Shrinking lung syndrome in systemic lupus erythematosus : A study of 9 patients
Copyright © 2023 Elsevier España, S.L.U. All rights reserved..
INTRODUCTION: Shrinking lung syndrome (SLS) is a rare manifestation of systemic lupus erythematosus. Our aim was to describe the clinical, radiological, and functional characteristics of a cohort with SLS and its evolution over time.
METHODS: A retrospective study was conducted between 2009 and 2018. Demographic, clinical, functional, radiological, and treatment data were collected.
RESULTS: Out of a total of 225 patients, 11 presented with SLS (prevalence of 4.8%). Two patients were excluded. The mean age was 39.33±16 years, and 6 were female. The main symptoms were dyspnea and pleuritic pain. The mean forced vital capacity was 49%, total lung capacity was 60%, carbon monoxide diffusing capacity was 66%, carbon monoxide transference factor was 128%, maximal inspiratory pressure was 66%, and maximal expiratory pressure was 82%. All patients received corticosteroids. After a median follow-up of 19 months, 4 cases showed improvement, and 4 cases remained stable.
CONCLUSIONS: SLS should be considered in every lupus patient with unexplained dyspnea. Although it often shows improvement, many cases experience persistent deterioration despite treatment.
| Medienart: |
E-Artikel |
|---|
| Erscheinungsjahr: |
2024 |
|---|---|
| Erschienen: |
2024 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:162 |
|---|---|
| Enthalten in: |
Medicina clinica - 162(2024), 7 vom: 12. Apr., Seite 350-353 |
| Sprache: |
Englisch |
|---|
| Weiterer Titel: |
Síndrome del pulmón encogido asociado a lupus eritematoso sistémico: estudio de 9 pacientes |
|---|
| Beteiligte Personen: |
Casey, Alberto [VerfasserIn] |
|---|
| Links: |
|---|
| Anmerkungen: |
Date Completed 01.04.2024 Date Revised 02.04.2024 published: Print-Electronic Citation Status MEDLINE |
|---|
| doi: |
10.1016/j.medcli.2023.10.023 |
|---|
| funding: |
|
|---|---|
| Förderinstitution / Projekttitel: |
|
| PPN (Katalog-ID): |
NLM366858777 |
|---|
| LEADER | 01000caa a22002652 4500 | ||
|---|---|---|---|
| 001 | NLM366858777 | ||
| 003 | DE-627 | ||
| 005 | 20240403235218.0 | ||
| 007 | cr uuu---uuuuu | ||
| 008 | 240114s2024 xx |||||o 00| ||eng c | ||
| 024 | 7 | |a 10.1016/j.medcli.2023.10.023 |2 doi | |
| 028 | 5 | 2 | |a pubmed24n1363.xml |
| 035 | |a (DE-627)NLM366858777 | ||
| 035 | |a (NLM)38195280 | ||
| 035 | |a (PII)S0025-7753(23)00703-0 | ||
| 040 | |a DE-627 |b ger |c DE-627 |e rakwb | ||
| 041 | |a eng | ||
| 100 | 1 | |a Casey, Alberto |e verfasserin |4 aut | |
| 245 | 1 | 0 | |a Shrinking lung syndrome in systemic lupus erythematosus |b A study of 9 patients |
| 246 | 3 | 3 | |a Síndrome del pulmón encogido asociado a lupus eritematoso sistémico: estudio de 9 pacientes |
| 264 | 1 | |c 2024 | |
| 336 | |a Text |b txt |2 rdacontent | ||
| 337 | |a ƒaComputermedien |b c |2 rdamedia | ||
| 338 | |a ƒa Online-Ressource |b cr |2 rdacarrier | ||
| 500 | |a Date Completed 01.04.2024 | ||
| 500 | |a Date Revised 02.04.2024 | ||
| 500 | |a published: Print-Electronic | ||
| 500 | |a Citation Status MEDLINE | ||
| 520 | |a Copyright © 2023 Elsevier España, S.L.U. All rights reserved. | ||
| 520 | |a INTRODUCTION: Shrinking lung syndrome (SLS) is a rare manifestation of systemic lupus erythematosus. Our aim was to describe the clinical, radiological, and functional characteristics of a cohort with SLS and its evolution over time | ||
| 520 | |a METHODS: A retrospective study was conducted between 2009 and 2018. Demographic, clinical, functional, radiological, and treatment data were collected | ||
| 520 | |a RESULTS: Out of a total of 225 patients, 11 presented with SLS (prevalence of 4.8%). Two patients were excluded. The mean age was 39.33±16 years, and 6 were female. The main symptoms were dyspnea and pleuritic pain. The mean forced vital capacity was 49%, total lung capacity was 60%, carbon monoxide diffusing capacity was 66%, carbon monoxide transference factor was 128%, maximal inspiratory pressure was 66%, and maximal expiratory pressure was 82%. All patients received corticosteroids. After a median follow-up of 19 months, 4 cases showed improvement, and 4 cases remained stable | ||
| 520 | |a CONCLUSIONS: SLS should be considered in every lupus patient with unexplained dyspnea. Although it often shows improvement, many cases experience persistent deterioration despite treatment | ||
| 650 | 4 | |a Case Reports | |
| 650 | 4 | |a Enfermedad pulmonar restrictiva | |
| 650 | 4 | |a Lupus eritematoso sistémico | |
| 650 | 4 | |a Restrictive lung disease | |
| 650 | 4 | |a Shrinking lung syndrome | |
| 650 | 4 | |a Systemic lupus erythematosus | |
| 650 | 4 | |a Síndrome de pulmón encogido | |
| 650 | 7 | |a Carbon Monoxide |2 NLM | |
| 650 | 7 | |a 7U1EE4V452 |2 NLM | |
| 700 | 1 | |a Enghelmayer, Juan I |e verfasserin |4 aut | |
| 700 | 1 | |a Legarreta, Cora G |e verfasserin |4 aut | |
| 700 | 1 | |a Berón, Ana María |e verfasserin |4 aut | |
| 700 | 1 | |a Perín, María Marta |e verfasserin |4 aut | |
| 700 | 1 | |a Dubinsky, Diana |e verfasserin |4 aut | |
| 773 | 0 | 8 | |i Enthalten in |t Medicina clinica |d 1948 |g 162(2024), 7 vom: 12. Apr., Seite 350-353 |w (DE-627)NLM000359017 |x 1578-8989 |7 nnns |
| 773 | 1 | 8 | |g volume:162 |g year:2024 |g number:7 |g day:12 |g month:04 |g pages:350-353 |
| 856 | 4 | 0 | |u http://dx.doi.org/10.1016/j.medcli.2023.10.023 |3 Volltext |
| 912 | |a GBV_USEFLAG_A | ||
| 912 | |a GBV_NLM | ||
| 951 | |a AR | ||
| 952 | |d 162 |j 2024 |e 7 |b 12 |c 04 |h 350-353 | ||