/4 (75.0)
Treatment, n (%)
Glucocorticoids	166/175 (94.9)	10/10 (100.0)
Azathioprine	46/175 (26.3)	3/10 (30.0)
Cyclophosphamide	33/175 (18.9)	2/10 (20.0)
Mycophenolate mofetil	20/175 (11.4)	1/10 (10.0)
Methotrexate	9/175 (5.1)	0
Rituximab	18/175 (10.3)	3/10 (30.0)
Beliuumab	2/175 (1.1)	0
Outcome, n (%)
Clinical improvement	78/106 (73.6)	10/10 (100.0)
PFT improvement	37/68 (54.4)	6/8 (75.0)
Image improvement	10/46 (21.7)	6/8 (75.0)

Shrinking lung syndrome in autoimmune inflammatory diseases: A case series and review of literature

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