Med2Date — Clinical Guidelines
Home Oncology Ovarian Cancer

Ovarian Cancer

Last updated 31 May 2026 · Oncology clinical guideline

📋 Key Information Summary

📋
  • Ovarian cancer is the 8th most common cancer in Australian women and the most lethal gynaecological malignancy — ~1,800 new diagnoses and ~1,000 deaths per year in Australia.
  • ~75% of women present at FIGO Stage III–IV with peritoneal dissemination; 5-year overall survival remains ~46% (all stages).
  • High-grade serous carcinoma (HGSC) accounts for ~70% of epithelial ovarian cancers and is the subtype most responsive to platinum-based chemotherapy.
  • Key modifiable risk factors include nulliparity, obesity, endometriosis, and talcum powder use; protective factors include OCP use, multiparity, and breastfeeding.
  • Germline BRCA1/BRCA2 pathogenic variants account for ~15% of cases; all women with non-mucinous ovarian cancer should be referred for genetic counselling and testing.
  • CA-125 is the principal tumour marker for HGSC; HE4 and ROMA score may aid pre-surgical risk assessment in adnexal masses.
  • Primary treatment is maximal cytoreductive surgery (ideally no macroscopic residual disease) followed by adjuvant platinum-taxane chemotherapy (carboplatin AUC5 + paclitaxel 175 mg/m² q3w × 6 cycles).
  • Neoadjuvant chemotherapy (NACT) followed by interval debulking surgery is an alternative for patients with unresectable disease or poor surgical candidates.
  • PARP inhibitors (olaparib, niraparib) are PBS-listed for maintenance in newly diagnosed advanced HGSC with BRCA1/2 mutation or HRD-positive tumours, and in platinum-sensitive relapse.
  • Hyperthermic intraperitoneal chemotherapy (HIPEC) at interval debulking may improve outcomes in selected patients but remains under evaluation in Australia.
  • Aboriginal and Torres Strait Islander women have higher mortality rates and later-stage diagnosis due to barriers in access to specialist care and genetic testing.
  • Bevacizumab is PBS-listed in combination with chemotherapy for advanced-stage disease; angiogenesis inhibition improves PFS especially in high-risk subgroups.

Introduction & Australian Epidemiology

Ovarian cancer is the most lethal gynaecological malignancy in Australia and worldwide. It encompasses a heterogeneous group of neoplasms arising from the ovary, fallopian tube, or primary peritoneal surface. Due to the absence of effective screening and non-specific early symptoms, the majority of women present with advanced-stage disease when cure is less achievable.

In Australia, approximately 1,800 women are diagnosed with ovarian cancer each year, and around 1,000 die from the disease, making it the 8th most common cancer in women but the 6th leading cause of cancer death. The age-standardised incidence rate is approximately 10.4 per 100,000 women per year (AIHW, 2023).

The 5-year relative survival for all stages combined is approximately 46%, a figure that has improved only modestly over recent decades. Survival is strongly stage-dependent: Stage I disease carries a 5-year survival exceeding 90%, whereas Stage IV disease falls below 20%.

This guideline covers the major epithelial ovarian cancers (serous, endometrioid, clear cell, mucinous) with emphasis on high-grade serous carcinoma (HGSC). Germ cell tumours and sex cord-stromal tumours, which have distinct biology and management, are addressed briefly.

⚠️
Late presentation: ~75% of ovarian cancers are diagnosed at FIGO Stage III or IV. There is no validated population-based screening programme. A high index of clinical suspicion is essential in women presenting with persistent new-onset pelvic/abdominal symptoms.

Epidemiology & Risk Factors

Incidence in Australia

Ovarian cancer is predominantly a disease of post-menopausal women, with a median age at diagnosis of 64 years. Incidence increases with age and peaks in the 75–79 age group. The lifetime risk is approximately 1 in 85.

Statistic Value (Australia)
New cases/year~1,800
Deaths/year~1,000
Age-standardised incidence10.4 per 100,000
5-year relative survival (all stages)~46%
Median age at diagnosis64 years
Lifetime risk1 in 85

Risk Factors

Category Risk Factor Effect
Increased riskGermline BRCA1 pathogenic variant40–60% lifetime risk
Germline BRCA2 pathogenic variant10–27% lifetime risk
Lynch syndrome (MLH1, MSH2, MSH6, PMS2, EPCAM)8–10% lifetime risk
EndometriosisOR 1.3–1.9 (clear cell, endometrioid)
Nulliparity, early menarche, late menopauseUnopposed oestrogen hypothesis
Decreased riskOral contraceptive pill (≥5 years)Risk reduction ~50%
Multiparity, breastfeedingOvulation suppression
Bilateral salpingo-oophorectomyNear-complete risk elimination

Risk-Reducing Surgery

Bilateral salpingo-oophorectomy (RRSO) is recommended for BRCA1 carriers aged 35–40 (after completion of childbearing) and BRCA2 carriers aged 40–45. Opportunistic salpingectomy at the time of benign pelvic or abdominal surgery is an emerging population-level prevention strategy (Australia's DOVE trial — Detecting OVarian cancer Early).

Pathology & FIGO Staging

Histological Subtypes

Epithelial ovarian cancers are classified by the WHO system. The dualistic model divides them into Type I (low-grade, indolent, often arising from precursor lesions) and Type II (high-grade, aggressive, often originating from serous tubal intraepithelial carcinoma of the fallopian tube).

Subtype Frequency Key Features
High-grade serous (HGSC)~70%TP53 mutations, BRCA-associated, platinum-sensitive; most common subtype
Endometrioid~10%Associated with endometriosis; ARID1A, PIK3CA mutations; favourable prognosis
Clear cell~10%Associated with endometriosis; ARID1A, PIK3CA mutations; relatively platinum-resistant
Mucinous~3%KRAS mutations; often large, unilateral; poor response to standard chemo
Low-grade serous~3%BRAF/KRAS mutations; endocrine therapy responsive; MEK inhibitor trials
Malignant Brenner, carcinosarcoma<2%Rare subtypes with variable prognosis

FIGO Staging (2014 revision)

Stage Description 5-Year Survival
ILimited to ovaries/tubes~90%
IAOne ovary, capsule intact, no surface tumour~94%
IBBoth ovaries, capsules intact~92%
ICSurgical spill, capsule rupture, positive washings~85%
IIPelvic extension~70%
IIIPeritoneal spread beyond pelvis and/or positive retroperitoneal nodes~35–40%
IVDistant metastases (pleural effusion with +ve cytology, liver parenchymal, splenic)~17%
ℹ️
Key pathology note: All HGSC should be tested for homologous recombination deficiency (HRD) status and BRCA1/2 mutations (germline and somatic) to guide PARP inhibitor therapy. This is now standard of care in Australia.

Clinical Features & CA-125

Symptoms

Ovarian cancer frequently presents with vague, non-specific symptoms that are often attributed to benign conditions. The following symptoms, if persistent (≥12 days per month for <12 months), should prompt investigation:

  • Abdominal bloating or increased abdominal girth
  • Early satiety or difficulty eating
  • Pelvic or abdominal pain
  • Urinary frequency or urgency
  • Unexplained weight loss
  • Fatigue
  • Change in bowel habit
⚠️
NICE/RCOG referral criteria: Women aged ≥50 with persistent symptoms suggestive of ovarian cancer (bloating, abdominal pain, early satiety, urinary urgency) should have a serum CA-125 and pelvic ultrasound within 2 weeks.

Tumour Markers

Marker Subtype Association Clinical Utility
CA-125HGSC (elevated in ~80%)Diagnosis, monitoring response, surveillance; limited sensitivity in early-stage disease (~50%)
HE4Serous, endometrioidROMA score (combines CA-125 + HE4 + menopausal status) improves pre-surgical risk stratification
AFPYolk sac tumourEssential in germ cell tumours; MBS item 66654
β-hCGChoriocarcinoma, mixed germ cellMonitor during and after treatment
InhibinGranulosa cell tumourSurveillance marker for sex cord-stromal tumours

Diagnostic Imaging

  • Transvaginal ultrasound (TVUS): First-line imaging. IOTA Simple Rules or ADNEX model used to assess malignancy risk of adnexal masses.
  • Pelvic MRI: Useful for characterising indeterminate adnexal masses and assessing local extent. MBS item 63537.
  • CT chest/abdomen/pelvis: Standard for staging; assessing operability and peritoneal disease burden.
  • PET-CT: Not routinely recommended for primary diagnosis; may have a role in detecting recurrent disease.

Management: Surgery, Chemotherapy & PARP Inhibitors

Principles of Surgery

The cornerstone of curative-intent treatment for advanced ovarian cancer is maximal cytoreductive (debulking) surgery. The goal is complete macroscopic cytoreduction (CC-0 / R0 resection), which is the single strongest prognostic factor.

Optimal
CC-0 / R0
No macroscopic residual disease
Median OS: 60+ months
Suboptimal
CC-1 (<1 cm)
Residual disease <1 cm maximum diameter
Median OS: ~40 months
Incomplete
CC-2/3 (>1 cm)
Residual disease >1 cm
Median OS: ~25 months

Surgical Procedures

  • Total hysterectomy + bilateral salpingo-oophorectomy (TH-BSO)
  • Omentectomy (infracolic or greater)
  • Peritoneal stripping (diaphragm, pelvic peritoneum)
  • Pelvic and para-aortic lymphadenectomy (if clinically negative)
  • Bowel resection (if required for complete cytoreduction)
  • Splenectomy ± distal pancreatectomy (for splenic hilum disease)
  • Appendicectomy (especially if mucinous histology)
⚠️
Centralisation of care: Surgery should be performed by a gynaecological oncologist at a high-volume centre (≥20 cases/year). Centralisation is associated with improved complete cytoreduction rates and survival (RANZCOG/Cancer Australia recommendation).

Primary vs Interval Debulking

For patients with potentially resectable disease (assessed by CT/clinical scoring), primary debulking surgery (PDS) followed by adjuvant chemotherapy is preferred. For patients with unresectable disease (e.g., extensive upper abdominal disease, poor performance status), neoadjuvant chemotherapy (NACT) × 3 cycles → interval debulking surgery (IDS) → adjuvant chemotherapy × 3 cycles is an equivalent alternative.

1
Primary Debulking Surgery (PDS)
TH-BSO, omentectomy, peritonectomy → Adjuvant chemo × 6 cycles. Preferred if complete cytoreduction achievable.
2
Neoadjuvant Chemotherapy (NACT)
Carboplatin AUC5 + Paclitaxel 175 mg/m² q3w × 3 cycles. Use when PDS unlikely to achieve CC-0.
3
Interval Debulking Surgery (IDS)
After NACT response assessment → IDS → Adjuvant chemo × 3 cycles → Maintenance PARP inhibitor if eligible.

First-Line Chemotherapy

The standard first-line regimen is carboplatin plus paclitaxel (the ICON3/GOG-158 backbone). Intravenous (IV) administration is standard; intraperitoneal (IP) chemotherapy may be considered in selected patients with optimally debulked disease.

💊
Carboplatin
Paraplatin® · Generic · Platinum agent
Adult dose AUC 5 (Calvert formula) IV q3w × 6 cycles
Paediatric dose Not applicable (adult disease)
Renal adjustment Dose by Calvert formula using eGFR; withhold if GFR <25 mL/min
PBS status ✔ PBS General Benefit
💊
Paclitaxel
Taxol® · Generic · Taxane
Adult dose 175 mg/m² IV over 3 h q3w × 6 cycles
Paediatric dose Not applicable
Renal adjustment No dose adjustment required
PBS status ✔ PBS General Benefit
💊
Bevacizumab
Avastin® · Anti-VEGF monoclonal antibody
Adult dose 15 mg/kg IV q3w, concurrent with chemo then maintenance to 15 months total
Indication Stage III–IV with >1 cm residual or Stage IV; improves PFS
Key toxicity Hypertension, proteinuria, GI perforation, impaired wound healing
PBS status ⚠ PBS Authority Required

Dose-Dense Paclitaxel (Alternative Regimen)

Based on the JGOG 3016 trial, dose-dense paclitaxel (80 mg/m² weekly) with carboplatin AUC 6 q3w may be considered, particularly in patients with high-risk disease. The benefit is debated in non-Japanese populations; discuss in multidisciplinary team (MDT).

PARP Inhibitors — Maintenance Therapy

PARP inhibitors exploit homologous recombination deficiency (HRD) in tumour cells through synthetic lethality. They are now a standard component of first-line maintenance for advanced HGSC in Australia.

💊
Olaparib
Lynparza® · PARP inhibitor
Adult dose 300 mg PO BD, continue for up to 2 years (first-line maintenance) or until progression (relapse)
First-line indication Stage III–IV HGSC with BRCA1/2 mutation or HRD-positive, in CR/PR after platinum-based chemo
Relapse indication Platinum-sensitive relapse with BRCA1/2 mutation, after ≥2 lines platinum chemo
Key toxicity Nausea, fatigue, anaemia, neutropenia; rare MDS/AML risk
PBS status ⚠ PBS Authority Required
💊
Niraparib
Zejula® · PARP inhibitor
Adult dose 200 mg PO OD (or 300 mg if weight >77 kg and platelets >150 × 10⁹/L); up to 3 years
First-line indication Stage III–IV HGSC in CR/PR after platinum chemo (all-comers, not restricted to BRCA/HRD per PRIMA trial)
Key toxicity Thrombocytopenia, nausea, fatigue, insomnia; monitor FBC weekly × 1 month then monthly
PBS status ⚠ PBS Authority Required
🚨
PARP inhibitor eligibility — PBS criteria: Olaparib first-line maintenance requires confirmed BRCA1/2 mutation (germline or somatic) OR HRD-positive status. Niraparib first-line maintenance PBS listing includes broader criteria. Refer to current PBS authority application requirements and provide pathology/genetics reports.

Platinum-Sensitive Relapse

Relapse >6 months after completion of first-line platinum-based chemotherapy is classified as platinum-sensitive. Re-challenge with platinum-based combination chemotherapy is standard:

  • Carboplatin + Gemcitabine: Carboplatin AUC4 D1 + Gemcitabine 1000 mg/m² D1, D8 q3w × 6 cycles (AGO-OVAR 2.5 / GCIG trial)
  • Carboplatin + Pegylated liposomal doxorubicin (PLD): Carboplatin AUC5 D1 + PLD 30 mg/m² D1 q4w × 6 cycles (CALYPSO trial)
  • Carboplatin + Paclitaxel: Standard combination; consider if no significant residual neuropathy

Maintenance olaparib (if BRCA1/2 mutated) or bevacizumab may be added after platinum-based re-induction.

Platinum-Resistant Relapse

Relapse within 6 months of last platinum therapy carries a poor prognosis. Treatment is with single-agent non-platinum chemotherapy:

  • Pegylated liposomal doxorubicin (PLD) 40–50 mg/m² IV q4w
  • Weekly paclitaxel 80 mg/m² IV
  • Topotecan 4 mg/m² IV D1, D8, D15 q4w
  • Gemcitabine 1000 mg/m² D1, D8, D15 q4w

Consider bevacizumab in combination with single-agent chemotherapy (AURELIA trial). Participation in clinical trials is strongly encouraged.

Emerging Therapies

  • Antibody-drug conjugates: Mirvetuximab soravtansine (ELAHERE®) for FRα-positive platinum-resistant disease (FDA approved; TGA evaluation pending)
  • Immunotherapy: Limited activity as monotherapy; combination strategies with PARPi under investigation
  • HIPEC: Hyperthermic intraperitoneal chemotherapy at IDS may improve OS in selected patients (OVHIPEC-1 trial); not yet standard of care in Australia

Monitoring & Surveillance

There is no evidence that routine intensive surveillance with CT imaging improves survival in asymptomatic patients. However, the following approach is standard in Australia:

Every 3 months × 2 years
Clinical review, symptom assessment, CA-125. Consider CT if symptoms suggest recurrence.
Every 4–6 months × years 3–5
Clinical review, CA-125. CT as clinically indicated.
Annually after 5 years
Annual clinical review and CA-125. Discharge to GP-led surveillance if stable.
ℹ️
CA-125 rise without symptoms: The MRC OV05/EORTC 55955 trial showed that early treatment of biochemical recurrence (CA-125 rise without symptoms) does not improve survival and worsens quality of life. Treatment should be initiated based on clinical/radiological progression, not CA-125 alone.

Special Populations

👶 Fertility-Sparing Surgery
Stage IA (IC1) non-serous, Grade 1–2 Unilateral salpingo-oophorectomy (USO) with comprehensive staging acceptable in women wishing to preserve fertility. Completion surgery after childbearing.
Stage IA (IC1) Grade 3 / clear cell Fertility-sparing surgery may be considered with careful counselling regarding higher recurrence risk. MDT discussion mandatory.
Germ cell tumours (all stages) Fertility-sparing surgery is standard of care. Fertility preservation (oocyte/embryo cryopreservation) should be offered before chemotherapy.
🤰 Pregnancy
First trimester diagnosis If early-stage and low-grade, may defer surgery to second trimester. If advanced-stage, urgent MDT discussion including obstetric team. Termination may be recommended if life-threatening disease.
Second/third trimester Surgery can be performed in second trimester. Platinum-based chemotherapy may be given in second/third trimester (avoid taxanes in first trimester due to teratogenicity risk).
👴 Elderly / Frail Patients
Assessment Comprehensive geriatric assessment (CGA) recommended. Performance status (ECOG) alone is insufficient for treatment decisions.
Dose modification Carboplatin monotherapy or reduced-dose carboplatin/paclitaxel if intolerant. Weekly paclitaxel may be better tolerated than 3-weekly.
🫘 Renal Impairment
Carboplatin Dose by Calvert formula using eGFR. Withhold if GFR <25 mL/min. Monitor renal function before each cycle.
Cisplatin Contraindicated if GFR <60 mL/min. Pre-hydration required. Carboplatin preferred in ovarian cancer.
🫁 Hepatic Impairment
Paclitaxel Dose reduction required if bilirubin >1.5× ULN. Avoid if severe hepatic impairment.
PARP inhibitors Caution in moderate hepatic impairment (Child-Pugh B); reduce olaparib to 200 mg BD. Avoid in severe impairment.
🛡️ Immunocompromised
General principles Standard chemotherapy regimens can be given. Increased infection risk; consider G-CSF prophylaxis if febrile neutropenia risk >20%. Ensure PJP prophylaxis if prolonged lymphopenia. COVID-19 and influenza vaccination recommended.

Aboriginal and Torres Strait Islander Health Considerations

Aboriginal and Torres Strait Islander Health
Incidence & mortality
ATSI women have similar incidence of ovarian cancer to non-Indigenous women but experience significantly higher mortality, likely due to later-stage presentation and reduced access to optimal treatment.
Diagnostic delay
Barriers include geographic remoteness, limited access to gynaecological services, cultural factors affecting healthcare engagement, and fewer available imaging and pathology services in remote areas.
Genetic testing access
ATSI women are less likely to receive genetic counselling and BRCA1/2 testing, limiting access to PARP inhibitor therapy. Telehealth genetics services and community-based genetic counselling programmes should be utilised.
Treatment access
Centralised gynaecological oncology services are concentrated in metropolitan centres. ATSI women in remote/very remote areas face long travel distances for surgery and chemotherapy. Patient-assisted travel schemes (PATS) and Cancer Council support services should be offered.
Culturally safe care
Engagement of Aboriginal Health Workers (AHWs), culturally appropriate information resources, acknowledgement of sorry business, and provision of women-only care pathways where preferred.
Recommended actions
Ensure all ATSI women with ovarian cancer are discussed at MDT. Offer genetic testing regardless of age. Facilitate telehealth genetics consultation. Link with Cancer Australia's Optimal Care Pathway for ATSI people with cancer. Provide smoking cessation and chronic disease management support.

Investigations & Australian Lab Availability

Essential CA-125 serum MBS item 66811. Available at all Australian pathology labs. Elevated in ~80% HGSC.
Available HE4 serum MBS item 66814. Available at major reference labs (Sullivan Nicolaides, Douglass Hanly Moir). ROMA score calculable with CA-125 + HE4 + menopausal status.
Essential BRCA1/2 germline testing MBS item 73295. Available through clinical genetics services. Turnaround 2–4 weeks. All non-mucinous epithelial OC patients eligible.
Available BRCA1/2 somatic tumour testing Performed on tumour tissue if germline negative. Available at major pathology centres. Turnaround 3–6 weeks.
Available HRD testing (myChoice CDx or equivalent) Companion diagnostic for niraparib. Available at select reference labs. Requires adequate tumour tissue.
Available Transvaginal ultrasound MBS item 55034. Widely available. IOTA Simple Rules or ADNEX model for risk stratification.
Available CT chest/abdomen/pelvis with contrast MBS item 56800. Standard staging imaging. Assessment of peritoneal disease and operability.
Referral Pelvic MRI MBS item 63537. For characterisation of indeterminate adnexal masses. Specialist referral required.
Specialist PET-CT Not MBS-listed for primary ovarian cancer diagnosis. May be used for suspected recurrence in specialist centres.

📚 References

  1. 1. Cancer Australia. Ovarian Cancer Statistics. Australian Government; 2024. Available at: canceraustralia.gov.au.
  2. 2. Australian Institute of Health and Welfare (AIHW). Cancer data in Australia. Cat. no. CAN 122. Canberra: AIHW; 2023.
  3. 3. Prat J; FIGO Committee on Gynecologic Oncology. Staging classification for cancer of the ovary, fallopian tube, and peritoneum. Int J Gynaecol Obstet. 2014;124(1):1–5.
  4. 4. Vergote I, Tropé CG, Amant F, et al. Neoadjuvant chemotherapy or primary surgery in stage IIIC or IV ovarian cancer. N Engl J Med. 2010;363(10):943–953.
  5. 5. du Bois A, Reuss A, Pujade-Lauraine E, et al. Role of surgical outcome as prognostic factor in advanced epithelial ovarian cancer: a combined exploratory analysis of 3 prospectively randomized phase 3 multicenter trials. Cancer. 2009;115(6):1234–1244.
  6. 6. Moore K, Colombo N, Scambia G, et al. Maintenance olaparib in newly diagnosed advanced ovarian cancer. N Engl J Med. 2018;379(26):2495–2505 (SOLO-1).
  7. 7. González-Martín A, Pothuri B, Vergote I, et al. Niraparib in patients with newly diagnosed advanced ovarian cancer. N Engl J Med. 2019;381(25):2391–2402 (PRIMA).
  8. 8. Burger RA, Brady MF, Bookman MA, et al. Incorporation of bevacizumab in the primary treatment of ovarian cancer. N Engl J Med. 2011;365(26):2473–2483 (GOG-218).
  9. 9. Pujade-Lauraine E, Hilpert F, Weber B, et al. Bevacizumab combined with chemotherapy for platinum-resistant recurrent ovarian cancer: the AURELIA open-label randomized phase III trial. J Clin Oncol. 2014;32(13):1302–1308.
  10. 10. Rustin GJ, van der Burg ME, Griffin CL, et al. Early versus delayed treatment of relapsed ovarian cancer (MRC OV05/EORTC 55955). Lancet. 2010;376(9747):1155–1163.
  11. 11. van Driel WJ, Koole SN, Sikorska K, et al. Hyperthermic intraperitoneal chemotherapy in ovarian cancer. N Engl J Med. 2018;378(3):230–240 (OVHIPEC-1).
  12. 12. RANZCOG. College Statement: Management of women identified at increased risk of ovarian cancer. C-Obs 43. Melbourne: RANZCOG; 2023.
  13. 13. Cancer Australia. National Framework for Gynaecological Cancer Control. Surry Hills: Cancer Australia; 2022.
  14. 14. National Health and Medical Research Council (NHMRC). Clinical Practice Guidelines for the Management of Ovarian Cancer in Australia. Canberra: NHMRC; 2004 (under review).
  15. 15. Australian Commission on Safety and Quality in Health Care (ACSQHC). Australian Cancer Care Pathways: Ovarian Cancer. Sydney: ACSQHC; 2023.
for PBS-listed medicines at participating pharmacies.
Cultural safety
Engagement with Aboriginal Community Controlled Health Organisations (ACCHOs) is essential. Cultural safety training for non-Indigenous clinicians, use of Aboriginal Health Workers and Liaison Officers, and incorporation of traditional healing practices alongside Western medicine improve treatment adherence and outcomes. Avoidance of eye contact, respect for gender-sensitive examination practices, and understanding of sorry business protocols are critical elements of culturally safe care.
Medication adherence
Complex DMARD regimens with frequent monitoring requirements present adherence challenges. Long-acting depot injections (e.g., methotrexate SC) may improve adherence compared to oral regimens. Community pharmacy partnerships through the Indigenous Pharmacy Programmes improve medication management.
Specific conditions
Rheumatic heart disease (RHD) requires secondary prophylaxis with benzathine penicillin G (BPG) 1.2 MU IM every 3–4 weeks for a minimum of 10 years or until age 21 (whichever is longer). RHD registers (e.g., NT RHD Register) facilitate recall and follow-up. The Australian RHD Endgame Strategy targets elimination by 2031.
Referral pathways
Referral through ACCHOs and Aboriginal Hospital Liaison Officers (AHLOs) improves engagement. The Specialist Outreach Assistance Programme provides funded specialist visits to remote communities. NT, WA, and QLD have specific rheumatology outreach programmes targeting Indigenous communities.

📚 References

  1. 1. Australian Institute of Health and Welfare (AIHW). Autoimmune disease in Australia. Cat. no. PHE 312. Canberra: AIHW; 2023.
  2. 2. Fraenkel L, Bathon JM, England BR, et al. 2021 American College of Rheumatology guideline for the treatment of rheumatoid arthritis. Arthritis Care Res. 2021;73(7):924–939.
  3. 3. Fanouriakis A, Kostopoulou M, Alber K, et al. 2019 update of the EULAR recommendations for the management of systemic lupus erythematosus. Ann Rheum Dis. 2019;78(6):736–745.
  4. 4. Chung SA, Langford CA, Maz M, et al. 2021 American College of Rheumatology/Vasculitis Foundation guideline for the management of antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Care Res. 2021;73(11):1583–1599.
  5. 5. Smolen JS, Landewé RBM, Bijlsma JWJ, et al. EULAR recommendations for the management of rheumatoid arthritis with synthetic and biological disease-modifying antirheumatic drugs: 2022 update. Ann Rheum Dis. 2023;82(1):3–18.
  6. 6. Australian Technical Advisory Group on Immunisation (ATAGI). Australian Immunisation Handbook. Australian Government Department of Health; 2024. Available from: immunisationhandbook.health.gov.au.
  7. 7. Rheumatic Heart Disease Australia (RHDAustralia). The 2020 Australian guideline for prevention, diagnosis, and management of acute rheumatic fever and rheumatic heart disease. 3rd ed. Darwin: Menzies School of Health Research; 2020.
  8. 8. Pharmaceutical Benefits Scheme (PBS). PBS Schedule. Australian Government Department of Health. Available from: pbs.gov.au. Accessed 2024.
  9. 9. Agarwal S, Cunnington J, Nossent J. Autoimmune disease in Indigenous Australians: a systematic review. Int J Rheum Dis. 2021;24(12):1487–1498.
  10. 10. Pisetsky DS. Antinuclear antibody testing — misunderstood or misused? Clin Immunol. 2023;255:109717.
  11. 11. Bertsias GK, Tektonidou M, Amoura Z, et al. Joint European League Against Rheumatism and European Renal Association–European Dialysis and Transplant Association (EULAR/ERA-EDTA) recommendations for the management of adult and paediatric lupus nephritis. Ann Rheum Dis. 2012;71(11):1771–1782.
  12. 12. Ledingham J, Deighton C; British Society for Rheumatology Standards, Audit and Guidelines Working Group. Update on the British Society for Rheumatology guidelines for prescribing TNFα blockers in adults with rheumatoid arthritis. Rheumatology. 2005;44(2):155–158.
  13. 13. National Health and Medical Research Council (NHMRC). National statement on ethical conduct in human research. Canberra: NHMRC; 2023 (updated).
for PBS-listed medicines at participating pharmacies.
Cultural safety
Engagement with Aboriginal Community Controlled Health Organisations (ACCHOs) is essential. Cultural safety training for non-Indigenous clinicians, use of Aboriginal Health Workers and Liaison Officers, and incorporation of traditional healing practices alongside Western medicine improve treatment adherence and outcomes. Avoidance of eye contact, respect for gender-sensitive examination practices, and understanding of sorry business protocols are critical elements of culturally safe care.
Medication adherence
Complex DMARD regimens with frequent monitoring requirements present adherence challenges. Long-acting depot injections (e.g., methotrexate SC) may improve adherence compared to oral regimens. Community pharmacy partnerships through the Indigenous Pharmacy Programmes improve medication management.
Specific conditions
Rheumatic heart disease (RHD) requires secondary prophylaxis with benzathine penicillin G (BPG) 1.2 MU IM every 3–4 weeks for a minimum of 10 years or until age 21 (whichever is longer). RHD registers (e.g., NT RHD Register) facilitate recall and follow-up. The Australian RHD Endgame Strategy targets elimination by 2031.
Referral pathways
Referral through ACCHOs and Aboriginal Hospital Liaison Officers (AHLOs) improves engagement. The Specialist Outreach Assistance Programme provides funded specialist visits to remote communities. NT, WA, and QLD have specific rheumatology outreach programmes targeting Indigenous communities.

📚 References

  1. 1. Australian Institute of Health and Welfare (AIHW). Autoimmune disease in Australia. Cat. no. PHE 312. Canberra: AIHW; 2023.
  2. 2. Fraenkel L, Bathon JM, England BR, et al. 2021 American College of Rheumatology guideline for the treatment of rheumatoid arthritis. Arthritis Care Res. 2021;73(7):924–939.
  3. 3. Fanouriakis A, Kostopoulou M, Alber K, et al. 2019 update of the EULAR recommendations for the management of systemic lupus erythematosus. Ann Rheum Dis. 2019;78(6):736–745.
  4. 4. Chung SA, Langford CA, Maz M, et al. 2021 American College of Rheumatology/Vasculitis Foundation guideline for the management of antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Care Res. 2021;73(11):1583–1599.
  5. 5. Smolen JS, Landewé RBM, Bijlsma JWJ, et al. EULAR recommendations for the management of rheumatoid arthritis with synthetic and biological disease-modifying antirheumatic drugs: 2022 update. Ann Rheum Dis. 2023;82(1):3–18.
  6. 6. Australian Technical Advisory Group on Immunisation (ATAGI). Australian Immunisation Handbook. Australian Government Department of Health; 2024. Available from: immunisationhandbook.health.gov.au.
  7. 7. Rheumatic Heart Disease Australia (RHDAustralia). The 2020 Australian guideline for prevention, diagnosis, and management of acute rheumatic fever and rheumatic heart disease. 3rd ed. Darwin: Menzies School of Health Research; 2020.
  8. 8. Pharmaceutical Benefits Scheme (PBS). PBS Schedule. Australian Government Department of Health. Available from: pbs.gov.au. Accessed 2024.
  9. 9. Agarwal S, Cunnington J, Nossent J. Autoimmune disease in Indigenous Australians: a systematic review. Int J Rheum Dis. 2021;24(12):1487–1498.
  10. 10. Pisetsky DS. Antinuclear antibody testing — misunderstood or misused? Clin Immunol. 2023;255:109717.
  11. 11. Bertsias GK, Tektonidou M, Amoura Z, et al. Joint European League Against Rheumatism and European Renal Association–European Dialysis and Transplant Association (EULAR/ERA-EDTA) recommendations for the management of adult and paediatric lupus nephritis. Ann Rheum Dis. 2012;71(11):1771–1782.
  12. 12. Ledingham J, Deighton C; British Society for Rheumatology Standards, Audit and Guidelines Working Group. Update on the British Society for Rheumatology guidelines for prescribing TNFα blockers in adults with rheumatoid arthritis. Rheumatology. 2005;44(2):155–158.
  13. 13. National Health and Medical Research Council (NHMRC). National statement on ethical conduct in human research. Canberra: NHMRC; 2023 (updated).