The exact physiopathology of this disease is not known and there is no specific treatment for it. 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. Plaquenil lowers blood sugar Chloroquine sulfa allergy Does plaquenil suppress the immune system Successful treatment of neonatal respiratory failure caused by a novel surfactant protein C p. Cys121Gly mutation with hydroxychloroquine Skip to main content Thank you for visiting Monoallelic mutations of the Surfactant Protein C gene SFTPC are associated with Interstitial Lung Disease in children. I73T is the most common mutation, accounting for 30 % of all cases describe three patients carrying the same. The exact physiopathology of this disease is not known and there is no specific treatment for it. 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. These data seem to cast some doubt on the efficacy of hydroxychloroquine, and they suggest that the clinical improvement was simply the natural evolution of the disease. Two years after the treatment was withdrawn, the twins were evaluated once again: they presented no new infections, growth and general state were normal and chest CT showed a notable additional reduction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. Surfactant protein c deficiency hydroxychloroquine Ten-year follow up of hydroxychloroquine treatment for., Surfactant Protein C-associated interstitial lung disease; three. Chloroquine dose in hindi 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. Hydroxychloroquin HCQ in chILD of Genetic Defect - Full.. Is Treatment With Hydroxychloroquine Effective in Surfactant.. Surfactant Protein C-associated interstitial lung disease.. The diagnosis of a surfactant protein C mutation c.545 GC.pGly182Arg was confirmed by genetic testing once available. Patient 1 had a family history of a maternal uncle dying of pneumonia at 6 months of age and a maternal grandfather dying of lung disease at 49 years of age, but neither one had genetic studies performed. 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. Focusing on immunomodulation and aggressive anti-inflammatory therapy, a triple-drug combination was chosen with hydroxychloroquine, azithromycin, and methylprednisolone pulse based on sporadic success in surfactant protein C deficiency.