Impact statement:
We report the largest single centre cohort of type 1 hereditary angioedema (HAE) from India. Most patients had novel mutation inSERPING1 gene. Clinical profile of patients in the present series was found to be similar to several previously reported series. Because of lack of availability of C1-INH therapy in India, patients were managed using attenuated androgens, tranexamic acid and fresh frozen plasma. Only one disease related mortality was reported in the entire cohort. Results of this study suggest that use of TA, stanozolol and FFP may still be an effective treatment option for patients with type 1 HAE in resource constrained settings.