AUTOIMMUNITY OF 13F

 This is an a online e log book to discuss our patient de-identified health data shared after taking his / her / guardians signed informed consent. Here we discuss our individual patients problems through series of inputs from available global online community of experts with an aim to solve those patients clinical problem with collective current best evident based input.


This E blog also reflects my patient centered online learning portfolio and your valuable inputs on the comment box is welcome.


I have been given this case to solve in an attempt to understand the topic of " patient clinical data analysis" to develop my competency in reading and comprehending clinical data including history, clinical findings, investigations and come up with diagnosis and treatment plan.

13year old female 

Came with chief complaints of Shortness of breath since yesterday (decreased now)

4 episodes of vomitings since yesterday 10pm


Birth history

1st child 

2nd degree consanguineous marriage 

Born in 2010

LSCS

Father has no idea about immunisation status


Mother-has 2 children

The current pt is the elder one(birth in 2010)

2nd child born in 2013

In 2014 mother diagnosed with kochs-expired in 2022 sept(did not use ATT regularly)


Patient was apparently asymptotic till the age of 11years

She was sent to hostel for studies

After few days of hostel stay she noticed that she has bilateral neck swellings 

So she was taken to RMP with complaints of neck swellings,fever and cough on and off

RMP has initiated her on ATT as her mother has also has kochs

They used ATT for 2months started in 2021 june

After initiating ATT fever increased so they stopped ATT and was referred to Hyd by the RMP

Patient was taken to NF hospital where she was evaluated for kochs but none of the investigations showed AFB,at that time she also had complaints of knee pains and wrist joint pains

In view of joint pains she was referred to N hospital 

In N hospital they suspected it to be autoimmune and started her on Tab Wysolone and Tab HCQ ,which she used for 15 days and stopped and later did not go there for follow up

(ANA ELISA-equivocal,ANA IFA-negative,Anti Ds DNA ELISA-Positive,Anti Ds DNA IFA negative)


She was taken to another local hospital with c/o joint pains,facial puffiness,pedal edema,fever ,cough

Lymph node biopsy was done in May 2022 ?reactive(no report available but attendor was informed that it was negative for kochs)

So Mycobacterial gene expert test was done on blood sample which was also negative

But she was initiated on ATT empirically on may/2022.

10-15days before starting ATT attendors have noticed that she is developing facial rash and Hair loss,due to hair loss scalp rash also became evident.


On ATT (started in May)-in the months of August and sept she has complete loss of appetite,generalised weakness and weight loss(9kgs in 2yrs),attendors have lost hope and thought that she may not survive anymore in sept 2022,but later in October and nov her appetite imporved ,pedal edema decreased.

ATT stopped in October(6months)

She even went to school for 1month in December 2022

But again in Jan 2023 she started developing pedal edema,facial puffiness,pain abdomen after taking food,so she was not eating properly


She was taken to hospital in Jan 2023,doctors have adviced for further tests (as CUE showed proteinuria) and evaluation but as attendors felt that she is ok apart from that pedal edema and facial puffiness they took her home.

From yesterday night she had 4episodes of vomitings ,food as content,non bilious and shortness of breath (decreased now),so she was brought to our hospital



TREATMENT HISTORY:

History of Anti Tubercular therapy for 6 months 1 year back.

PERSONAL HISTORY:

Diet - Mixed

Appetite - Normal

Sleep - Decreased

Bowel and Bladder - Oliguria since 5 days, Bowel movements are normal

No Addictions

 

FAMILY HISTORY:

Mother was diagnosed with Tuberculosis in 2014.

ATT course was not taken completlely.

Symptoms got worse in 2022 and Passed way in 2022


MENSTRUAL HISTORY: 

Not attained menarche

 

GENERAL EXAMINATION:

Patient is conscious, coherent and cooperative and well oriented to time, place and person.

After taking consent , Patient is examined in a well lit room

PALLOR - PRESENT

ICTERUS - Absent

CYANOSIS - Absent

CLUBBING-Absent

PEDAL EDEMA- PRESENT, BILATERAL , GRADE 1 PITTING TYPE

 

VITALS : 

TEMPERATURE - AFEBRILE

BLOOD PRESSURE - 130/80 mmhg right arm supine position 

No pulsus paradoxsus

PULSE RATE - 110bpm , Regular, Normal volume

RESPIRATORY RATE - 32cpm

SpO2 - 99% at room air










SYSTEMIC EXAMINATION:


CARDIOVASCULAR SYSTEM EXAMINATION:


    INSPECTION:

    CHESTWALL SHAPE - NORMAL

    PRECORDIAL BULGE - ABSENT

    PECTUS CARINATUM OR PECTUS EXCAVATUM ARE ABSENT

    KYPHOSCOLIOSIS - ABSENT

    NO DILATED VEINS ,SCARS, SINUSES

    APICAL IMPULSE IS NOT SEEN

    NO PULSATIONS ARE SEEN

JVP-NORMAL


PALPATION:

    Kyphoscoliosis is absent

    Apical Impulse -NOT FELT

    No pulsations felt

    No thrills felt

    No dilated veins felt


    PERCUSSION:

    Right heart border is Normal

    Left Heart border - INCREASED DULLNESS ON LEFT SIDE

     

    AUSCULTATION:

    S1S2 heard in all areas-Faint,soft,muffled

 

RESPIRATORY SYSTEM EXAMINATION

     Inspection -

    Chest is symmetrical
    Trachea is midline
    Bilateral air entry is present 
    No retractions
    No kyphoscoliosis
    No Winging of scapula
    No Scars, sinuses, Dilated Veins
    All areas move equally and symmetrically with respiration
 
    Palpation - 
 
    Trachea is Midline
    No tenderness, local rise in temperature
    Tactile Vocal Fremitus 
        
                                       Right        Left
    Supra clavicular:  Present  Present    
    Infra clavicular:    
Present Present  
    Mammary:            
Present  Diminished
    Infra mammary: Diminished Diminished
    Axillary:                Present    Diminished
    Infra axillary:     Diminished Diminished
    Supra scapular:  
Present       Present
    Infra scapular:   Diminished Diminished
    Inter scapular:    Present     Present
 
 
    Percussion - 
 
                                        Right        Left
    Supra clavicular:  resonant  resonant    
    Infra clavicular:    resonant  resonant  
    Mammary:           resonant  
DULLNESS 
    Infra mammary:  DULLNESS DULLNESS 
    Axillary:                resonant   DULLNESS 
    Infra axillary:     DULLNESS DULLNESS
    Supra scapular:   resonant   resonant
    Infra scapular:   
DULLNESS DULLNESS 
    Inter scapular:    resonant   resonant   
    No tenderness

    Auscultation - 
                                      Right         Left

    Supra clavicular:   NVBS       NVBS
    Infra clavicular:     NVBS       NVBS
    Mammary:             NVBS      
Diminished
    Infra mammary:Diminished   Diminished
    Axillary:                 NVBS       Diminished
    Infra axillary:   Diminished Diminished
    Supra scapular    NVBS          NVBS
    Infra scapular: Diminished 
Diminished
    Inter scapular:   NVBS           NVBS

 
    Vocal Resonance 
                                         Right         Left

    Supra clavicular: Resonant   Resonant
    Infra clavicular:  
Resonant    Resonant
    Mammary:        Resonant   Diminished
    Infra mammary:Diminished Diminished
    Axillary:           Resonant  Diminished
    Infra axillary:   Diminished      Diminished
    Supra scapular:Resonant     Resonant
    Infra scapular: Diminished Diminished
    Inter scapular: Resonant     Resonant
 
    No added Sounds
    No Bronchophony, Egophony, Whispering Pectoriloquy
 
    ABDOMINAL EXAMINATION:
    INSPECTION
 
    SHAPE - distended 
    UMBILICUS - Inverted

    SKIN -  No scars, sinuses, scratch marks, striae, no dilated veins, hernial orifices free, skin over the abdomen is smooth 
    EXTERNAL GENETILIA - Normal
 
    PALPATION
 
    No local rise in temperature, no local tenderness
    Liver not palpable
    Spleen not palpable
    Kidneys are not palpable
     
    PERCUSSION 

    Shifting Dullness - present
    Liver span - Normal
    Spleen Percussion - Normal
    Tidal Percussion - Absent

    AUSCULTATION

    Bowel sounds are heard
    No bruit or Venous hum

    CENTRAL NERVOUS SYSTEM EXAMINATION: 
    HIGHER MENTAL FUNCTIONS INTACT
    CRANIAL NERVE EXAMINATION - NORMAL
    SENSORY SYSTEM EXAMINATION - NORMAL
    MOTOR EXAMINATION - NORMAL
     NO MENINGEAL SIGNS
     

    PROVISIONAL DIAGNOSIS:
   PERICARDIAL EFFUSION WITH BILATERAL PLEURAL EFFUSION
 WITH ASCITES ?POLYSEROSITIS
?ANA NEGATIVE SLE
?EXTRA PULMONARY KOCHS

INVESTIGATIONS 



  • MONEY BAG SHAPED HEART SHADOW
  • ENLARGED CARDIAC SHADOW 
  • LOSS OF COSTO-PHRENIC ANGLE ON BOTH SIDES
ECG:

ECHO

MASSIVE PERICARDIAL EFFUSION WITH NO RV DIASTOLIC COLLAPSE



ULTRASONOGRAM
  • Liver, gall bladder , pancreas , spleen , uterus , ovaries are normal.
  • Mild Ascitis
  • Bilateral Pleural Effusion
  • Moderate Pericardial Effusion
  • Bilateral Grade 2 Renal Pelvis Dilatation changes
  • Sub-mucosal edema of small bowel loops 
CBP:


CBP showing Anemia-?Anemia of chronic disease ?Hematological manifestation of SLE,coexisting iron and b12 deficiency 


ARTERIAL BLOOD GAS:
 
pH - 7.4
PCO2- 14.9mmhg
pO2 - 79.8mmhg
HCO3 - 9.2 mmol/l
O2 saturation - 96%
 
SERUM ELECTROLYTES
 
    SODIUM - 136 meq/l
    POTASSIUM - 4.4meq/l
    CHLORIDE - 106meq/l
RENAL FUNCTION 

  SERUM CREATININE - 0.6mg/dl
BLOOD UREA 29mg/dl

LIVER FUNCTION TEST:


     
ESR - 70mm
CRP Negative
 
FBS - 100mg/dl
BLOOD GROUP - O positive
Rheumatoid factor - Negative
HIV - non reactive
HbS AG - non reactive
HCV-non reactive

 
URINE EXAMINATION:
Colour - Pale yellow
Appearance - Clear
Reaction - Acidic
Specific Gravity - 1.010
Albumin -  ++
No sugar, bile pigments, RBC, Crystals, Casts, Amorphous deposits
Pus Cells - 3 to 4 /hpf
Epithelial cells - 2 to 3 / hpf
Spot upcr-10
 
24 hour Urine Protein - 654 mg/day (normal - <150mg/day) 
24 hour urine creatinine -0.9
Ratio -0.07
Urine volume 300ml

NEPHROLOGIST REFERRAL:Advice for renal biopsy 

DERMATOLOGIST REFERRAL:Scabies ,adviced for strict isolation





PREVIOUS RELEVANT REPORTS

ANA ELISA - EQUIVOCAL
ANA IFA - NEGATIVE
ANTI dsDNA ELISA - POSITIVE
ANTI dsDNA IFA - NEGATIVE 
C3,C4 levels NORMAL

FINAl DIAGNOSIS:
POLYSEROSITIS-Massive pericardial effusion,B/L pleural effusion,Ascites
GLOMERULONEPHRITIS
CUTANEOUS INVOLVEMENT
?AUTOIMMUNE ETIOLOGY-ANA NEGATIVE LUPUS(as IF ANA IS NEGATIVE)
SCABIES

SLEDAI score
arthritis+:4
Proteniuria+:4
Pericarditis+:2
Rash 2
Alopecia 2
Pleurisy 2
—————-
Total:16


TREATMENT:
INJ.METHYL PREDNISOLONE  250mg IV OD for 3days (Pulse therapy was considered as massive pericardial effusion can be life threatening)
Converted to Tab WYSOLONE 40Mg given for 7days post pulse therapy
Tab HCQ 50mg BD
INJ LASIX 40Mg IV BD
TAB ALDACTONE 50mg OD
Salt and fluid restriction 
Protein rich diet
PERMITE lotion applied on day 1 and at home after discharge on day 8

Advice at discharge 
Tab WYSOLONE 30Mg OD
Tab HCQ 50Mg BD
Tab LASIX 10Mg BD

Review USG at discharge: No pleural effusion and ascites(resolved)
——————————-

PAJR DISCUSSION POST DISCHARGE 
———————————
Patient came for review on 27/3/2023
C/o abdominal distension increased 
C/o post prandial pain abdomen resolving on its own
C/o shortness of breath on exertion

Plan for renal biopsy on 29/3/2023
Scabies treatment completed

Investigations:on 29/3/2023

Chest x ray:



ECHO on 27/3/2023

Significant reduction of the pericardial effusion 

Serum albumin has dropped from 4–>2 ,worsening proteinuria

PT -16SEC
INR-1.11
APTT-17SEC

RBS-107
CUE-4+ albumin
Spot upcr-5.5

24hr urine
Protein:1636mg/day
Creatinine 0.5g/day
Ratio 3.27
Urine volume : 600ml

Urea 55
Creat 0.5

Ascites has reaccumilated ?Due to worsesned proteinuria
?Asymptomatic gall stones with serositis changes of gall bladder

Plan 24hr hour collection,Renal biopsy
Plan to add Cyclophosphamide/MMF 

RENAL BIOPSY DONE ON 29/3/2023

Renal biopsy procedure :YOUTUBE LINK



After making a USG guided marking of left kidney renal biopsy is done on 29/3/2023


Patient was discharged 2days after the procedure.

On 4/4/2023


Patient was brought to the casualty at around 9 20am


Initially drowsy but arousable after 5 min 

Patient Conscious coherent

Oriented to time place person

Gcs - 15

Inconsolable cry due to headache



??Gtcs for 5 minutes at 6:30 while cooking in the kitchen 

Frothing+ uprolling eye balls+no involuntary micturation+ tongue bite+

Fall+ sustained head injury at frontal area


5- 6 such episodes for 2 min while travelling to area hospital and was given Midaz 

Seizure activity subsided and was brought here for further evaluation


In casualty Patient 

Vitals- 

Bp- 180/120mmHg

Pr - 122 cpm

Rr- 24cpm

Grbs -118mg/dl


Pupils - reactive bilaterally 

Hypotonia all limbs

Reflexes

B.   +      +

T.   +       +

S.    +     +

K.   +      +

A.  +       +

P F.       E


soft swelling over frontal area( 5×5cms)


Auscultation 

Lungs Bae+


Plan :NEUROIMAGING ,ECHO,RFT


Renal BIOPSY Reported on 4/4/2023 just after the patient presented to casualty with seizures


Features suggestive of class 4 LUPUS NEPHRITIS(DIFFUSE GLOBAL PROLIFERATE AND SCLEROSING LUPUS NEPHRITIS)

List of differentials:
1.Hypertensive nephritic PRES
2.CNS thrombosis due to anti phospholipid antibody
3.CNS vasculitis 
4.Cardiac vegetations can cause cardio embolia stroke


MRI brain suggestive of Hypertensive encephalopathy -PRES
Patient was started on anti Hypertensives ,anti epileptics
After initial stabilization attendors were counselled about cyclophosphamide therapy and risks associated.


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