Sildenafil (CTD-Associated PAH / Raynaud's)

⚠️ SCOPE — PAH ONLY; NO RAYNAUD'S / DIGITAL-ULCER DOSE IS PUBLISHED. This page is 'Sildenafil (CTD-Associated PAH / Raynaud's)'. PAH is its primary and licensed indication and the sourced UK SPC doses it; §4.4 confirms connective-tissue-disease-associated PAH is one of the forms studied: 'Studies with sildenafil have been performed in forms of pulmonary arterial hypertension related to primary (idiopathic), connective tissue disease associated or congenital heart disease associated forms of PAH. The use of sildenafil in other forms of PAH is not recommended.' THE BUNDLE CONTAINS NO RAYNAUD'S PHENOMENON OR DIGITAL-ULCER POSOLOGY OF ANY KIND — that use is off-label and neither provider doses it, so the page's secondary indication is left unsourced rather than filled from the erectile-dysfunction label, whose 25–100 mg figures are for a different indication entirely. || VERBATIM, UK SPC §4.2: 'Treatment should only be initiated and monitored by a physician experienced in the treatment of pulmonary arterial hypertension. In case of clinical deterioration in spite of Revatio treatment, alternative therapies should be considered.' 'Adults — The recommended dose is 20 mg three times a day (TID).' || METHOD OF ADMINISTRATION (§4.2): 'Revatio powder for oral suspension is for oral use only. The reconstituted oral suspension (a white, grape flavoured oral suspension) should be taken approximately 6 to 8 hours apart with or without food. Before withdrawing the required dose, shake the bottle vigorously for a minimum of 10 seconds.' || MISSED DOSE (§4.2): 'Physicians should advise patients who forget to take Revatio to take a dose as soon as possible and then continue with the normal dose. Patients should not take a double dose to compensate for the missed dose.' || CO-ADMINISTERED CYP3A4 INHIBITORS (§4.2): 'A downward dose adjustment to 20 mg twice daily should be considered when sildenafil is co-administered to patients already receiving CYP3A4 inhibitors like erythromycin or saquinavir. A downward dose adjustment to 20 mg once daily is recommended in case of co-administration with more potent CYP3A4 inhibitors clarithromycin, telithromycin and nefazodone.' The MOST potent CYP3A4 inhibitors (ketoconazole, itraconazole, ritonavir) are a CONTRAINDICATION, not a dose reduction (§4.3). §4.2 adds: 'Dose adjustments for sildenafil may be required when co-administered with CYP3A4 inducers.' || ELDERLY (§4.2): 'Dose adjustments are not required in elderly patients. Clinical efficacy as measured by 6-minute walk distance could be less in elderly patients.' || STOPPING (§4.2): 'Limited data suggest that the abrupt discontinuation of Revatio is not associated with rebound worsening of pulmonary arterial hypertension. However to avoid the possible occurrence of sudden clinical deterioration during withdrawal, a gradual dose reduction should be considered. Intensified monitoring is recommended during the discontinuation period.' || WHICH FORMS OF PAH (§4.4): 'Studies with sildenafil have been performed in forms of pulmonary arterial hypertension related to primary (idiopathic), connective tissue disease associated or congenital heart disease associated forms of PAH. The use of sildenafil in other forms of PAH is not recommended.' §4.4 also states efficacy 'has not been established in patients with severe pulmonary arterial hypertension (functional class IV)' and that the benefit-risk balance 'has not been established in patients assessed to be at WHO functional class I pulmonary arterial hypertension'. || MAX DOSE LEFT EMPTY DELIBERATELY: the SPC states no adult ceiling with any explicit maximum cue. The '100 mg' maximum in the bundle's other provider belongs to the ERECTILE-DYSFUNCTION tablet label and must never be applied to PAH. §4.8 records that 40 mg and 80 mg TID were given in the trials ('Doses up to 80 mg three times a day (4 times the recommended dose of 20 mg three times a day) were administered') — that is trial exposure, not a licensed ceiling, and discontinuation for adverse events rose with dose (2.9% at 20 mg TID vs 8.5% at 80 mg TID).

PAEDIATRIC PAH, 1 to 17 years — the PAH half of this page. The SPC gives no paediatric regimen for Raynaud's phenomenon or digital ulcers. || ⚠️ WEIGHT-BANDED, NOT PER-KG: dosePerKg is deliberately null so the weight calculator does not run — a band is not a mg/kg figure and must not be interpolated between bands. || VERBATIM, UK SPC §4.2, paediatric population (1 year to 17 years): 'For paediatric patients aged 1 year to 17 years old, the recommended dose in patients ≤ 20 kg is 10 mg (1 ml of reconstituted suspension) three times a day and for patients > 20 kg is 20 mg (2 ml of reconstituted suspension) three times a day. Higher than recommended doses should not be used in paediatric patients with PAH (see also sections 4.4 and 5.1).' Note the boundary: 20 kg itself falls in the 10 mg band. || ⚠️ MORTALITY SIGNAL ABOVE THE RECOMMENDED DOSE — §4.4: 'In the long term paediatric extension study, an increase in deaths was observed in patients administered doses higher than the recommended dose. Therefore, doses higher than the recommended doses should not be used in paediatric patients with PAH.' || ⚠️ NEONATES / PPHN — §4.2: 'Outside its authorised indications, sildenafil should not be used in neonates with persistent pulmonary hypertension of the newborn as risks outweigh the benefits (see section 5.1).' || UNDER 1 YEAR — §4.2: 'The safety and efficacy of Revatio in other conditions in children below 1 year of age has not been established. No data are available.' || CONCENTRATION 10 mg/ml is the strength of the sourced product (Revatio 10 mg/ml powder for oral suspension), confirmed by the SPC's own '10 mg (1 ml of reconstituted suspension)'; confirm the strength of the preparation in hand. Shake the bottle vigorously for a minimum of 10 seconds before withdrawing the dose.