Name : Siti Huzainah Binti Malim

RN : 28991

Age : 36 years old

Gender : Female

Race : Melanau

Occupation : Housewife

Date of admission : 10/12/2015

Chief complaint

Patient complained of generalized lethargy and urinary retention for 2 days.

History of Presenting Illness

This is patient's first hospitalization and was brought by her husband. Patient was initially well
until she developed facial rash associated with itch for the past two months. The rash worsens on
exposure to sunlight but was not associated with pain. Prior to this, she had photosensitivity
whenever exposed to sunlight but it would resolve spontaneously. She also claimed to have
bilateral lower leg swelling for the past one week. She also had painful oral ulcers for four days
prior to admission. The ulcers were over the hard palate and lips. Then, she developed sudden
onset of generalized lethargy and urinary retention associated with increased urinary frequency
for two days. she described only very minimal amount of urine was passed. However, she
denied pain on urination, oliguria, hematuria and urinary incontinence. She also had loss of
appetite for two days but denies loss of weight. She admitted started developing thinning of the
hair and hair loss for the past 2 weeks. She claimed to have dryness over the lips but no dryness
over the eyes.

She also denied having fever, discolouration of digits whenever exposed to cold, joint pain or
any bleeding tendencies (gum bleeding, menorrhagia, melena).

Systemic Review

 General: No fever, + loss of appetite, no loss of weight

 Cardiovascular system: No chest pain, no palpitation, no orthopnea, no PND, no syncope
  Respiratory system: No dyspnoea, no cough, no hemoptysis
 Gastrointestinal system: No nausea, no vomiting, no abdominal pain, no change in bowel
movement, no melena or hematochezia
 Neurovascular: No headache, no dizziness, no loss of conscious, no blurring of vision
 Genitourinary system: + Urinary retention, + increased frequency, no oliguria, no
hematuria, no urinary continence
 Musculoskeletal: No joint pain, no muscle pain

Past Medical History

Patient had pregnancy induced hypertension in both pregnancies but resolved after delivering.
She denies having hypertension, diabetes mellitus or ischaemic heart disease.

Past Surgical History

There was no relevant past surgical and trauma history.

Family History

Her parents and siblings are currently healthy and well. There is no family history of systemic
lupus erythematus, ischaemic heart disease, hypertension and diabetes mellitus.

Drug History

Patient had consumed traditional herbs during pregnancy but denied any usage of other
medications.

Social History

Patient is a housewife, married with 2 children and currently living with her husband and
children. She used to work in a chemical factory for 6 years but had to stop to take care of her
children. She denied any history of smoking, consuming alcohol or taking illicit drugs.

Physical Examination
General Examination

Patient was alert and sitting comfortably on bed with IV branula attached at the left wrist. She
was not in respiratory distress. There was malar rash with sparing of nasolabial fold. There was
no signs of yellowish discolouration of the sclera or pallor. Oral hygiene was poor with ulcers
over the palate but there was no central cyanosis. There was no signs of clubbing, nicotin
staining, peripheral cyanosis, splinter haemorrhage, Raynaud phenomenon, vasculitis, rhematoid
nodules, leuconychia or koilonychia. JVP was not elevated and she had no enlarged lymph nodes
over the clavicle, neck and axillary region. However, there was bilateral pedal edema extending
up to the knees.

Cardiovascular Examination

Pulse rate was 88 beats/min, with regular rhythm, strong volume and no radial-radial and radial-
femoral delay. Blood pressure was 156/106mmHg with a temperature of 36.4˚C. Upon
inspection of the chest, there was no scars, no deformities or abnormal pulsation was seen. Apex
beat was felt at the left 5th intercostal space at the mid-clavicular line. There was no parasternal
heaves or thrills. On ausculation, 1st and 2nd heart sounds were heard with no additional heart
sounds or murmur.

Respiratory examination

There was no tracheal shift and no reduced chest movement. Respiratory rate was 16
breathes/min. Chest expansion was equal and good on both sides. Vocal fremitus was equal on
both side. Upon precussion of the chest, resonant note was heard over the lungs. Vesicular breath
sound was heard over the lung field with no additional breath sounds. Vocal resonance was equal
on both sides.

Abdominal examination

On inspection, there was no scar, no dilated veins or skin discolouration. However, the abdomen
was distended but abdomen was soft, non-tender and no organomegaly and no mass was felt.
Shifting dullness was positive but fluid thrill was negative. On auscultation, normal bowel sound
with no bruit was heard.

Neurovascular examination

Normal muscle tone, power and reflex were elicited over the upper and lower limbs.

Provisional Diagnosis

Acute flare of systemic lupus erythematus with lupus nephritis

Differential Diagnosis

Mixed connective tissue disease - if

Antiphospholipid syndrome - characterized by recurrent vascular thrombosis and fetal loss

Sjogren syndrome - no dry eyes or sore eyes.

Lab investigations

1. Full Blood Count

White blood cell 9.7 x103/μL High 4.0-11.0

Red blood cells 2.3 x106/μL Low 4.5-6.5
Platelet count 51 x103/μL Low 150-400
Haemoglobin 9.4 g/dL Low 11.5-16.0
Hematocrit 50.4 % High 35-47

2. ESR : 36 mm/hr High (0-40)

3. CRP : 4.8 mg/dl High (0-0.74)

4. BUSE
 Sodium 133 mmol/L (137-149)
 Potassium 4.6 mmol/L (3.8-5.2)
 Chloride 107 mmol/L (97-107)
 Urea 11.7 mmol/L High (1.7-8.3)
 Creatinine 169 μmol/L (40-177)

5. Albumin 15 g/L Low (55-80)

6. Hemoglucose test 6.8 mmol/L

7. Urine dipstick
 Blood 3+
 Protein 1+

8. Fasting Lipid Profile

 Total cholesterol 7.3 mmol/L High (< 5.2)
  Triglyceride 4.31 mmol/L High (< 2.3)
 HDL 0.78 mmol/L Low (> 0.9)
 LDL 4.6 mmol/L High (< 3.9)

9. Cardiac Enzymes
 AST 32 U/L (< 37)
 Creatinine Kinase 106 U/L (24-195)
 LDH 356 U/L High (< 248)

10. Rheumatoid factor 19 IU/ml High

11. C3 0.20 g/L High (0.7-1.7)

C4 0.05 g/L High (0.2-0.5)
12. ANA 1:2560 High (< 80)

13. DsDNA 597.4 High (< 2)

14. Coombs test

 Direct : Negative
 Indirect : Negative

15. CXR : Cardiomegaly. No pleural effusion.

16. ECG : Sinus rhythm. Heart rate : 84 beats/min

17. Echocardiogram : LVEF > 55% ; no RWMA ; + pericardial effusion

Management

Investigation
1. FBC - screen for anemia, leukopenia and thrombocytopenia to aid in diagnosis
2. ESR - raised during acute flare of SLE
3. Blood culture and sensitivity - To rule out any infection
4. PT/PTT/INR - May be increase in anti-phospholipid syndrome
5. Renal function test - to check renal function before starting therapy
6. UFEME and urine culture and sensitvity - check for signs of nephritis or nephrotic
7. Liver function test - to check liver function before starting hydrochloroquine therapy
8. Immunological profile (ANA, anti-DsDNA, anti-Ro, anti-La, antiphospholipid and
anticardiolipin antibodies) - to aid in diagnosis
9. Serum complement level - low in active SLE
10. Coombs test - positive in patient with autoimmune hemolytic anemia
11. Skin biopsy - lupus band test
12. Ultrasound of kidney and urinary bladder - for evidence of renal involvement
13. Renal biopsy - To aid diagnosis or establish prognosis of SLE
14. ECG - To check for signs of MI and pericardial effusion (low voltage, tachycardia and
electrical alternans)
15. Echocardiogram - To check for pericardial effusion and valve abnormalities secondary to
connective tissue disease
16. CXR - To rule out pleural effusion
17. Thyroid function test - to screen for autoimmune thyroid disease
18. VDRL - A false-positive syphilis test can reveal presence of antiphospholipid antibody

Treatment
1. Education
2. Physiotherapy and exercise
3. Closed bladder drainage (CBD) for urinary retention
4. High protein diet and encourage to increase oral intake as she has low albumin
5. NSAID - Ibuprofen
6. Corticosteroid - IV methylprednisolone
7. Immunosuppressant - cyclophosphamide, methotrexate, azathioprine
8. Anti-malarial - hydroxychloroquine
9. Biological therapy - belimumab, rituximab
10. Antibiotics - IV cefuroxime to cover for urinary tract infection
11. Hypertensive - nifedipine
12. IV albumin - To reduce oedema
13. ACE inhibitors - captopril to reduce proteinuria and generalized oedema
14. Bonjela gel for oral ulcer
15. Regular follow up
16. Be aware of Raynaud's phenomenon and signs of DVT

Discussion

Systemic lupus erythematus is a multisystem, autoimmune connective tissur disorder. The exact
cause of SLE is unknown, although genetic, hormonal and environmental factors (infections,
drugs and exposure to UV light) play a role. Women are affected ten times more often than men
and it is most common in people aged between 15-40 years old.

Common early features include fever, arthralgia, malaise, tiredness and weight loss. There is a
huge spectrum of disease and any of the systems may be involved. Musculoskeletal system is
involved in 90% of cases with patient presenting with symmetrical and migratory arthralgia.
Aseptic necrosis affecting the hip may rarely occur. Skin is involved in 80% of cases. Patient
may have a butterfly rash over the bridge of the nose and spreading over both cheeks. Other
features include photosensitivity, alopecia, Raynaud's phenomenon and oral ulceration. Discoid
lesions may develop in SLE. Renal system is also commonly involved and patient may present
with nephrotic or nephritic syndrome. CNS involvement include psychiatric disturbances,
seizures, stroke, headache and peripheral neuropathies. CVS involvement may present with
pericarditis with pericardial effusion, myocarditis, aortic or mitral valve lesion. In blood and
lymphatic system, patient may present with normochromic normocytic anemia, leucopenia,
thrombocytopenia, hemolytic anemia, generalized lymphadenopathy or arterial and venous
thrombosis.

This patient meets the 8 of the 11 SLICC criteria for diagnosis, including cutaneous lupus, oral
ulcers, leukopenia, thrombocytopenia, anemia, renal involvement, positive ANA titre, high anti-
DNA, low C3 and C4. She also has raised ESR, high anti-DsDNA titres and low C3 and C4
complement level which all indicates high disease activity in SLE.

Siti Huzainah has signs of kidney involvement as she has abdominal distension, bilateral lower
limb swelling and high blood pressure with abnormal urea, proteinuria and blood in urine. She
also has impending pericardial tamponade as echocardiogram revealed the presence of
pericardial fluid.

When treating SLE, patient education is the key. Patient should be educated that this disease is
incurable and it is presented with flares and remissions. Patient should decrease exposure to UV
rays and apply sunscreen or wear clothes that cover the extremities. Patient should also protect
their hands and feet from cold. Patient also can join support groups to share their stress and ways
to cope SLE. Regular exercise helps prevent muscle fatigue and weakness. Patient on
corticosteroid or immunosuppressants should be monitor for signs of infection and avascular
necrosis. Any infection should be promptly treated. Regular follow up should be done to monitor
the progression of the disease.

Early diagnosis and appropriate treatment of SLE can have a positive outcome. Patients have a
higher risk of vascular morbidity and mortality, especially if poorly controlled disease or higher
doses of steroids needed.

References

1. Dr Raj Carmona, R.C. 1. 2012 SLICC SLE Criteria. [Online]. Available from:
respiratorytherapycave.blogspot.my [Accessed 3 January 2016].

2. Sara Gress, Kelly Mudlo, Micheal Rangel, and Shelby Wagoner, S.G.,. .K.M.,. .M.R.,.
.S.W. 1. Systemic Lupus Erythematosus Case Study. [Online]. Available from:
http://www.physio-pedia.com/Systemic_Lupus_Erythematosus_Case_Study [Accessed 3
January 2016].

3. Dan Horton-Szar, D.H.S. 1. Crash Course: Rheumatology and

Orthopaedics. [Online]. Available from: [Accessed 3 January 2016].