Inclusion Criteria:

- If female, documentation of negative pregnancy test prior to enrolment.

- Participants, including males, must use an effective form of contraception, whilst
taking sirolimus and for twelve weeks after stopping the drug

- One or more renal angiomyolipomata of at least two centimetres or greater in largest
diameter

- Adequate renal function :glomerular filtration rate > 40 ml/min

- Clinically definite diagnosis of tuberous sclerosis (modified Gomez criteria) or
sporadic LAM (biopsy-proven or compatible high resolution chest CT scan and
respiratory function tests.)

- Signed and dated informed consent

Exclusion Criteria:

- History of non-compliance or inability to give informed consent

- Significant haematological or hepatic abnormality (i.e. transaminase levels > 150
i.u./L serum albumin < 30 g/L, haematocrit< 30%, platelets < 100,000/ mm3, adjusted
absolute neutrophil count < 1,500/mm3, total WBC < 3,000/ mm3)

- Greater than 1 g proteinuria daily

- Multiple bilateral AMLs, where individual lesions cannot be distinguished

- Renal haemorrhage within preceding year

- In those who have had a renal haemorrhage, known conservatively managed renal
aneurysm(s) greater than 10mm

- Patients who have had embolisation for AML(s) within the preceding 6 months

- Patients who are unable to walk 100 metres on the flat

- Continuous requirement for supplemental oxygen

- Patients who have had or are being considered for organ transplant

- Uncontrolled hyperlipidaemia

- Intercurrent infection at initiation of Sirolimus

- Surgery within last 2 months

- Pregnant or lactating women

- Use of an investigational drug within the last 30 days

- Change in anti epileptic drug medication within the last 3 months

- Likely to need vaccination e.g. for travel during the course of the trial (except for
influenza vaccine in patients with LAM)

- Current usage of strong inhibitors of CYP3AE ( such as ketoconazole, voriconazole,
itraconazole, tilithromycin or clarithromycin) or strong inducers (such as
rifampicin or rifabutin)