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A-20-year-old female
presenting with fever,
respiratory distress & skin
rash
Dr.Md Shadman Shakib
Intern Doctor
Dhaka Medical College Hospital.
Salient Features
Mrs.Shanta, 20-year-old-woman, hailing from Jurain, Dhaka was seen
by the medicine admission unit on 9th June,2019 at this hospital for
respiratory distress , fever & rash involving both lower limbs, trunk &
upper limbs.
On the day of admission, she presented with respiratory distress (MRC
grade 3) for last 5 days associated with occasional dry cough but not
associated with orthopnea or PND or any history of SOB since childhood.
She also complains high grade intermittent fever, the highest recorded
temperature was 103 degree F that was not associated with any chills &
rigors, but relieved with paracetamol.She also noticed pinkish to violet
coloured skin rash that is occasionally itchy, painless & gradually both
lower limbs,trunk & upper limbs for last 4 days
On query, she gives a history of non specific joint pain involving small
joints of hands & also knee joint 3 days back that was associated with
swelling of the entire hand, spontaneously resolved on the day before
admission.She also gave a history of 1 bag whole blood transfusion at a
local clinic on that
day.No reaction occured.She denied any history of abdominal
pain,jaundice,cough with productive sputum,previous history of low
grade fever associated with night sweat & evening rise of temperature
,chest pain, hemoptysis , history of leg swelling or swelling of
abdomen,bloody urine or stool, nasal crusting, epistaxis, weight gain,
cold intolerance, constipation, hisory of pregnancy or spontaneous
abortion/loss of pregnancy, convulsion, gum bleeding, oral ulcer, alopecia,
photosensitivity, menorrhagia or passage of any blood clots during
menstruation, per vaginal discharge or burning micturition or lower
abdominal pain, significant weight loss, bone painExtramarital affair, h/o
travelling to Malaria or Kala Azar endemic zone, previous history of
asthma or similar type of illness in childhood, h/o vaccination with
tetanus toxoid.Drug history was negative for taking any anti-
arrhythmic,anti-hypertensive, anticonvulsant or biologic drugs or
consumption of INH, minocycline & pyrazinamide .
1 weeks before this evaluation, the patient was seen in the OPD of a
private hospital.She was diagnosed with left sided pleural effusion & was
prescribed Cefuroxime-Clauvulonic acid & paracetamol along with advise
for hospitalisation, however she denied to be admitted there.
Menstrual history
Period 5-7 d, Cycle 21-28 Flow -average
Contraceptive history
Did not adopt any contraceptive method so far.
Patients family history was not noteworthy of asthma/ TB or any
rheumatological condition or any history of pregnancy loss in mother or
siblings.She is married for 6 months & her electrician husband works in
Oman.She lives in her in laws house situated in old town of Dhaka.
Past history
10 weeks before this presentation, when she noticed fever , respiratory
distress associated with dry cough & sore throat for 3 days, patient was
evaluated in the OPD of another tertiary care hospital.The patient was
empirically treated as a case of Para-pneumonic effusion.Initially she was
prescribed azithromycin, pantoprazole, paracetamol for 7 days, later
Azithromycin was substituted with combination of co amoxiclav &
clarithromycin & patient was Improved.
8 weeks before this evaluation,during follow-up at that that hospital, her
condition was slowly improving, however, she was again prescribed co
amoxiclav & clarithromycin for 14 days in addition to salbutamol &
fluticasone inhaler, montelukast & rabeprazole.She took them accordingly
& she describes her condition was stable & she was symptom free after
taking them.
Physical Examination
General Examination
Ill looking, anxious
Body Built- Average
Co-operative, Decubitus on choice
Pulse -90/min, regular rhythm
BP - 104/72 mm Hg
Respiratory Rate 25 /min
Temperature-101^F
Anemia- Mild
Jaundice/Cyanosis/ clubbing/koilonychia/leukonychia/flapping
tremor/edema/dehydration -Absent
Lymph Node- not palpable
Thyroid gland- normal
JVP-Not raised
Skin survey revealed pinkish to violet coloured painless macular skin rash
in both lower limbs, more in legs, along with eccymoses in both the
popliteal fossae.There were some non palpable purpuric spots present
in the abdomen & upper limbs.
• Repiratory System
Trachea- deviated to right
Chest movement asymmetrical
Reduced chest expansion in left mid zone & lower zone( <1.5 cm ),apex
beat in left 6th ICS medial to the MCV line, normal in character
Vocal fremitus reduced in left mid to lower zone (4th ICS downwards) but
normal in the rest of the lung fields.
Percussion note was stony dull & breath sound absent in the
aforementioned area of the lung, along with reduced vocal resonance.
Rest of the lung field was normal.There was no added sound.
Except some reddish to purplish non palpable purpura in the abdomen,
abdominal exam did not yield any positive sign i.e. any palpable organ or
any abdominal/ pelvic mass or ascitis & there was no scare mark.
Cardiovascular exam revealed no sign of pericardial
effusion/pericarditis/vulvular heart disease/HF.All the peripheral pulses
were palpable with normal character, there was no audible bruit.
Examination of the Musculoskeletal system was not remarkable for any
sign suggestive of tender/swollen joint.
Nervous system examination revealed no cognitive impairment,HPF was
normal.Cranial nerves in conjunction with motor system & sensory system
were intact.Fundoscopy revealed no sign of retinal haemorrhage.There
was no cerebellar sign.
Problem List
History
Recurrent respiratory distress
Fever
Rash involving both lower limbs & trunk
Physical Examination
Anemia
Non palpable purpura & echymosis
Left sided massive pleural effusion
Provisional Diagnosis
SLE
Points in favour
Fever
Recurrent pleural effusion
Anemia
History of polyrthritis
D/D
• Anti-Phospholipid Syndrome
• Dengue Fever
• Systemic Vasculitis
• Atypical Pneumonia
Investigations
Blood Picture
Date 24.03.19 03.06.19 10.06.19 11.06.19 17.06.19
Hb% 9.8 8.8 11.1 9.3 8.4
RBC (/cmm) 4,78,000 4,54,000 5,09,000 4,24,000 3,75,000
WBC (/cmm) 17,500
(Neutrophil-80%
Lymphocyte-
10%)
9000
(Neutrophil-63%
Lymphocyte-
29%)
9,000
(Neutrophil-65%
Lymphocyte-
30%)
10,230
(Neutrophil-83%
Lymphocyte -
11.9%)
7,870
Neutrophil-
68.6%
Lymphocyte -
20.5%)
Platelet (/cmm) 3,50,000 3,30,000 4,45,000 3,16,000 3,23,000
Hct % 32.2 30 34 29.3 28.2
ESR (mm/hr) 41 74 60 112 -
PBF (11.06.19)
● Anisopoikilocytosis with Microcytic hypochromic anemia
● WBCs are mature , No blast cell
● Platelets are normal in distribution
Sputum for AFB & Gene Xpert (-)ve (27.03.19) (ICDDR,B)
Dengue NS1 (-)ve (03.06.19)
Chest X-ray PA view
Date 24.03.19 28.03.19 15.04.19 03.06.19 10.06.19
Impression Right sided
pleural effusion
No fibrosis or
patchy opacity
Right sided
pleural effusion
No fibrosis or
patchy opacity
Right sided
pleural effusion
(improved)
No fibrosis or
patchy opacity
Left sided
massive
pleural effusion
No fibrosis or
patchy opacity
Left sided
pleural effusion
-improved
No fibrosis or
patchy opacity
Urine R/M/E (10.06.19)
Protein
Glucose
RBC cast.
WBC cast.
RBC (Isomorphic ) Nil
Dysmorphic RBC
Hyalin cast
Pus cell 8-10/HPF
UPCR- 0.35 (13.06.19)
Thyroid profile (10.06.19)
TSH -2.10 pg/mL
FT3 -1.68 ng/dL
FT4 -1.80 uIU/L
Pleural fluid
Drainage note - With all aseptic precautions, about 1L of straw coloured pleural
fluid was drained.Appearance was clear, neither turbid nor hemorrhagic.
Study report (10.06.19)
Microscopic examination
WBC 390/cmm (90% lymphocyte, 5% plasma cells , 5% lymphocyte)
RBC 310/cmm
Bacteriological examination
Gm stain - no organism detected
Zn Stain -AFB not found
Biochemistry
Glucose 9.0 g/dL (markedly reduced)
Protein 5.0 g/dL (mildly elevated)
Pleural fluid ADA -38 U/L
USG (13.06.19)
Normal size of liver or spleen
No abdomino-pelvic lymphadenopathy
No ascitis / pericardial effusion
Moderate effusion in left pleural cavity having multiple septations
PT - 13.3 sec (control 12.0 sec) (16.06.19)
INR -1.11 (16.06.19)
APTT -29.0 sec (control 28.0 sec) (16.06.19)
HBsAg
Anti HCV. Negative (10.06.19)
HIV screening
Antibody Titre Ref. Value Inference
ANA (ELISA) 5.5 U/mL <10 U/mL Negative
c-ANCA (ELISA) 3.10 U/mL <6.00 U/mL Negative
p-ANCA (ELISA) 2.88 U/mL <6.00 U/mL Negative
Anti-ds-DNA
(ELISA)
10.2 U/mL <30.0 U/mL Negative
Antibody Titre Reference
range
Inference
aPL Ig-M
(ELISA)
22.5 <10 U/mL POSITIVE
aPL Ig-G
(ELISA)
5.23 <10 U/mL Negative
aCL Ig-M
(ELISA)
22.0 <10 U/mL POSITIVE
aCL Ig-G
(ELISA)
4.82 <10 U/mL Negative
Skin biopsy was planned but was not possible according to the opinion of
Department of Dermatology, DMCH.
Final Diagnosis
SLE
Follow Up- After 1 month
Further plan
-Repeat ANA , aCL Ig-M & Ig-G, aPL Ig-M & Ig-G after 3 month
-Biopsy from affected skin lesion, if recurrence
-Fundoscopic examination in each follow up.
-VDRL test
-Echocardiogram
-CTPA
-Duplex study of lower limbs
-ENA Profile
Inputs &
Discussion
Anti-Phospholipid Syndrome Grand Round Presentation Dhaka Medical College Hospital (Medicine Unit 2)

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Anti-Phospholipid Syndrome Grand Round Presentation Dhaka Medical College Hospital (Medicine Unit 2)

  • 1. A-20-year-old female presenting with fever, respiratory distress & skin rash Dr.Md Shadman Shakib Intern Doctor Dhaka Medical College Hospital.
  • 2. Salient Features Mrs.Shanta, 20-year-old-woman, hailing from Jurain, Dhaka was seen by the medicine admission unit on 9th June,2019 at this hospital for respiratory distress , fever & rash involving both lower limbs, trunk & upper limbs.
  • 3. On the day of admission, she presented with respiratory distress (MRC grade 3) for last 5 days associated with occasional dry cough but not associated with orthopnea or PND or any history of SOB since childhood. She also complains high grade intermittent fever, the highest recorded temperature was 103 degree F that was not associated with any chills & rigors, but relieved with paracetamol.She also noticed pinkish to violet
  • 4. coloured skin rash that is occasionally itchy, painless & gradually both lower limbs,trunk & upper limbs for last 4 days On query, she gives a history of non specific joint pain involving small joints of hands & also knee joint 3 days back that was associated with swelling of the entire hand, spontaneously resolved on the day before admission.She also gave a history of 1 bag whole blood transfusion at a local clinic on that
  • 5. day.No reaction occured.She denied any history of abdominal pain,jaundice,cough with productive sputum,previous history of low grade fever associated with night sweat & evening rise of temperature ,chest pain, hemoptysis , history of leg swelling or swelling of abdomen,bloody urine or stool, nasal crusting, epistaxis, weight gain, cold intolerance, constipation, hisory of pregnancy or spontaneous
  • 6. abortion/loss of pregnancy, convulsion, gum bleeding, oral ulcer, alopecia, photosensitivity, menorrhagia or passage of any blood clots during menstruation, per vaginal discharge or burning micturition or lower abdominal pain, significant weight loss, bone painExtramarital affair, h/o travelling to Malaria or Kala Azar endemic zone, previous history of asthma or similar type of illness in childhood, h/o vaccination with
  • 7. tetanus toxoid.Drug history was negative for taking any anti- arrhythmic,anti-hypertensive, anticonvulsant or biologic drugs or consumption of INH, minocycline & pyrazinamide . 1 weeks before this evaluation, the patient was seen in the OPD of a private hospital.She was diagnosed with left sided pleural effusion & was prescribed Cefuroxime-Clauvulonic acid & paracetamol along with advise for hospitalisation, however she denied to be admitted there.
  • 8. Menstrual history Period 5-7 d, Cycle 21-28 Flow -average Contraceptive history Did not adopt any contraceptive method so far.
  • 9. Patients family history was not noteworthy of asthma/ TB or any rheumatological condition or any history of pregnancy loss in mother or siblings.She is married for 6 months & her electrician husband works in Oman.She lives in her in laws house situated in old town of Dhaka.
  • 10. Past history 10 weeks before this presentation, when she noticed fever , respiratory distress associated with dry cough & sore throat for 3 days, patient was evaluated in the OPD of another tertiary care hospital.The patient was empirically treated as a case of Para-pneumonic effusion.Initially she was prescribed azithromycin, pantoprazole, paracetamol for 7 days, later Azithromycin was substituted with combination of co amoxiclav &
  • 11. clarithromycin & patient was Improved. 8 weeks before this evaluation,during follow-up at that that hospital, her condition was slowly improving, however, she was again prescribed co amoxiclav & clarithromycin for 14 days in addition to salbutamol & fluticasone inhaler, montelukast & rabeprazole.She took them accordingly & she describes her condition was stable & she was symptom free after taking them.
  • 12. Physical Examination General Examination Ill looking, anxious Body Built- Average Co-operative, Decubitus on choice Pulse -90/min, regular rhythm BP - 104/72 mm Hg Respiratory Rate 25 /min Temperature-101^F
  • 13. Anemia- Mild Jaundice/Cyanosis/ clubbing/koilonychia/leukonychia/flapping tremor/edema/dehydration -Absent Lymph Node- not palpable Thyroid gland- normal JVP-Not raised
  • 14. Skin survey revealed pinkish to violet coloured painless macular skin rash in both lower limbs, more in legs, along with eccymoses in both the popliteal fossae.There were some non palpable purpuric spots present in the abdomen & upper limbs.
  • 15.
  • 16.
  • 17. • Repiratory System Trachea- deviated to right Chest movement asymmetrical Reduced chest expansion in left mid zone & lower zone( <1.5 cm ),apex beat in left 6th ICS medial to the MCV line, normal in character Vocal fremitus reduced in left mid to lower zone (4th ICS downwards) but normal in the rest of the lung fields.
  • 18. Percussion note was stony dull & breath sound absent in the aforementioned area of the lung, along with reduced vocal resonance. Rest of the lung field was normal.There was no added sound. Except some reddish to purplish non palpable purpura in the abdomen, abdominal exam did not yield any positive sign i.e. any palpable organ or any abdominal/ pelvic mass or ascitis & there was no scare mark.
  • 19. Cardiovascular exam revealed no sign of pericardial effusion/pericarditis/vulvular heart disease/HF.All the peripheral pulses were palpable with normal character, there was no audible bruit. Examination of the Musculoskeletal system was not remarkable for any sign suggestive of tender/swollen joint.
  • 20. Nervous system examination revealed no cognitive impairment,HPF was normal.Cranial nerves in conjunction with motor system & sensory system were intact.Fundoscopy revealed no sign of retinal haemorrhage.There was no cerebellar sign.
  • 21. Problem List History Recurrent respiratory distress Fever Rash involving both lower limbs & trunk Physical Examination Anemia Non palpable purpura & echymosis Left sided massive pleural effusion
  • 23. SLE
  • 24. Points in favour Fever Recurrent pleural effusion Anemia History of polyrthritis
  • 25. D/D • Anti-Phospholipid Syndrome • Dengue Fever • Systemic Vasculitis • Atypical Pneumonia
  • 26. Investigations Blood Picture Date 24.03.19 03.06.19 10.06.19 11.06.19 17.06.19 Hb% 9.8 8.8 11.1 9.3 8.4 RBC (/cmm) 4,78,000 4,54,000 5,09,000 4,24,000 3,75,000 WBC (/cmm) 17,500 (Neutrophil-80% Lymphocyte- 10%) 9000 (Neutrophil-63% Lymphocyte- 29%) 9,000 (Neutrophil-65% Lymphocyte- 30%) 10,230 (Neutrophil-83% Lymphocyte - 11.9%) 7,870 Neutrophil- 68.6% Lymphocyte - 20.5%) Platelet (/cmm) 3,50,000 3,30,000 4,45,000 3,16,000 3,23,000 Hct % 32.2 30 34 29.3 28.2 ESR (mm/hr) 41 74 60 112 -
  • 27. PBF (11.06.19) ● Anisopoikilocytosis with Microcytic hypochromic anemia ● WBCs are mature , No blast cell ● Platelets are normal in distribution Sputum for AFB & Gene Xpert (-)ve (27.03.19) (ICDDR,B) Dengue NS1 (-)ve (03.06.19)
  • 28. Chest X-ray PA view Date 24.03.19 28.03.19 15.04.19 03.06.19 10.06.19 Impression Right sided pleural effusion No fibrosis or patchy opacity Right sided pleural effusion No fibrosis or patchy opacity Right sided pleural effusion (improved) No fibrosis or patchy opacity Left sided massive pleural effusion No fibrosis or patchy opacity Left sided pleural effusion -improved No fibrosis or patchy opacity
  • 29. Urine R/M/E (10.06.19) Protein Glucose RBC cast. WBC cast. RBC (Isomorphic ) Nil Dysmorphic RBC Hyalin cast Pus cell 8-10/HPF
  • 30. UPCR- 0.35 (13.06.19) Thyroid profile (10.06.19) TSH -2.10 pg/mL FT3 -1.68 ng/dL FT4 -1.80 uIU/L
  • 31. Pleural fluid Drainage note - With all aseptic precautions, about 1L of straw coloured pleural fluid was drained.Appearance was clear, neither turbid nor hemorrhagic. Study report (10.06.19) Microscopic examination WBC 390/cmm (90% lymphocyte, 5% plasma cells , 5% lymphocyte) RBC 310/cmm
  • 32. Bacteriological examination Gm stain - no organism detected Zn Stain -AFB not found Biochemistry Glucose 9.0 g/dL (markedly reduced) Protein 5.0 g/dL (mildly elevated) Pleural fluid ADA -38 U/L
  • 33. USG (13.06.19) Normal size of liver or spleen No abdomino-pelvic lymphadenopathy No ascitis / pericardial effusion Moderate effusion in left pleural cavity having multiple septations
  • 34. PT - 13.3 sec (control 12.0 sec) (16.06.19) INR -1.11 (16.06.19) APTT -29.0 sec (control 28.0 sec) (16.06.19) HBsAg Anti HCV. Negative (10.06.19) HIV screening
  • 35. Antibody Titre Ref. Value Inference ANA (ELISA) 5.5 U/mL <10 U/mL Negative c-ANCA (ELISA) 3.10 U/mL <6.00 U/mL Negative p-ANCA (ELISA) 2.88 U/mL <6.00 U/mL Negative Anti-ds-DNA (ELISA) 10.2 U/mL <30.0 U/mL Negative
  • 36. Antibody Titre Reference range Inference aPL Ig-M (ELISA) 22.5 <10 U/mL POSITIVE aPL Ig-G (ELISA) 5.23 <10 U/mL Negative aCL Ig-M (ELISA) 22.0 <10 U/mL POSITIVE aCL Ig-G (ELISA) 4.82 <10 U/mL Negative
  • 37. Skin biopsy was planned but was not possible according to the opinion of Department of Dermatology, DMCH.
  • 39. Follow Up- After 1 month Further plan -Repeat ANA , aCL Ig-M & Ig-G, aPL Ig-M & Ig-G after 3 month -Biopsy from affected skin lesion, if recurrence
  • 40. -Fundoscopic examination in each follow up. -VDRL test -Echocardiogram -CTPA -Duplex study of lower limbs -ENA Profile