A 32-years-old Male with Bluish
Coloration of Fingers and Toes
I
Particulars of the Patient
• Md. Abdul Malek
• 32 years
• Farmer
• Monohardi, Narsingdi
• 25/09/2018
Background history
• SLE (2012)
• Bronchial asthma (2014)
Chief Complaints
1. Pain and bluish coloration of all fingers and toes
for 7 days
2. Bilateral leg swelling for 15 days.
History of Present Illness
• SLE (2012)
- Mucocutaneous
- Musculosketetal
- Systemic features
- Hb- 6.4, ESR-170, WBC-10900, Plt-291000
- ANA, Anti-ds DNA (+)
- Coombs test (+)
- Urine: Normal
History of Present Illness
• SLE ( May, 2012)
- HCQ 300 mg
- Paracetamol 1500 mg daily
- Prednisolone 50 mg daily
- Significant improvement
History of Present Illness
• SLE (2013-2014)
- Lost to follow up
- Mild intermittent exacerbation
- HCQ 300mg , prednisolone 5 mg daily
irregularly
History of Present Illness
• SLE (2014)
- Flare of symptoms
- Cough, SOB, wheeze
- HB-8.5, ESR 98, MCV 73, MCH 23, RDW-CV 17.2
- Lupus flare with bronchial asthma
- HCQ 300 mg daily
- Prednisolone 25 mg daily
- Salbutamol, salmetorol/ fluticasone inhaler
- Montelukast 10mg daily
History of Present Illness
• SLE(2016-2018)
- Irregular follow up and treatment
- Intermittent exacerbation
- No major organ involvement
- HCQ 300mg , prednisolone 5 mg daily
irregularly
History of Present Illness
• Bluish coloration of fingers and toes for 7 days
– Acute, excruciating pain
– Occurs simultaneously
-- Progressive , become blackish over right
great
toe and left 2nd
toe
– No discharge, ulceration
– No H/O trauma, Raynaud's phenomenon
Cont..
• Bilateral leg swelling for 15 days
- Gradual onset
- Distal part of legs and feet
- Has no shortness of breath, orthopnea,
paroxysmal nocturnal dyspnea, puffy face,
decreased urine output, no history of jaundice
cold intolerance
Past History
Nothing contributory
Personal History
• Non smoker, non alcoholic
• Has no history of substance abuse and intravenous
drug abuse
Family History
There is no such type of illness in his family and
no history of premature death.
Immunization history
• 2016
- Pneumococcal (polysaccharide)
- Influenza
-Hepatitis B
Socio-economic History
• Monthly family income- 25,000 taka
General Examination
• Alopecia
• Anemia- (+)
• Pulse-80b/min, peripheral pulses normal
• BP-110/80 mm(Hg)
• Resp. rate-14b/min
• Temp-Normal
• Cyanosis- fingers and toes
• Edema present
• BMI -20kg/m2
• JVP-normal
MSK system
• Gait- Antalgic
• Arms-
-Pale with distal cyanosis,
-Reduced temperature
-MCP squeeze test (+)
• Legs-
- Fingers are pale with distal cyanosis
- Blackish discoloration with line of demarcation- right
great toe and left 2nd
toe
- Reduced temperature
Spine –normal
Other System
Cardiovascular ,Respiratory, Abdominal and
Neurological--Normal
• INVESTIGATIONS
Investigations 18/09/18
CBC
Hb
WBC
N
L
E
Platelets
MCV
MCH
MCHC
RDW-CV
9.8gm/dl
10,700/cumm
67%
25%
6%
470000/cumm
73.5 fl
23.8 pg
32.5gm/dl
16%
ESR 101 mm in 1st
hr
Urine R/M/E Normal
Investigation 19/09/18
S. creatinine 0.96mg/dl
ALT 19U/L
23/09/18
Anti cardiolipin Ig G Negative
Anti cardiolipin Ig M Negative
Investigation 24/09/18
Duplex study of both lower limbs Normal blood flow was observed in all arteries of left/
right lower limb.
Varicose veins of left /right lower limb
Chronic venous insufficiency
APTT 30 sec
Investigations 27/09/18
CBC
Hb.
WBC
N
L
E
Platelets
MCV
MCHC
MCH
RDW-CV
9.3gm/dl
12500/cumm
66%
18%
12%
380000/cumm
72.1 fl
33.3g/dl
24 pg
16.4 %
ESR 100 mm in 1st
hr
PBF Microcytic hypochromic anemia,
Neutrophilic leukocytosis
Investigation 01/10/18
Bilirubin
Total 0.4 mg/dl
Direct 0.3 mg/dl
Indirect 0.1 mg/dl
Reticulocyte 1.23% (˂2.5%)
Iron 17 µg/dl (31-144 µg/dl )
Ferritin 544.86 ng/dl (12-300 ng/dl)
TIBC 196 µg/dl (110-370 µg/dl )
TSAT 8.67
C3 o.191 g/l (0.9- 1.8 g/dl)
C4 <0.0655 g/l (0.1-0.4 g/dl)
27/09/18
FBS 3.9 mmol/l
2hrs after 75gm glucose 6.4 mmol/l
Lipid profile
TC
HDL
LDL
TG
100 mg/dl
21mg/dl
61 mg/dl
90 mg/dl
HBsAg Negative
Anti HBc Negative
Investigation 27/09/18
ECG Normal
Echocardiography Good LV systolic function
Investigation 29/09/18
Anti-ds-DNA
CRP
110.2U/ml ( < 30U/ml= negative)
152.0 mg/L ( < 6mg/L)
30/09/18
Chest X- ray P/A view Cardiomegaly (mild)
Nail-fold capillaroscopy
Nail-fold capillaroscopy
Nail-fold capillaroscopy
• Capillary dropout (4-5/mm)
• Haemorrhage (multiple)
• Neovascularization
• Elongated capillaries
SELENA-SELEDAI = 16
Hospital course
• LMW heparin with Warfarin 5mg
• Prednisolone 1 mg /kg
• Indomethacin 150 mg /day
• Calcium, vit- D
• Vaccination
-Pneumococcal conjugate
- Influenza
Ferrous sulphate, Ascorbic acid
PROVISIONAL DIAGNOSIS
SLE with vasculitis
DIFFERENTIAL DIAGNOSIS
SLE WITH APS
Problem Lists
Diagnostic dilemma
Management
• Report pending
Lupus anticoagulant
D-dimer
SLE with vasculitis
Points in favor Points against
Simultaneous involvement of all four
limbs
Presence of cyanosis and gangrene
High disease activity of SLE
Normal peripheral pulses
High CRP
SLE with APS
Points in favour Points against
Simultaneous involvement of all four
limbs
Presence of cyanosis and gangrene High CRP
Thank you

Systemic lupus erythematosus with Vasculitis.pptx