u-62840 and Respiratory-Insufficiency

Topics: Anti-Asthmatic Agents; Antibodies, Monoclonal, Humanized; Antihypertensive Agents; Asthma; COVID-19; COVID-19 Vaccines; Cytokines; Epoprostenol; Humans; Hypertension, Pulmonary; Infection Control; Injections, Subcutaneous; Intensive Care Units; Lung Diseases, Interstitial; Nursing Homes; Oxygen; Oxygen Inhalation Therapy; Respiratory Insufficiency; Symptom Flare Up; Thymic Stromal Lymphopoietin; Walk Test

This case demonstrates chronic fibrosing pleuritis, as a rare pulmonary aetiology for mortality in patients with Degos disease or malignant atrophic papulosis (MAP). Knowledge of this unusual complication will help physicians identify this entity early and provide appropriate treatment.Patients with MAP die from gastrointestinal and brain involvement within 2-3 years of diagnosis. This case is unique as the patient survived for 9 years and died secondary to respiratory failure, which had not been reported before. Our patient was a young man, diagnosed with MAP at the age of 17. His skin and gastrointestinal disease were controlled with eculizumab and parenteral treprostinil. The patient developed severe restrictive pulmonary disease, required ventilatory support, and died from respiratory failure. An autopsy revealed chronic fibrosis pleuritis. Longer surviving patients with MAP might suffer from significant respiratory disease. Pulmonary function test should be obtained to identify subclinical respiratory limitation.

Topics: Antibodies, Monoclonal, Humanized; Chronic Disease; Complement Inactivating Agents; Conservative Treatment; Diagnosis, Differential; Drug Therapy, Combination; Epoprostenol; Fatal Outcome; Humans; Male; Malignant Atrophic Papulosis; Pleurisy; Respiratory Insufficiency; Young Adult

A 20-year-old man with pulmonary arterial hypertension secondary to systemic sclerosis was admitted to our hospital. Prior to admission, his PAH had been successfully managed with the use of tadalafil, ambrisentan and inhaled Tyvaso. Owing to respiratory failure from vocal cord paralysis, he underwent an emergent tracheotomy. The delivery of inhaled Tyvaso through a tracheostomy tube was explored. Post-tracheostomy, the patient continued his ability to self-administer the medication. His WHO functional classification, brain natriuretic peptide levels, and echocardiograms were not significantly different when Tyvaso was administered via tracheostomy compared with oral administration. This case report summarises the method used to deliver Tyvaso via a tracheostomy tube, which proved to be successful in this patient.

Topics: Administration, Inhalation; Administration, Oral; Adult; Antihypertensive Agents; Epoprostenol; Humans; Hypertension, Pulmonary; Male; Respiratory Insufficiency; Tracheostomy; Tracheotomy; Vocal Cord Paralysis; Young Adult