Systemic Lupus Erythematosus

History Taking

Chief Complaint

• Generalized weakness and fatigue

• Joint pain and stiffness

• Skin rash, particularly a butterfly-shaped rash over the cheeks

History of Presenting Complaint

• Duration and onset of symptoms

• Flares of symptoms with periods of improvement

• Photosensitivity (rashes worsen after sun exposure)

• Oral or nasal ulcers

• Unexplained fevers

• Hair loss

System Review

• Cardiovascular: chest pain, shortness of breath, palpitations (could indicate pericarditis, myocarditis, or lung involvement)

• Respiratory: pleuritic chest pain, dry cough

• Renal: frothy urine, swelling of legs (could indicate nephritis)

• Neurological: seizures, memory loss, headaches, mood changes, peripheral neuropathy

• Gastrointestinal: nausea, vomiting, loss of appetite, abdominal pain (possible liver or pancreas involvement)

• Hematological: easy bruising, bleeding, recurrent infections (could indicate cytopenias)

• History of conditions like rheumatoid arthritis, Sjogren's syndrome, scleroderma, vasculitis, or polymyositis

• Frequent episodes of tendonitis or carpal tunnel syndrome

• Dry eyes or dry mouth, which could suggest Sjogren's syndrome

• Skin changes such as tightening or color changes in fingers or toes in response to cold, suggesting scleroderma or Raynaud's phenomenon

• Muscle weakness and skin rashes, which could indicate polymyositis or dermatomyositis

Past Medical History

• History of diagnosed autoimmune diseases

• History of blood clots, strokes, or heart attacks at a young age

• Previous history of clots in the lungs, and clots in the legs - Antiphospholipid syndrome

• Recurrent miscarriages or other pregnancy complications

Drug History

• Use of medications that could induce lupus-like symptoms, such as hydralazine, procainamide, isoniazid, and some anticonvulsants

Family History

• Relatives with autoimmune diseases, particularly SLE

• Family history of heart disease or strokes at young age

Personal History

• Stress levels, as stress can trigger lupus flares

• Smoking and alcohol history, as these can influence disease progression and management

OBG History

• Number of pregnancies and outcomes

• History of pre-eclampsia, eclampsia, or other pregnancy-related hypertension

• History of premature birth or intrauterine growth restriction

Physical Examination

General Examination

• Fatigue or general malaise

• Fever

• Weight loss

• Pallor

• Jaundice

Examination of Hands and Arms

• Joint swelling and deformity, especially in the small joints of the hands

• Raynaud's phenomenon (color changes in the fingers with cold or stress)

• Gottron's papules (raised, red or violet scaly areas on knuckles) in case of overlap with dermatomyositis

• Vasculitis rash

Examination of Mouth

• Oral or nasopharyngeal ulcers

Examination of the Neck

• Enlarged, painless lymph nodes

Cardiac Examination

• Signs of pericarditis (pericardial rub, distant heart sounds)

• Signs of endocarditis, particularly Libman-Sacks endocarditis (heart murmurs, evidence of embolic events)

Respiratory Examination

• Signs of pleural effusion (decreased breath sounds, dullness to percussion)

• Signs of pleuritis (pleuritic rub)

• Signs of interstitial lung disease (crackles)

Abdominal Examination

• Hepatosplenomegaly (enlarged liver and spleen)

Neurological Examination

• Signs of cerebritis such as altered mental status, seizures, focal neurological deficits

• Signs of peripheral neuropathy

Additional Examination

• Dermatological: malar rash (butterfly-shaped rash across cheeks and nose), discoid rash (raised, red, scaly lesions), photosensitive rash, alopecia (hair loss)

• Ophthalmic: evidence of dry eyes (Sjögren's syndrome), retinal vasculitis

• Livido reticularis in antiphospholipid syndrome

Investigations

Laboratory Investigations

• Full Blood Count: to identify anemia, leukopenia, or thrombocytopenia common in SLE

• Urinalysis: to look for proteinuria or cellular casts indicative of nephritis

• Creatinine and urea: to assess renal function

• Liver function tests: to check for any liver involvement

• ESR and CRP: usually elevated in active disease

• Autoantibody profile: ANA, anti-dsDNA, anti-Sm, anti-RNP, anti-SSA/Ro, and anti-SSB/La for confirmation and classification

• Complement levels (C3, C4): often low in active disease

• Coagulation profile: to screen for lupus anticoagulant, anticardiolipin antibodies (to assess risk for antiphospholipid syndrome)

Imaging

• Chest X-ray: to identify pleural effusions or interstitial lung disease

• Echocardiogram: to evaluate for pericardial effusion or valvular disease (Libman-Sacks endocarditis)

• Renal ultrasound: to assess for kidney size and exclude obstructive uropathy

Invasive

• Renal biopsy: if there is evidence of nephritis; it helps in classification, guiding treatment, and determining prognosis

• Skin biopsy: if cutaneous lesions are present, for confirmation and to differentiate from other skin disorders

• Lumbar puncture: in cases of suspected neurological involvement (neuropsychiatric SLE)

Other Tests

• Pulmonary function tests: in case of respiratory symptoms, to assess for restrictive lung disease

• Electrocardiogram: to screen for conduction abnormalities or signs of pericarditis

• 24-hour urine protein: if proteinuria is present on urinalysis, to quantify and monitor

Management

General Management

• Education: regarding nature of disease, need for regular follow-up, importance of sun protection

• Lifestyle modification: including adequate rest, stress management, smoking cessation

• Avoidance of triggers: such as UV light exposure, certain medications

• Vaccination: pneumococcal, influenza, and other routine vaccines (avoid live vaccines during active disease or immunosuppressive treatment)

• Regular exercise: to maintain joint flexibility and muscle strength

Medical Management

• NSAIDs: for arthralgia, arthritis, and serositis

• Antimalarials (Hydroxychloroquine): to manage skin and joint symptoms, reduce disease flares

• Corticosteroids: for moderate to severe disease, major organ involvement

• Immunosuppressants (Methotrexate, Azathioprine, Mycophenolate mofetil): used in steroid-sparing strategy, major organ involvement (e.g., lupus nephritis), refractory disease

• Biologics (e.g., Belimumab): for patients with active, autoantibody-positive SLE despite standard treatment

• Anticoagulation: in patients with antiphospholipid syndrome

Surgical Management

• Joint replacement: for severe, debilitating arthropathy

• Dialysis or renal transplant: for end-stage renal disease secondary to lupus nephritis

Other Management

• Multidisciplinary team approach: involving rheumatologists, nephrologists, dermatologists, psychologists, physiotherapists, and occupational therapists

• Regular monitoring: of disease activity, organ function, and drug side effects

• Mental health support: as depression and anxiety are common in SLE patients

• Pregnancy planning: as SLE and its treatment can impact pregnancy and vice versa