The Consensus recommends that sirolimus be used after the diagnosis of LAM in the presence of:

(1) Decreased lung function (FEV, <70% of predicted value); 

(2) Rapid decline in lung function (FEV, ≥90mL/year); 

(3) Symptomatic celiac disease or celiac ascites; 

(4) Renal angiomyolipoma or retroperitoneal and pelvic lymphangioleiomyoma (largest single tumor >3cm in diameter); 

(5) LAM associated with the tubemus sclemsis complex (TSC).

It should be noted that the methods of detection of whole blood drug concentration of sirolimus include immunoassay and chromatography, and the values between the different methods are not used interchangeably; the data measured by chromatography are on average about 20% lower than those by immunoassay. Patients need to be aware of this when visiting different hospitals and when adjusting drug doses based on drug concentrations.

This consensus recommends that the usual dose of sirolimus is 1-2 mg(once/d), and that the dose of the drug needs to be adjusted by monitoring the whole blood drug trough concentration, therapeutic response, and adverse effects of sirolimus. The target whole blood drug trough concentration is recommended to be 5~10 μg/L.

Sirolimus blood levels should be checked 2 to 4 weeks after the administration of sirolimus and adverse drug reactions should be observed. Initial follow-up can be every 3 months, and 6-12 months for stable patients.

This consensus recommends that patients with LAM should be evaluated periodically for disease progression, assessment of treatment options, and monitoring of adverse drug reactions.

This consensus recommends that sirolimus is recommended for long-term use if treatment is effective. Discontinuation of the drug is required in the event of allergic reactions, severe or serious adverse reactions severe infections, emerging interstitial pneumonitis, surgery or pregnancy.

Anaphylactic reactions (including severe fatal anaphylactic reactions) due to sirolimus, although rare, require close attention, especially during the initial period of administration. Common adverse drug reactions include mouth ulcers, acne-like skin changes, increased blood lipids and menstrual disorders.

If it is necessary to switch to a different manufacturer's product during the course of treatment, the doses of sirolimus corresponding to 0.5 to 2.0 mg may be interchangeable in equal amounts, and it is not clear whether doses of more than 2 mg are equivalent between different products and dosage forms. Considering the possible differences, it is recommended that blood levels be reviewed 2 weeks after switching products and dosage forms, regardless of the dose used.

Sirolimus treatment for LAM is a super drug indication in China, and patients need to be clearly informed of the current status, efficacy and safety of sirolimus treatment for LAM, and to obtain their informed consent. Although the efficacy of sirolimus in the treatment of LAM has been confirmed, there are still many issues that need to be further investigated, such as the timing of the drug, the minimum effective dose, long-term safety, and the choice of other therapeutic methods, which need to be studied in depth.