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Shrinking lung syndrome in pediatric systemic lupus erythematosus.
- Source :
-
Lupus [Lupus] 2021 Jun; Vol. 30 (7), pp. 1175-1179. Date of Electronic Publication: 2021 Apr 22. - Publication Year :
- 2021
-
Abstract
- Objective: To describe clinical, radiological and treatment characteristics in pediatric patients with SLS.<br />Material and Methods: This is a descriptive and retrospective study in patients under 16 years old with the diagnosis of SLE complicated by SLS at the General Hospital. National Medical Center La Raza. Clinical, radiological and treatment variables were analyzed. Results are shown in frequencies and percentages.<br />Results: Data from 11 patients, 9 females and 2 males were collected. Mean age at diagnosis of SLS was 12.2 years. Age at diagnosis of SLE was 11.1 years. SLEDAI 17.3. Renal desease 72%, hematological 91%, lymphopenia 63%, mucocutaneous 72%, neurological 9%, arthritis 54%, serositis 91%, fever 81%, secondary antiphospholipid syndrome, low C3 72%, low C4 81%, positive ANA 91%, positive anti-DNA 91%. Regarding clinical manifestations of SLE: cough 81%, dyspnea 91%, hipoxemia 81%, pleuritic pain 71%, average oxygen saturation 83%. Chest X-rays findings: right hemidiaphragm affection 18%, left 63%, bilateral 18%. Elevated hemidiaphragm 91%, atelectasis 18%, pleural effusion 91%, over one third of the cardiac silhouette under the diphragm 36%, bulging diaphragm 45%, 5th. anterior rib that crosses over the diaphragm 91%. M-mode ultrasound: diaphragmatic hypomotility 100%, pleural effusion 63%. Pulmonary function tests: restrictive pattern in 45% of the cases. Treatment was with supplementary oxygen 100%, intubation 18%, antibiotics 100%, steroids 100%, intravenous immunoglobulin 54%, plasmapheresis 18%, cyclophosphamide 54% and rituximab 18%. The clinical course was favorable in 81%.<br />Conclusions: SLS should be suspected in patients with SLE and active disease who present hipoxemia, pleuritic pain, cough, dyspnea, pleural effusion and signs of restriction on chest X-rays. Therefore, a diaphragmatic M-mode ultrasound should be performed in order to establish the diagnosis.
- Subjects :
- Adolescent
Anti-Bacterial Agents therapeutic use
Antiphospholipid Syndrome complications
Antiphospholipid Syndrome diagnosis
Antiphospholipid Syndrome immunology
Chest Pain etiology
Child
Combined Modality Therapy methods
Cyclophosphamide therapeutic use
Diaphragm diagnostic imaging
Dyspnea etiology
Female
Humans
Hypoxia etiology
Immunoglobulins, Intravenous therapeutic use
Immunologic Factors therapeutic use
Immunosuppressive Agents therapeutic use
Intubation, Intratracheal methods
Lung Diseases therapy
Lupus Erythematosus, Systemic diagnosis
Lupus Erythematosus, Systemic immunology
Male
Mexico epidemiology
Oxygen administration & dosage
Oxygen therapeutic use
Plasmapheresis methods
Pleurisy complications
Pulmonary Atelectasis etiology
Retrospective Studies
Rituximab therapeutic use
Steroids therapeutic use
Ultrasonography methods
Diaphragm abnormalities
Diaphragm physiopathology
Lung Diseases etiology
Lung Diseases physiopathology
Lupus Erythematosus, Systemic complications
Subjects
Details
- Language :
- English
- ISSN :
- 1477-0962
- Volume :
- 30
- Issue :
- 7
- Database :
- MEDLINE
- Journal :
- Lupus
- Publication Type :
- Academic Journal
- Accession number :
- 33888011
- Full Text :
- https://doi.org/10.1177/09612033211010331