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 and hyperpigmentation Chloroquine mnemonic 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. 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. Childhood interstitial lung disease due to surfactant protein C deficiency frequent use and costs of hospital services for a single case in Australia Neil J Hime 1, 2 Dominic Fitzgerald 2, 3 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 and Surfactant Protein C Deficiency, Recurrent diffuse lung disease due to surfactant protein C. Anti malaria pills chloroquineHydroxychloroquine side effects liverChloroquine belong to what drug familyChikungunya disease and chloroquine treatment In an infant and mother with respiratory insufficiency due to surfactant metabolism dysfunction and surfactant protein C deficiency SMDP2; 610913, Nogee et al. 2001 identified a heterozygous 460+1G-A mutation genomic DNA base 1728 in the splice donor site of intron 4 of the SFTPC gene. The mutation resulted in the skipping of exon 4 and the deletion of 37 amino acids in the C-terminal domain of the precursor protein, which was predicted to disrupt intracellular transport. SURFACTANT, PULMONARY-ASSOCIATED PROTEIN C; SFTPC - OMIM. Childhood interstitial lung disease due to surfactant.. Hydroxychloroquine Uses, Dosage & Side Effects -. Surfactant protein C deficiency is characterised by deficiency of the protein and its intracellular accumulation, with subsequent local inflammation and interstitial lung disease. There is no specific treatment, but hydroxychloroquine has been reported to be effective. 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.