1. Shrinking lung syndrome in pediatric systemic lupus erythematosus.
- Author
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Torres Jimenez AR, Ruiz Vela N, Cespedes Cruz AI, Velazquez Cruz A, and Bernardino Gonzalez AK
- 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
- Abstract
Objective: To describe clinical, radiological and treatment characteristics in pediatric patients with SLS., 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., 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%., 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.
- Published
- 2021
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