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Systemic lupus erythematosus
This patient has SLE. What questions would you ask in history and please present your
Think of the clinical manifestations of lupus according to each organ system:
1) Constitutional symptoms. Ask about lethargy, fatigue, fever, weight loss, nausea and vomiting.
2) Musculoskeletal. Ask about arthralgia, myalgia. hip pain due to ischaemic necrosis of the head of
3) Skin. Ask about rash with photosensitivity, patchy alopecia, discoid skin lesion, sicca symptoms
4) CNS. Ask about seizures, psychotic episodes, headaches, peripheral sensory impairment, anxiety,
depression, delirium, dementia, eye irritation, visual disturbance.
5) Renal. Ask about Hematuria , Edema and renal failure.
6) Cardiorespiratory. Ask about dyspnea, pleuritic chest pain.
7) Hematological. Ask about recurrent thromboembolic disease (M I, Ischaemic stroke, P E and
Drug history: mnemonic: "PICk A MaP Home"
• Procainamide • Isoniazid • Chlorpromazine • Anti-convulsants: phenytoin. • Methyldopa • Penicillamine. • Hydralazine
Impact on patients life which includes reduced activity of daily living, inability to work.
Ask about treatment given and complications of treatment
Ask about problems during pregnancy and use of contraception
Ask about patient's understanding of the implications of this chronic and incurable disease and its
On examination, the patient had a cushingoid appearance. Her mental state was normal.
The hands showed signs of vasculitis, nail fold infarcts and gangrene.
there is evidence of Raynaud's phenomenon and arthropathy as evidenced by swelling of the P I P
The forearm shows livedo reticularis and purpura.
There is proximal myopathy of the upper limbs.
On the head, there is alopecia and lupus hair.
The eyes show keratoconjunctivitis sicca and conjunctival pallor.
Fundoscopy shows cytoid lesions seen as hard exudates.
There are no cervical or axillary lymphadenopathy.
On cardiorespiratory exam, there is no pericardial rub. Heart sounds are dual and no murmurs were
There are bilateral inspiratory crackles on inspiration.
Abdomen examination reveals hepatosplenomegaly.
The patient has proximal weakness in the legs and walks with an ataxic gait.
There is evidence of peripheral neuropathy which is mainly sensory.
How would you manage this patient's SLE?
Along with the clinical symptoms and signs presented, I would also require several investigations to
help me confirm the diagnosis of SLE in this patient.
I would like to request for a full blood count to look for anemia, lymphopenia and
Also a hemolytic screen and iron studies if anemia is present.
Then I would like to see the patient's autoimmune serology results especially the antinuclear
antibody , anti - double stranded DNA antibody and anti smith antibody.
With the history of recurrent thromboembolism and fetal loss, I would like to look for the presence
of anti phospholipid syndrome by checking for presence of anti cardiolipin antibody and lupus
I would also check the Complement levels such as C3, C4 and total hemolytic component, the CH50
which may be low due to complement consumption.
An electrolyte profile and renal function indices to look for evidence of renal failure.
Urinalysis to look for proteinuria and hematuria followed by a urine microscopy to look for red cell
casts. If proteinuria is present, I would like to quantify it with a 24 hour urine collection.
If renal failure is present, I would perform a renal biopsy. The abnormalities that I would expect to
see would either be a mesangial, focal, diffuse or membranous proliferation.
A chest x ray to look for pleural effusion and evidence of fibrosis along with a high resolution CT
chest to further characterize the pulmonary parenchyma.
And finally an echocardiogram to look for pericardial effusion and valvular vegetations.
Once the diagnosis of SLE is established, my treatment goals are:
In managing this patient's SLE, I would involve a multidisciplinary team involving the physician,
physiotherapist, occupational therapist, psychologist and social worker
Arthralgia, myalgia and fever respond to rest and N SAIDs.
Patient should avoid exposure to sunlight and they should wear sunscreen to avoid photosensitivity
Arthritis and skin rash can be managed with hydroxychloroquine 400miligrams per day for 3 months,
then a maintenance dose of 200miligrams per day. However, I should remind the patient that they
will require annual retinal and visual field examination to avoid retinal toxicity from this drug.
Raynaud's phenomenon can be managed with calcium channel antagonist.
Acute exacerbations should be managed with high dose intravenous methylprednisone given at
Steroid may be used for central nervous system involvement, pericarditis, myocarditis, pleurisy,
severe hemolytic anemia and thrombocytopenia.
I would start with high dose therapy then gradually taper down the dose as improvement occurs.
For the recurrent thromboembolic disease, I would commence the patient on lifelong warfarin with
In terms of managing this patient's renal failure, mesangial proliferation carries the best prognosis
where as diffuse proliferation carries the worst prognosis and therefore aggressive treatment with
high dose pulse steroids plus cyclophosphamide should be commenced.
Other options would include the addition of mycophenolate, azathioprine, cyclosporine and
Renal transplant is not contraindicated, but there is a higher than average risk of graft failure in
To protect against osteoporosis, I would commence the patient on calcium and vitamin D
If the patient is to be on long term steroids, I would then consider adding on calcitonin and
Azathioprine could be considered as a steroid sparing agent.
The overall prognosis of SLE is generally good with 75% 10 year survival rate.
How do you differentiate between lupus and rheumatoid arthritis?
The joints in lupus are asymmetrically involved compared to symmetrical joint involvement in
Stiffness is all day in lupus compared to morning stiffness in rheumatoid
There is no bony erosion in patients with lupus compared to the erosions seen over the periarticular
surfaces of the bone over the wrist, MCP and PIP joints seen in rheumatoid.
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