Adult-onset Still disease

Adult-onset Still disease (AOSD) is a rare systemic autoinflammatory disorder characterised by the classic triad of quotidian (daily spiking) fever, evanescent salmon-pink rash, and arthritis or arthralgia. It is considered the adult counterpart of systemic juvenile idiopathic arthritis (sJIA) and belongs to the spectrum of autoinflammatory conditions driven by innate immune dysregulation rather than autoantibody-mediated mechanisms. First described by Eric Bywaters in 1971 in a cohort of adults with clinical features resembling those originally described by George Still in children in 1897, AOSD remains an exclusion diagnosis requiring systematic elimination of infectious, malignant, and other inflammatory conditions.

In Australia, AOSD is estimated to affect approximately 1–34 per 1,000,000 adults annually, with a bimodal age distribution peaking at 15–25 years and 36–46 years. There is a slight female predominance in the younger peak. The condition is managed primarily by rheumatologists in tertiary centres, though general practitioners play a crucial role in recognition, initial investigation, and long-term co-management. The course of AOSD is heterogeneous: approximately one-third of patients experience a self-limiting monocyclic course resolving within 12 months, one-third have a polycyclic pattern with intermittent flares separated by periods of remission, and one-third develop a chronic articular-dominant pattern with persistent destructive arthropathy.

Disease Patterns

AOSD is fundamentally a disorder of innate immune dysregulation, driven by an exaggerated inflammatory response mediated predominantly through the NLRP3 inflammasome and pro-inflammatory cytokines — particularly interleukin-1β (IL-1β), interleukin-18 (IL-18), and interleukin-6 (IL-6). Unlike classical autoimmune diseases, antinuclear antibodies and rheumatoid factor are absent (or only transiently and weakly positive), and T-cell receptor rearrangements typical of lymphoproliferative disorders are not found.

Key Cytokine Pathways

• IL-1β: Central mediator — drives fever, acute phase response, neutrophil activation, and MAS susceptibility. Activated via NLRP3 inflammasome in response to danger signals (DAMPs). Elevated in serum and synovial fluid of AOSD patients. The therapeutic efficacy of IL-1 blockade (anakinra, canakinumab) validates this pathway.
• IL-18: Extremely elevated in AOSD (often >10,000 pg/mL) — one of the highest levels seen in any inflammatory condition. IL-18 drives interferon-gamma (IFN-γ) production from NK and T-cells, contributing to macrophage activation, haemophagocytosis, and MAS. The IL-18/IL-18BP (binding protein) ratio is markedly elevated, reflecting free bioactive IL-18.
• IL-6: Contributes to acute-phase response including extreme hyperferritinaemia via stimulation of ferritin synthesis in the liver and macrophages. IL-6 blockade with tocilizumab can be effective, particularly in the chronic articular pattern.
• S100A8/A9 (calprotectin) and S100A12: Alarmin proteins released by activated neutrophils and monocytes. Act as endogenous NLRP3 activators. Serum S100A8/A9 levels are dramatically elevated in AOSD and correlate with disease activity, particularly useful in monitoring.
• Ferritin: Produced by macrophages and hepatocytes under IL-6/IL-1 stimulation. Serves as an acute phase reactant and also has direct immunomodulatory activity (suppresses NK cell function, promoting further immune dysregulation). Glycosylated ferritin fraction is characteristically low (<20%) in AOSD, reflecting saturation of glycosylation pathways.

Genetic and Environmental Triggers

No single causative gene has been identified. HLA associations (HLA-DR2, HLA-DR5, HLA-B17, HLA-B35) suggest a polygenic predisposition. Environmental triggers including viral infections (EBV, CMV, rubella, parvovirus B19, adenovirus, Yersinia, Borrelia) may precipitate the initial episode in genetically susceptible individuals by activating innate immune pathways through pattern recognition receptors (TLRs). However, no infectious aetiology is found in the majority of AOSD cases at presentation.

Macrophage Activation Syndrome — Pathomechanism

MAS in AOSD results from uncontrolled activation and proliferation of macrophages and cytotoxic T-lymphocytes, with defective NK-cell cytotoxic function (often due to mutations in perforin pathway genes in some cases). Haemophagocytosis occurs in the bone marrow, spleen, and lymph nodes. The resulting cytokine storm — predominantly driven by IL-18, IFN-γ, and IL-6 — produces profound hyperferritinaemia, cytopenias, coagulopathy, and multi-organ dysfunction. MAS is a life-threatening complication occurring in 7–17% of AOSD patients.

Cardinal Clinical Features

Fever in Adult-Onset Still Disease

Fever occurs in virtually all patients (99%) and is typically quotidian — spiking to ≥39°C (often 39–41°C) once or twice daily, predominantly in the late afternoon or early evening, then returning to normal or subnormal. This pattern is characteristic and clinically helpful. Fever is often accompanied by worsening of rash, rigors, sore throat, myalgia, and arthralgia. Patients feel relatively well between spikes, which helps distinguish AOSD from sepsis (where fever is more sustained and patients remain systemically unwell). Documenting a fever diary (home thermometer, twice daily) is diagnostically valuable.

Rash in Adult-Onset Still Disease

The characteristic rash is evanescent, salmon-pink (pale orange-pink), macular or maculopapular, non-pruritic (or mildly so), typically distributed on the trunk, proximal limbs, and occasionally the face. Crucially, the rash appears and fades with the fever spikes — it is often present only during febrile episodes and absent between spikes. Patients and clinicians may miss the rash entirely if not specifically looked for during febrile periods. Koebner phenomenon (isomorphic response) may be present. The rash may have a linear or dermographic quality. Urticaria-like lesions can occasionally occur.

Arthritis and Arthralgia in Adult-Onset Still Disease

Joint involvement ranges from arthralgia alone to frank inflammatory arthritis. Arthritis may be mono-, oligo-, or polyarticular. The most commonly affected joints are the knees, wrists, ankles, and proximal interphalangeal joints. Wrist arthritis is particularly characteristic and can be bilateral. In the chronic articular pattern, a destructive carpal ankylosis may develop, producing a characteristic "bamboo spine-like" wrist appearance on radiograph. Hip involvement may lead to aseptic necrosis or rapid joint destruction, requiring arthroplasty. Morning stiffness is common.

Rarer Clinical Features of Adult-Onset Still Disease

• Sore throat / pharyngitis (76–92%): Non-exudative pharyngitis, often the first symptom. May lead to misdiagnosis as pharyngitis or viral upper respiratory tract infection.
• Lymphadenopathy (44–74%): Generalised or cervical. Lymph node biopsy shows reactive hyperplasia — may be confused with lymphoma (hence biopsy often required to exclude).
• Hepatosplenomegaly (44–65%): Hepatomegaly with elevated transaminases common. Rarely, severe hepatitis or hepatic failure can occur (associated with MAS or direct drug toxicity).
• Myalgia (84%): Severe myalgia accompanying fever spikes. Correlates with CK elevation in a minority; CK dramatically elevated suggests MAS or myositis overlap.
• Weight loss: Significant in active disease due to catabolic state.

Abdominal and Chest Pain in Adult-Onset Still Disease

Serositis — pleuritis, pericarditis, and peritonitis — occurs in approximately 20–30% of patients. Pericarditis may present as pleuritic chest pain and is confirmed by echocardiography showing pericardial effusion; myocarditis and tamponade are rare but life-threatening. Pleuritis produces characteristic pleuritic chest pain and pleural effusions (exudative). Peritonitis manifests as abdominal pain and tenderness, sometimes leading to unnecessary surgical exploration — AOSD should be considered in unexplained serositis. Pulmonary infiltrates, pleural effusions, and rarely acute respiratory distress syndrome (ARDS) can complicate severe AOSD or MAS.

Diagnostic Criteria — Yamaguchi Criteria (1992)

The Yamaguchi criteria are the most widely used and sensitive (93.5%) for AOSD. Diagnosis requires ≥5 criteria with ≥2 major criteria, after exclusion of infections, malignancies, and other rheumatic diseases.

Investigations

• Essential
  Serum Ferritin (total and glycosylated fraction)

  Total ferritin typically >5× upper limit of normal; often >10,000 μg/L in active AOSD. Glycosylated ferritin <20% of total ferritin is highly specific for AOSD (sensitivity 43%, specificity 93%). Available at major Australian laboratories (SEALS, PathWest, Melbourne Pathology). Serial monitoring guides treatment response.
• Essential
  Full Blood Count + Differential

  Leukocytosis ≥10,000/mm³ with neutrophilia (≥80% granulocytes) is characteristic. Anaemia of chronic disease common. Thrombocytopenia in MAS (falling platelets — key warning sign). Eosinopenia supports inflammatory state.
• Essential
  Inflammatory Markers: CRP, ESR, LDH

  CRP markedly elevated. ESR elevated. LDH very high — reflects macrophage activation and tissue destruction. Disproportionate elevation of ferritin relative to CRP/ESR is characteristic. LDH >2000 IU/L with falling platelet count should prompt MAS evaluation.
• Essential
  Liver Function Tests + Coagulation Screen

  Transaminase elevation (AST, ALT) in 50–75%. Coagulation (PT, APTT, fibrinogen, D-dimer) mandatory to screen for DIC in MAS. Hypofibrinogenaemia (falling fibrinogen) is an early MAS marker.
• Essential
  Autoantibody Screen: ANA, RF, anti-CCP, ANCA, anti-dsDNA, complement (C3, C4)

  Should be negative or only weakly positive in AOSD. Positive high-titre ANA or anti-dsDNA suggests SLE. Positive RF/anti-CCP suggests RA. Complement consumption (low C3/C4) points to SLE or vasculitis. Anti-Smith, anti-Ro/La, anti-Sm help exclude SLE.
• Essential
  Infectious Serology: EBV, CMV, Parvovirus B19, HIV, Hepatitis B & C, Blood cultures ×3

  Mandatory exclusion of infectious mimics. EBV and CMV can produce fever, rash, lymphadenopathy, splenomegaly, and elevated ferritin. Parvovirus B19 causes arthritis with rash. Three sets of blood cultures to exclude bacteraemia. Throat swab if pharyngitis present.
• Essential
  Bone Marrow Biopsy + Trephine

  Required in most cases to exclude lymphoma (particularly T-cell lymphoma, which can be an AOSD mimic or rare complication) and haematological malignancy. Demonstrates reactive hyperplasia in AOSD. Haemophagocytosis if MAS present. Available at major Australian haematology centres.
• Recommended
  FDG-PET/CT Scan

  Highly sensitive for detecting AOSD activity (intense FDG uptake in spleen, liver, lymph nodes, bone marrow reflecting active inflammation). Also assists in excluding lymphoma and occult malignancy. Available at PET centres in major Australian cities; Medicare rebatable under specific criteria.
• Recommended
  Echocardiography

  To detect pericardial effusion, myocarditis, and assess cardiac function in patients with chest pain or dyspnoea. Urgently indicated if haemodynamic compromise or tamponade suspected.
• Recommended
  Serum IL-18 and S100A8/S100A9 (Calprotectin)

  Emerging biomarkers with high specificity for AOSD. IL-18 levels often >10,000 pg/mL (reference <200 pg/mL). Available at selected Australian research laboratories (Royal Melbourne Hospital, SEALS). Useful for monitoring and predicting MAS.

Macrophage Activation Syndrome in Adult-Onset Still Disease

MAS is the most feared complication of AOSD. It represents a form of secondary haemophagocytic lymphohistiocytosis (sHLH) driven by the same cytokine storm (IL-18, IFN-γ) that underlies AOSD itself, but in an uncontrolled, life-threatening form. Risk factors for MAS in AOSD include: markedly elevated ferritin (>10,000 μg/L), high LDH, thrombocytopenia, lymphopenia, elevated IL-18, and failure to respond to initial corticosteroids.

HScore — Probability of MAS/HLH

The HScore quantifies the probability of HLH/MAS based on 9 clinical and laboratory parameters (temperature, organomegaly, cytopenias, ferritin, triglycerides, fibrinogen, haemophagocytosis on BM biopsy, immunosuppressed state, AST). An HScore >169 carries >93% probability of HLH/MAS. Available via online calculator (mdcalc.com). All AOSD patients should be monitored for MAS features at each review.

AOSD Disease Activity Score

The Pouchot score is the validated disease activity measure for AOSD, incorporating 12 clinical and laboratory variables. A modified Pouchot score ≥7 indicates highly active disease. While not routinely used in all Australian centres, it provides a structured framework for treatment escalation decisions and clinical trial enrolment.

MAS Diagnostic Criteria (2016 Classification — Ravelli et al.)

Adapted for use in AOSD: ferritin >684 μg/L AND any two of the following — platelet count ≤181×10⁹/L; AST >48 IU/L; triglycerides >1.76 mmol/L; fibrinogen ≤3.6 g/L. Bone marrow haemophagocytosis supports but is not required for diagnosis if clinical and laboratory criteria are met.

Treatment Overview

Management of AOSD follows a step-up approach based on disease severity, pattern (systemic vs articular-dominant), and response to initial therapy. The goal is to rapidly control systemic inflammation, prevent organ damage (particularly MAS), and achieve sustained remission with the lowest effective medication dose. All treatment decisions should be made in collaboration with a rheumatologist. Regular monitoring for adverse effects of immunosuppressive therapy is mandatory.

Step-Up Treatment Algorithm

Symptomatic Treatment for Systemic Symptoms

Symptomatic measures complement disease-modifying therapy and are important for patient comfort and quality of life during active disease flares.

• Fever management: Paracetamol 1 g QID (preferred over NSAIDs alone for fever if renal or GI concerns). NSAIDs (naproxen 500 mg BD, ibuprofen 400–600 mg TDS) effective for fever and arthralgia when GI risk acceptable. Avoid aspirin (Reye syndrome risk not applicable to adults, but inferior efficacy).
• Pain management: NSAIDs as above. Avoid regular opioids for inflammatory arthritis. Intra-articular corticosteroid injections for predominantly oligoarticular disease can provide effective local control.
• Osteoporosis prophylaxis: Mandatory when corticosteroids used for >3 months. Calcium 1200 mg/day + vitamin D 1000–2000 IU/day. Bisphosphonate (alendronate 70 mg/week or zoledronate 5 mg/year IV) per osteoporosis guidelines. DEXA scan at baseline and 12 monthly.
• PPI prophylaxis: Mandatory with concurrent NSAID + corticosteroid use. Omeprazole 20 mg daily or pantoprazole 40 mg daily.
• VTE prophylaxis: During hospitalisation for active AOSD — LMWH (enoxaparin 40 mg SC daily) per VTE prophylaxis protocol.

Biologic and targeted synthetic disease-modifying antirheumatic drugs (b/tsDMARDs) are increasingly used for AOSD refractory to corticosteroids and conventional DMARDs. The choice of biologic is guided by the predominant disease pattern (systemic vs articular), prior treatment failure, comorbidities, and PBS availability. All biologics require specialist initiation and monitoring, screening for latent TB, hepatitis B/C, and other infections before use.

JAK Inhibitors — Emerging Evidence

Tofacitinib (Xeljanz®, 5 mg BD) and baricitinib (Olumiant®, 2–4 mg daily) have shown efficacy in small series and case reports of refractory AOSD. JAK inhibitors suppress multiple pro-inflammatory cytokine signalling pathways simultaneously (JAK1/3 or JAK1/2), potentially useful in AOSD where multiple cytokines contribute. Not PBS-listed for AOSD; evidence base growing. Consider in refractory cases after IL-1 and IL-6 inhibitor failure under specialist guidance. Screen for TB, hepatitis B, varicella immunity, lipids, and absolute lymphocyte count before initiation.

Management of Acute AOSD Flare

An acute AOSD flare presenting with high fever, severe arthritis, rash, and markedly elevated inflammatory markers requires urgent clinical assessment to exclude infection and MAS before escalating immunosuppression.

Excluding Bacterial Sepsis and Other Differential Diagnoses

The single greatest diagnostic challenge in AOSD is distinguishing it from bacterial sepsis. Both present with high fever, elevated CRP, neutrophilia, and systemic toxicity. Key differentiating features include:

Other important differential diagnoses to exclude: lymphoma (particularly T-cell lymphoma — requires BM biopsy and lymph node biopsy), systemic lupus erythematosus (ANA, anti-dsDNA, complement), viral infections (EBV, CMV, parvovirus — serology), sarcoidosis (chest CT, serum ACE, biopsy), reactive arthritis (preceding infection history, HLA-B27, microbiology), vasculitis (ANCA, biopsy), drug reaction (DRESS — drug exposure history, eosinophilia).

Disease Activity Monitoring

MAS Warning Signs — Escalate Urgently

• Ferritin >10,000 μg/L or rapidly rising ferritin (doubling within 48 hours)
• Platelet count falling (particularly <100×10⁹/L from previously normal or high levels)
• Fibrinogen <3.6 g/L or falling
• LDH >2000 IU/L
• Triglycerides >3 mmol/L
• New or worsening cytopenias (paradoxical leucopenia in known AOSD with leucocytosis)
• Unexplained hepatic dysfunction
• Haemophagocytosis on BM biopsy

Pregnancy and AOSD

• AOSD may improve, worsen, or remain unchanged during pregnancy. New-onset AOSD can present in pregnancy — diagnosis is challenging given physiological changes in inflammatory markers.
• NSAIDs: Acceptable in first and second trimesters; avoid from 32 weeks (premature ductus arteriosus closure, oligohydramnios).
• Corticosteroids: Safe at lowest effective dose. Increased risk of gestational diabetes, hypertension, preterm birth. Monitor blood glucose closely.
• Methotrexate: Absolutely contraindicated in pregnancy and breastfeeding. Cease ≥3 months before planned conception in both men and women.
• Hydroxychloroquine: Safe in pregnancy and breastfeeding. Continue if needed for disease control.
• Anakinra: Limited human data. Animal studies show no teratogenicity. Use only if benefits outweigh risks; discontinue at 20 weeks if possible. Not recommended in breastfeeding.
• Tocilizumab: Contraindicated in pregnancy. Effective contraception required.

Elderly Patients

• AOSD in the elderly (≥60 years) has a higher frequency of atypical presentations with less prominent rash and arthritis but more organomegaly and systemic features.
• Higher risk of MAS and serious infections on immunosuppressive therapy. Lower doses of corticosteroids and DMARDs, with careful monitoring.
• Malignancy (particularly lymphoma) is more prevalent in this age group and must be rigorously excluded before immunosuppression is initiated.
• Bisphosphonate therapy for corticosteroid-induced osteoporosis is particularly important; IV zoledronate preferred if oral compliance is a concern.

Immunocompromised Patients

• AOSD in patients with pre-existing immunosuppression (organ transplant, HIV, haematological malignancy) requires extreme caution in further immunosuppression. Infection must be definitively excluded.
• Live vaccines contraindicated on biologic therapy. Ensure vaccinations (influenza, pneumococcal, COVID-19, herpes zoster) up to date before biologic initiation.

Renal Impairment

• NSAIDs: Avoid in eGFR <30 mL/min; use with caution in eGFR 30–60 mL/min with close monitoring.
• Methotrexate: Avoid in eGFR <30 mL/min; reduce dose and increase monitoring in eGFR 30–60 mL/min.
• Anakinra: Dose reduce in eGFR <30 mL/min; avoid in dialysis-dependent patients.
• Tocilizumab: No dose adjustment required for mild-moderate renal impairment.

The ACSQHC National Safety and Quality Health Service Standards (NSQHS Standard 4 — Medication Safety) and the principles of appropriate immunosuppression stewardship underpin the management of AOSD. Given that AOSD requires potent immunosuppressive agents — corticosteroids, conventional DMARDs, and biologics — with significant toxicity profiles, careful stewardship is essential to maximise clinical benefit while minimising harm.

Principles of Immunosuppression Stewardship in AOSD

• Infection exclusion before treatment escalation: Never escalate immunosuppression without a systematic infection screen. Sepsis and AOSD may coexist. Document the rationale for immunosuppression in the medical record.
• Corticosteroid minimisation: Corticosteroids should be tapered to the lowest effective dose as rapidly as clinically feasible (typically 10% reduction per week when disease controlled). Prolonged high-dose corticosteroids lead to significant morbidity (osteoporosis, diabetes, cardiovascular risk, adrenal insufficiency, infection). Use steroid-sparing agents early.
• Biologic initiation: Require specialist rheumatologist initiation with documented indication, baseline infection screening, vaccination review, and consent. Adhere to TGA registration and PBS criteria where applicable. Document off-label use with rationale and patient consent.
• Treatment tapering and discontinuation: Attempt DMARD taper after ≥12 months of sustained clinical remission (monocyclic pattern). Monitor closely for flare on reduction. Abrupt cessation of biologics carries risk of rebound flare.
• Vaccination: Influenza (annual), pneumococcal (5-yearly), COVID-19 (per ATAGI schedule), hepatitis B (if non-immune), and varicella zoster vaccine before commencing biologics. Live vaccines contraindicated on biologics.
• Opportunistic infection prophylaxis: Pneumocystis jirovecii pneumonia (PJP) prophylaxis with trimethoprim-sulfamethoxazole (Bactrim DS three times weekly) when prednisolone ≥20 mg/day for >4 weeks AND on another immunosuppressant. CMV monitoring in high-risk patients on high-dose immunosuppression.

Follow-Up Schedule

The frequency of follow-up depends on disease activity, treatment phase, and medication monitoring requirements. All patients with AOSD should have a clearly documented management plan shared between the rheumatologist, GP, and patient.

• Active disease / Treatment initiation: Rheumatology review every 2–4 weeks until disease controlled and treatment stabilised. Urgent access pathway for fever recurrence or MAS concern.
• Stable on DMARD therapy: Rheumatology every 3 months. GP every 4–6 weeks for medication monitoring (FBC, LFTs, renal function per DMARD). Shared care letter to GP documenting monitoring obligations.
• Sustained remission (>12 months): Rheumatology 6-monthly. Discuss biologic or DMARD taper. Annual FBC, ferritin, CRP, LFTs, renal function, DEXA (if on long-term corticosteroids).
• Chronic articular AOSD: Annual joint radiography (hands, wrists, feet, hips) to monitor for erosive progression. Consider DAS28 / Pouchot score at each visit. Physiotherapy referral for joint rehabilitation and exercise programme.

Prognosis and Patient Education

Patients should be informed of the three disease patterns and that prognosis is variable. Key education points include: recognition of fever diary keeping and symptom documentation; early presentation if fever recurs or MAS warning signs develop; importance of medication adherence and not self-cessating corticosteroids or DMARDs; sun protection and skin care (corticosteroid skin fragility); dietary advice for corticosteroid-induced weight gain and hyperglycaemia; and the importance of regular GP surveillance monitoring.

Patient support resources in Australia include Arthritis Australia (arthritisaustralia.com.au), which provides patient information on rare inflammatory arthritis. Online support communities and rare disease organisations (Rare Voices Australia) provide peer support for patients with AOSD. Psychosocial support — including referral to a psychologist or social worker — should be offered, as the uncertainty and chronic nature of AOSD significantly impacts quality of life.

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