Systemic Lupus Erythematosus (SLE) is a multisystem autoimmune disease characterised by immune complex deposition and widespread organ inflammation. It predominantly affects women of childbearing age (F:M ratio = 9:1).
Low C3/C4 = active disease (consumed by immune complexes). Rising anti-dsDNA + falling complement = impending flare.
Entry criterion: ANA titre ≥1:80. Score ≥10 = SLE classification.
| Domain | Criterion | Score |
|---|---|---|
| Constitutional | Fever | 2 |
| Mucocutaneous | Malar rash / photosensitivity | 6 |
| Discoid rash | 4 | |
| Oral ulcers / alopecia | 2 | |
| Arthritis | Synovitis ≥2 joints | 6 |
| Neuropsychiatric | Seizures / psychosis / mononeuritis multiplex | 5 |
| Serosal | Pleuritis / pericarditis | 5 |
| Renal | Nephritis — proteinuria / haematuria / renal biopsy | 8–10 |
| Haematological | Haemolytic anaemia / leucopenia / thrombocytopenia | 3–4 |
| Immunological | Anti-dsDNA / Anti-Smith / antiphospholipid Ab | 6 |
| Complement | Low C3 / Low C4 | 3–4 |
The SLE Disease Activity Index (SLEDAI) quantifies disease activity across organ systems. Scores are weighted — renal and CNS manifestations carry highest weight.
Inactive disease / remission
Target for most patients on maintenance therapy
Mild activity
Monitor closely, consider optimising hydroxychloroquine
Moderate–Severe flare
Escalate therapy — consider IV methylprednisolone, specialist review
Most serious complication of SLE. Affects 40–60% of SLE patients. Leading cause of long-term morbidity.
Triad: Thrombosis (arterial/venous) + recurrent miscarriage + positive antiphospholipid antibodies (anticardiolipin, lupus anticoagulant, anti-β2GPI).
Hydroxychloroquine is first-line for ALL SLE patients (unless contraindicated) — reduces flares, nephritis risk, and mortality.
SLE prevalence is higher in Arab and South Asian women living in the GCC — ethnic predisposition compounded by environmental factors.
Sun exposure in the GCC is extreme year-round. UV light is a potent SLE flare trigger. Sun avoidance education is critical — SPF 50+, protective clothing, avoid peak sun (10am–4pm). Many patients are initially reluctant to limit outdoor activity.
Rheumatoid Arthritis is a chronic symmetrical inflammatory polyarthritis causing synovial inflammation, cartilage destruction, and bone erosion. Predominantly affects MCP and PIP joints (spares DIP — distinguishes from OA).
DAS28 uses 28-joint count (tender + swollen joints), ESR or CRP, and patient global assessment (VAS 0–100mm).
Treat-to-target: Aim for remission (DAS28 <2.6) or low disease activity. Review every 1–3 months until target achieved.
Aorta and major branches. GCA: age >50, temporal artery. Takayasu: young women, subclavian involvement — pulseless disease.
PAN: HBV-associated, renal/mesenteric arteries. Kawasaki: children — coronary artery aneurysm risk, IVIG treatment.
cANCA/PR3 → GPA; pANCA/MPO → MPA or EGPA. Renal, pulmonary, ENT involvement.
OPHTHALMIC EMERGENCY — visual loss is irreversible.
Do NOT wait for biopsy result before starting steroids. Start high-dose prednisolone immediately.
Upper/lower airway + renal. Saddle-nose deformity, sinusitis, haemoptysis, rapidly progressive GN. cANCA/PR3.
Asthma + eosinophilia + vasculitis. pANCA/MPO. Cardiac involvement common. Peripheral neuropathy.
High-risk chemotherapy agent. Requires specialist nursing protocols, monitoring, and patient education.
Acrolein metabolite causes haemorrhagic cystitis. Prevent with:
Nadir at 10–14 days post-IV dose.
CREST syndrome:
Anti-centromere antibody. PAH is leading cause of death in lcSSc.
Widespread skin involvement proximal to elbows/knees. Rapid progression. Anti-topoisomerase I (anti-Scl-70).
ILD is leading cause of death in dcSSc. Scleroderma renal crisis risk higher.
Digital vasospasm triggered by cold or emotional stress. Phases: white (ischaemia) → blue (cyanosis) → red (reperfusion).
In GCC: air conditioning exposure is a common and overlooked Raynaud trigger. Patients should carry warm wraps to offices and shopping malls.
Medical emergency: Rapidly rising BP (often >150/90 acutely) + AKI in dcSSc.
Typically within first 4 years of diffuse SSc. High-dose steroids (>15mg/day) are a precipitant — avoid if possible.
Hypertension in any SSc patient = check creatinine + urinalysis immediately. Renal crisis can present without severe hypertension occasionally.
Pulmonary complications are the leading cause of mortality in SSc. ILD (especially dcSSc) and PAH (especially lcSSc) must be screened for regularly.
Very painful ulcers on fingertips — risk of gangrene, osteomyelitis, amputation if untreated.
GCC Context: SSc and connective tissue diseases are prevalent in the Gulf region. Air conditioning creates persistent cold environments — a major Raynaud's trigger. Higher diagnostic awareness is needed in GCC rheumatology services, particularly among South Asian expatriate populations.
| Drug | Monitoring Tests | Frequency | Stop/Action If |
|---|---|---|---|
| Methotrexate | FBC, LFTs, creatinine | Every 3 months (once stable) | WBC <3.5, ALT >3×ULN, creatinine rising |
| Hydroxychloroquine | Annual eye review (Humphrey VF, OCT) | Annually from year 5 (year 1 if risk factors) | Bull's eye maculopathy on OCT |
| Azathioprine | FBC, LFTs | Every 3 months (once stable) | WBC <3.5, MCV rising, LFTs >3×ULN |
| Cyclophosphamide | FBC, U&E, urine dipstick | Fortnightly during induction (±weekly if high-risk) | WBC <3.0, haematuria (persistent) |
| Mycophenolate | FBC, renal function | Every 3 months | WBC <3.0, rising creatinine |
| Rituximab | FBC, immunoglobulins, pre-dose B-cell count | Pre-infusion + 3-monthly | IgG <5g/L (infection risk), neutropenia |
| Anti-TNF | FBC, LFTs, TB surveillance | Every 3–6 months | Active infection, TB reactivation signs |
| Leflunomide | FBC, LFTs, BP | Monthly for 6 months, then 3-monthly | ALT >3×ULN, WBC falling, hypertension |
Immunosuppressed patients may not mount fever or leukocytosis. Any deterioration, new symptoms, or subtle change requires urgent infection screen.
Steroids: NEVER stop abruptly. During acute illness: double the steroid dose. Vomiting = go to ED for IV hydrocortisone.
MTX/Leflunomide: Hold during acute febrile illness or GI upset. Resume when recovered.
NO live vaccines on biologic DMARDs, cyclophosphamide, or high-dose steroids.
DHA / DOH / SCFHS / QCHP Exam Focus: Autoimmune disease questions frequently tested in GCC licensing exams. These boards favour pattern recognition, emergency priorities, and drug safety. Click each question to reveal the answer.
| Disease | Key Antibody | Most Serious Complication | First-line Treatment | Key Nursing Action |
|---|---|---|---|---|
| SLE | Anti-dsDNA, Anti-Smith | Lupus nephritis (class IV) | Hydroxychloroquine + steroids ± MMF | Urine dipstick, sun avoidance, ophthalmology |
| RA | RF, Anti-CCP (ACPA) | ILD, accelerated CVD, AAI subluxation | Methotrexate + folic acid | DAS28, joint protection education, TB screen pre-biologic |
| GCA | None specific (ESR ↑↑) | Irreversible visual loss | High-dose prednisolone IMMEDIATELY | Ophthalmology urgently — do NOT delay steroids |
| GPA/MPA | cANCA/PR3 (GPA), pANCA/MPO (MPA) | RPGN, pulmonary haemorrhage | Rituximab or cyclophosphamide + steroids | Daily urinalysis, haemoptysis monitoring, Mesna with CYC |
| SSc (diffuse) | Anti-Scl-70 (anti-topoisomerase I) | Scleroderma renal crisis, ILD | ACEi for renal crisis; MMF for ILD | BP monitoring, renal function, Raynaud's/digital ulcer care |
| SSc (limited) | Anti-centromere | PAH | Sildenafil/bosentan for PAH; nifedipine for Raynaud's | Annual echo, SpO₂ monitoring, GI positioning |