In station 5 you may be asked to assess a patient with joint pains, rash, hair loss etc.
History (3 minutes):
- Timing: Since when, sudden/gradual (over days/weeks/months), all the time or coming and going? getting worse?, ever had this problem before?
- Joint pain/swelling/stiffness/redness/function (which joints? Both sides? What order did they become affected? Large/small joints/both? Morning stiffness for how long?
- Extraarticular features screen:
- Systemic: fever, night sweats, weight loss, fatigue, lymph nodes, sleep disturbance, appetite, malaise
- Raynauds: do your fingers go cold and change colour in cold weather? (white-blue-red)
- Any rashes? Sensitivity to the sun? Hair loss/thinning? Scarring hair loss? Mouth or nose ulcers? Genital ulcers? Swollen/tight skin of hands/arms?
- Any dry eye/mouth? Sore/red eye? Any visual disturbance?
- CVS/Resp: Chest pain, SOB (and on exertion), Orthopnoea, PND, Palps, Ankle swelling, Syncope, presyncope, Wheeze, Cough (and sputum colour, amount, consistency, blood, smell), Haemoptysis, Exercise tolerance. CVS risk factors.
- GI/GU: Appetite/weight change, Dysphagia, Nausea, vomiting, haematemesis, Indigestion/heartburn, Jaundice, Abdominal pain, Bowels: change/constipation/diarrhoea/stool/blood/mucus/flatus/incontinence, Bloating, tenesmus, urgency, dysuria, urethral/vaginal discharge
- Renal: high BP, blood/protein in urine
- Neuro: Headaches, Vision- blurring/double/bits missing, Fits/faints/LOC, Dizziness, Hearing, Weakness, Numbness/tingling, Loss of memory/personality change/confusion/hallucinations, Anxiety/depression. PMH TIA/stroke.
- Muscle: pain/weakness/stiffness, which muscles? (proximal/distal), swallow/voice/breathing
- Pregnancy: miscarriages, baby have heart block/rash, any history of DVT/PE/MI, any premature births.
- PMH: Joint disease eg. Gout/RA/OA/SLE, Inflammatory bowel disease, Psoriasis, Autoimmune conditions eg. Thyroid, coeliac, diabetes. Other eg. Heart, lung, kidney problems, cancer, recent infections
- FH: as per above
- DH: including any newly started medications such as hydralazine, procainamide, isoniazid, phenytoin, interferon (drug-induced lupus)
- SH: including whether pregnant/ breastfeeding/pregnancy plans
Examine (3 minutes):
- Head including scalp: malar rash, alopecia, discoid lupus, mouth ulcers, conjunctival pallor, lymph nodes
- Hands and arms: nailfold vasculitis, synovitis, raynauds, fistula
- Abdo (organomegaly, renal transplant)
- Legs- oedema, DVT
- Skin- discoid lupus, vasculitic rash, photosensitivity rash, livedo reticularis
- May appear Cushingoid from steroid use
ICE+Explanation (2 minutes)
A suggested explanation is as follows:
“It seems that you are having symptoms and signs of inflammation in various parts of the body. A condition called lupus affects the skin, joints and other organs. I’d like to test you for this with blood tests. I also want to test your urine and check your blood pressure, get a CXR and tracing of your heart. I’ll refer you to a rheumatologist who specialises in this condition. In the mean time we can try a NSAID to help your joint symptoms and try to avoid the sun/use sunscreen. If it is lupus then treatments can be given to control your symptoms and reduce the inflammation”
FBC, U+E, LFT, clotting
ANA, RF, anti-CCP, ANCA (+PR3/MPO), Immunoglobulins, complement (+/- cryoglobulins)
Antiphospholipid Abs (anticardiolipin, antiB2GPI), lupus anticoagulant screen
Anti-dsDNA, antiscl70, anticentromere Ab, AntiRo, AntiRNP, antism, Antijo1, AntiLa, Antism/RNP, antichromatin, antihistone
Serum electrophoresis+ urine bence jones proteins, LDH, blood film
HIV, Hep B+C screen, CMV, EBV
Muscle symptoms/signs: CK, TFT, Vitamin D, Ca, P, PTH
Miscellaneous: ACE (sarcoid), ferritin (stills),anti-GBM (goodpastures)
Renal: BP, urine dipstick for blood and protein, urine PCR, renal USS, renal biopsy
Joints/Spine: Xrays/USS, aspiration, MRI
CVS: ECG, troponin, ECHO, BNP, cMRI
Resp: Sats, ABG, CXR, pulmonary function tests, HRCT, Right heart catheterisation, BAL, broncoscopy
Muscle/Neuro: EMG/NCS, CT/MRI head
GI: abdo USS,OGD, barium swallow, flexible sigmoidoscopy/colonoscopy, CT
Biopsy- skin, renal
A multisystem inflammatory autoimmune disorder
Broad spectrum of clinical presentations encompassing all organs and tissues
F>M, 9:1, 2-8/100000/year, African American and Latin Americans, Afrocaribbean, South Asian, Hispanic, 16-55 years old
Chronic waxing and waning course
SLICC 2012 Classification Criteria
- 4/17 including 1 clinical and 1 immunological
- Biopsy proven lupus nephritis and ANA/AntidsDNA
- Acute cutaneous lupus (malar rash, bullous lupus, toxic epidermal necrosis variant of SLE, maculopapular lupus rash, photosensitive lupus rash) OR Subacute cutaneous lupus (nonindurated psoriaform and or annular polycyclic lesions that resolve without scarring)
- Chronic cutaneous lupus (classic discoid rash, hypertrophic lupus, lupus panniculitis, mucosal lupus, chilblains lupus OR discoid lupus/lichen planus overlap
- Oral or nasal ulcers (palate, buccal, tongue or nasal ulcers in the absence of other causes) NB: usually painless
- Nonscarring alopecia (diffuse thinning or hair fragility with visible broken hairs in the absence of other causes) NB: discoid lupus can cause scarring alopecia
- Synovitis (2 or more swollen joints OR 2 or more tender joints and early morning stiffness >3o mins) NB: jaccoud’s arthropathy: nonerosive, reducible joint deformities
- Serositis (pleurisy/effusions/rub OR typical pericardial pain/effusion/rub/pericarditis in the absence of other causes)
- Renal (urine PCR 500mg protein/ 24 hours OR red blood cell casts)
- Neuro (seizures, psychosis, mononeuritis multiplex, myelitis, neuropathy, confusion)
- Haemolytic anaemia
- WCC<4 or lymphocytes <1 at least once
- Platelets <100 at least once
- ANA (raised)
- AntidsDNA (raised)
- Antismith (presence of this antibody)
- Antiphospholipid antibodies (any of the following positive: lupus anticoagulant, anticardiolipin, anti-beta2glycoprotein)
- Low complement (C3 or C4 or CH50)
- Positive direct coombs test in the absence of haemolytic anaemia
ANA (95% sensitive, low specificity). Positive in 98% of SLE patients.
Antiribosomal P (specific, can be positive in ANA negative patients).
AntidsDNA (70% of SLE, 95% specific)
Antism (pathognomic for SLE) Positive in 10-30%
Antiphospholipid Abs positive in 30-50%
Anti Ro and La (neonatal lupus, heart block)
Anti-RNP (mixed connective tissue disease)
RF (positive in 40%)
Antihistone antibodies for drug induced lupus
- Drug-induced lupus
- Stills disease
- Undifferentiated or mixed connective tissue disease
- Primary sjogrens
- Antiphospholipid syndrome
- Pulmonary-renal syndrome (goodpastures, ANCA-associated vasculitis)
- Systemic sclerosis
Spares nasolabial folds. Red papular butterfly rash on face, photosensitive.
Differential Diagnosis of malar rash: seborrhoeic dermatitis, rosacea, atopic dermatitis, contact dermatitis
Plaques and scaly erythema and atrophic centre. Scarring. Affects scalp, neck, face, back, arms. Follicular plugging. Associated scarring alopecia and hypopigmentation and painful joints. Autoimmune process. Rarely progresses to SLE (5%). ANA negative usually though can be positive. Management: avoid sun, use sunscreen, topical potent steroid, hydroxychloroquine.
Differential diagnosis of discoid lupus: eczema, psoriasis, tinea corporis
Lupus and the lungs:
Pleurisy, pleural effusion
Infection (due to immunosuppression)
Acute lupus pneumonitis
Pulmonary haemorrhage (rare)
Shrinking lung (rare)
ARDS (reaction to biologic therapy)
Lupus and the heart:
Pericarditis and pericardial effusion
Increased risk coronary heart disease
Can be primary or secondary
- Arterial thrombosis <50 years old
- Unprovoked venous thrombosis < 50 years old
- Recurrent thrombosis
- Both arterial and venous events
- Unusual sites eg. renal, liver, cerebral sinuses, mesenteric, vena cava, retinal
- Obstetrical: fetal loss (miscarriage after 10 weeks/3 unexplained miscarriages <10 weeks), recurrent miscarriages, early/severe preeclampsia, unexplained intrauterine growth restriction
Lab criteria on 2 occasions 12 weeks apart: anticardiolipin Abs, Anti-B2GPI, lupus anticoagulant, raised APTT.
- Antiphosholipid Abs and thrombosis such as DVTs/stroke
- Low platelets
- Recurrent miscarriages
- Livedo reticularis
10% go on to develop lupus within 10 years.
Secondary: 50% with SLE.
Management: avoid oestrogen contraceptives, prophylactic aspirin, heparin for thrombosis then warfarin
Monitoring in lupus:
- Disease Activity – SLEDAI (SLE disease activity index), BILAG (British IslesLupus Assessment Group Index), ECLAM (European Consensus Lupus Activity Measurement). Helps make treatment decisions.
- Damage in SLE due to disease/treatment- SLICC (SystemicLupus Collaborating Clinics), ACR (American College of Rheumatology) damage index
- Clinical features
- Laboratory: FBC, complement, antidsDNA, ESR, U+E, urine dip
Lupus flare suggested by low lymphocytes, low complement, normal/low CRP, raised ESR, anaemia, thrombocytopenia, raised antidsDNA
NB: infection suggested by normal/raised WCC if usually low, raised CRP
Morbidity and Mortality in Lupus from infections, atherosclerosis, osteoporosis, malignancy especially lymphoma, lung cancer, cervical.
Management of lupus:
Conservative: Sunscreen, pregnancy advice
Medical: Manage CVS risk factors. ACEI for proteinuria.
Mild-moderate lupus (MSK and mucocutaneous features): low dose oral prednisolone and bone and GI protection, hydroxychloroquine, azathioprine, methotrexate. NSAIDs for joint pain (watch U+E)
Severe (major organ involvement): iv methylprednisolone, cyclophosphamide, mycophenolate mofetil, rituximab/belimumab
Surgical: renal transplant
Written by Dr Sarah Kennedy
Resources used to write this document include those listed in the references section of this webpage and also:
Rheumatology Secrets by Sterling West