No clinical trials of medications for ch ILD have been conducted to date. Corticosteroids have been the mainstay of therapy in most children and adults with interstitial lung disease (ILD), despite little conclusive evidence of their efficacy. Hydroxychloroquine plaquenil Hydroxychloroquine for low platelets Can plaquenil cause psoriasis Surfactant protein B SP-B and C SP-C are mainly involved in preventing alveolar collapse while surfactant protein A SP-A and D SP-D play a role in the lung’s immune defense. ABCA3 is a protein that transports surfactant within the alveolar Type II cell, the cell type in the lung that produces pulmonary surfactant. While most surfactant deficiencies are secondary to prematurity or lung injury and infection, hereditary surfactant protein B deficiency, caused by mutations in the surfactant ommbid. With surfactant deficiency, a greater pressure is needed to open the alveoli. Feb 21, 2020 In addition to its anti-inflammatory properties, hydroxychloroquine is thought to inhibit intracellular processing of the precursor of surfactant protein C SP-C, which may be the mechanism of action in SP-C deficiency. Hydroxychloroquine sulfate 200 mg equivalent to 155 mg hydroxychloroquine base and 250 mg chloroquine phosphate. These agents elicit anti-inflammatory properties and cause profound and varied metabolic effects. The medications commonly used for pharmacotherapy in ch ILD and common adverse effects are reviewed below. Hydroxychloroquine surfactant protein c deficiency Hydroxychloroquine Uses, Dosage & Side Effects -, Surfactant Deficiency Causes & Reasons - Symptoma® Plaquenil lupus hair lossWhat is plaquenil 200mgHydroxychloroquine for sjogrensIs there a drug interaction between ibuprofen and plaquenilHydroxychloroquine with requip The ABCA3 protein transports phospholipids into lamellar bodies where they form surfactant. The ABCA3 protein also appears to be involved in the formation of lamellar bodies. ABCA3 gene mutations, which cause a type of surfactant dysfunction sometimes referred to as ABCA3 deficiency, lead to reduction or absence of the protein's function. Without ABCA3 protein function, the transport of surfactant phospholipids is decreased. Surfactant dysfunction - Genetics Home Reference - NIH. Childhood Interstitial Lung Disease ChILD Medication.. Hydroxychloroquine and Surfactant Protein C Deficiency.. Subsequently, surfactant protein gene analysis revealed mutations in the ABCA3 gene. This case of ABCA3 deficient ILD describes a stable clinical course over 10 years and highlights the potential benefit of hydroxychloroquine for this condition. Pediatr Pulmonol. 2014; 9–301. In a recently published report, three infants with SP-C deficiency due to an I73T mutation and unusually early respiratory symptoms carried an additional mutation in the ATP binding cassette protein A3 gene ABCA3, a phospholipid carrier essential for lamellar body formation and surfactant intracellular assembly. Chest Radiographs and a CT Scan in a Boy with Surfactant Protein C Deficiency Treated with Hydroxychloroquine. Panel A shows a chest radiograph and Panel B a CT scan obtained at the initiation of.