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. Plaquenil generic shortage Hydroxychloroquine side effects kidney 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. There is merely news from a few previous descriptions in the literature about the use of hydroxychloroquine for surfactant protein C deficiency with satisfactory results. Two years after the treatment was withdrawn, the twins were evaluated once again they presented no new infections, growth and general state were normal and chest CT showed a notable additional reduction in the interstitial pneumopathy. SFTPC surfactant protein C mutations resulting in SP-C deficiency causing ongoing respiratory failure in the neonatal period represent a rare entity. 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 Successful treatment of neonatal respiratory failure caused., Is Treatment With Hydroxychloroquine Effective in. Can plaquenil cause tirednessAffects of stopping plaquenil SFTPC gene mutations associated with surfactant dysfunction affect the processing of the SP-C protein. Many of the mutations occur in a particular region of the gene called the BRICHOS domain, which appears to be involved in the processing and cellular placement of the SP-C protein. SFTPC gene - Genetics Home Reference - NIH. Successful treatment of neonatal respiratory failure.. Childhood interstitial lung disease due to surfactant.. Surfactant protein C, which is expressed as a pro-protein in AEC2s, is critical for surfactant homeostasis. Mutations in SFTPC result in the production of an abnormal pro-protein that accumulates in AEC2s. Chloroquine or hydroxychloroquine has been previously reported to improve symptoms in patients with surfactant protein C dysfunction. Hydroxychloroquine 200mg twice daily was added to his regimen, and his prednisone dose has been tapered to 15mg daily over the subsequent three months. This may be the mechanism by which HCQ tend to help in chILD, especially in those cases related to surfactant protein deficiency. SP-B and SP-C are synthesized in the endoplasmic reticulum ER of alveolar type II cells as large precursor proteins, are cleaved by proteolytic enzymes and transported through Golgi apparatus to multivesicular. 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.