CASE OF LUPUS NEPHRITIS IN PEDIATRIC POPULATION

1. CASE
PRESENTATION
MODERATED BY UNIT-2
Dr.Aheed Khan
DNB Resident
Second year
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2. PARTICULARS
➤ 14 year old boy
➤ Resident of east Delhi
Chief complaints of:
1.Multiple Joint Pains for 15 days
2. Fever for 10 days
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3. HOPI:
➤Joint pains:
➤For 15 days
➤initially B/L ankle, not a/w swelling or redness,
➤then progressed to B/L shoulder joint->wrist->knee-
>small joint in symmetric fashion
➤severe in intensity,
➤causing restriction of movement
➤relieved by medication
➤affecting sleep and activity

4. HOPI:
➤ FEVER:
➤ For 10 days, high grade, undocumented, continuous, relieved
by medication
➤ onset 5 days after joint pains started
➤ not a/w chills
➤ Accompanied with rash, throat pain, difficulty in swallowing,
generalised body ache
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5. RASH
➤ started as vesicles on lower extremities as vesicles, 10 days
ago with onset of fever,
➤ on day of admission developed rashes over trunk-reddish, 3x4
cm, asymmetric, flat, non-itchy.
➤ started on Lower extremities—>trunk—> upper extremities
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6. OTHER HISTORY
➤ oral medication from OPD for 2 days on opd basis- Antipyretic [paracetemol]
➤ Admitted in PVT nursing home from 11-06-2018 to 16-06-2018
➤ Given- IV fluid/iv antibiotic/iv antipyretics-referred pediatrician.
➤ Seen by Pediatrician-Oral prednisolone +PCM + Cefixime
➤ Outside Investigation- Hb- normal, TLC-normal ,Platelet-1.20 lakh, blood-c/s- sterile, Urine r/m-normal, KFT-
Normal , LFT-Normal
➤ MTX-NR, Chest-X-ray =normal, USG-normal
➤ Parents brought to Max hospital on 19 June 2018, as fever and joint pains persisted and child was uneasy.

7. NO HISTORY OF
➤ headache, blurred vision, abnormal behaviour, vomiting.
➤ burning micturition
➤ cold/cough
➤ palpitation, breathlessness
➤ reddishness of eyes
➤ swelling at any site
➤ oral ulcers
➤ loose stools or constipation
➤ recent weight loss
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8. PAST HISTORY
No h/o
➤ similar complaints in the past,
➤ previous hospitalisation or surgery,
➤ prolonged medication.
➤ TB contact

9. Family history:
➤ only child
➤ h/o hypertension in mother
➤ no h/o similar illness in the past
Personal history:
➤ Goes to school 9th grade
➤ Good in studies

10. Diet history:
➤ non-Vegetarian by diet
➤ Calorie and pr Intake adequate
Socio-Economic
➤ Modified Kuppuswami scale- upper middle class

11. ➤Birth and Developmental history was normal
➤immunisation done from family paediatrician
➤last vaccine at 5 years

12. ANTHROPOMETRY
➤ Height- 160 CM
➤ (b/w 25-50th)
➤ Weight-48.64 kg
(b/w 25-50th)
➤ BMI: 19 [at 50th
centile]

13. GPE [AT ARRIVAL]
➤ Conscious, well oriented to time, place and person
➤ uncomfortable, restricting movement
➤ Resp Rate- 20/min; no signs of distress
➤ SpO2: 99 % on room air
➤ pulse= 102/min, good volume, regular, all peripheral
palpable, no RR, RF delay
➤ BP=on arrival: 120/82 mm of mercury on, in left
brachial artery in supine position;
➤ Temp- 101 F in right axilla

14. No
➤ pallor,
➤ Icterus,
➤ Cyanosis,
➤ Lymphadenopathy,
➤ pedal edema,
➤ clubbing

15. HEAD TO TOE EXAMINATION
➤head normal- no dysmorphism
➤rash- multiple maculopapular, asymmetrical, non-blanchable, non-
scaly, non-friable rash, present all over the body, with healing seen
over lower extremities.
➤hairs and nails normal
➤eyes normal, no congestion
➤oral cavity normal
➤BCG scar +
➤spine normal
➤joint/muscles- no joint swelling or redness. child restricting movement
due to pain. no gross muscle wasting
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16. Cns exam:
➤ higher functions- normal
➤ Motor and sensory examination- normal
➤ Reflexes- normal
➤ meningeal signs- absent
Systemic examination:

17. Per abdomen:
➤all quadrants moving normally,umbillicus
central, no visible veins, rash or lump seen.
All hernial sites intact, genitalia normal.
➤soft, non-tender, no hepatosplenomegaly
➤no fluid thrill
➤bowel sounds normal

18. Respiratory System :
➤ chest b/l symmetrical in shape
➤ trachea central
➤ air entry b/l equal on auscultation
➤ no adventitious sounds
Cardiovascular System:
➤ Precordium looks normal
➤ apex beat palpated at 5th ICS @MCL
➤ no thrill or parasternal heaves
➤ s1s2 heard on auscultation
➤ no murmurs present
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19. SUMMARY
➤ A 14 year old boy presented with ℅ multiple joint pains for 15
days, gradually involving major joints and restricting
movement, and fever for 10 days, a/w rash present over lower
extremities and trunk. General and Systemic examination was
normal.
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20. FEVER + JOINTS PAIN + VESICULAR RASH
➤ Infective – Dengue, Chikungunya , Rheumatic
Fever viral exanthem
➤ Non-infective- JIA, CTD,
➤ Investigations sent- CBC, CRP, Blood culture,
peripheral smear, SGPT, weil-felix, chikungunya
PCR
➤ ASO,CRP, ESR, ANA

21. ADMISSION
➤ Started on IV Fluid / I.V. antibiotic(ceftriaxone)/ oral analgesic
➤ Oral Naproxen added
➤ Vitals charting 2 hrly
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22. INVESTIGATIONS- AT ADMISSION(19TH
JUNE)
HB 12.4gm/dl
TLC 13200
DLC
N-83% L-8% M2% E-5% B-0% BC-
2%
Platelet count 2.07 lakh
ESR
CRP
85mm/hr
24.0mg/dl (<5.0mg/dl)

23. INVESTIGATIONS…………..
➤ PS- MP-negative
➤ Malaria Ag- negative
➤ KFTBU= 50.8mg/dl, S.crt.=0.62mg/dl
➤ LFT Total protein =7.8gm/dl
Albumin =2.9gm/dl
Globulin =4.9 gm/dl
SGOT(AST) =11.3 IU/L
SGPT(ALT) =19.0 IU/L
Bilirubin (T) =0.41mg/dl

24. COURSE DURING HOSPITAL STAY-D1
➤ Started on IV fluid/ inj. Ceftriaxone/ IV analgesics
➤ BP-record(2-4 hrly),on higher side- 130/80 mmHg
➤ BP- Fluctuating- After 12 hours of admission had record of 170/110
mmHg associated with perspiration, palpitation and discomfort
➤ ECG-normal
➤ Shifted to PICU and labetolol infusion started
➤ Another oral antihypertensive started(amlodipine)

25. D2 –OF ADMISSION…
➤ BP- controlled
➤ Infusion of labetolol tapered over 24hrs
➤ Fever- persisting
➤ Joints pain +
An opinion from Endocrinologist Urine VMA, S.
cortisol,
Cardiologist opinion ECHO- normal

26. ➤ USG-KUB with renal doppler- normal
➤ Urine R/M- RBC + , No dysmorphic RBCs.
➤ ASO-normal
➤ CRP+
➤ ESR- increased

27. INVESTIGATIONS
➤ ANA(IF) =Positive(4+)
➤ ANA titre =1:2560
➤ ASO titre =51.0 IU/ml (<200)
➤ Weil Felix test-Negative

28. D2 –REPORT CAME
➤ ANA+
➤ dsDNA+
➤ SmD1+
➤ PO+
➤ Nucleosome+
➤ Histone+
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29. AUTO-ANTIBODIES
HISTONE Positive (+++)*
GBM Negative
MPO-ANCA Negative
PR3-ANCA Negative
CENP-B Negative
dsDNA Positive (+++)*
Jo1 Negative
NUCLEOSOME Positive (++)*
PO Positive (+++)*
ScL70 Negative
SmD1 Positive (+)*
SS-A/Ro52 Negative
SS-A/Ro60 Negative
SS-B/La Negative

31. OTHER INVESTIGATIONS
➤Rheumatoid factor- negative
➤c3 levels - 54mg% [decreased]
➤c4 levels- <8mg% [10-40]
➤urine protein/creat ratio-1.04
➤thyroid profile- wnl
➤cortisol- wnl
➤ Random urine Protein /creatinine ratio= 1.04[<0.2]
➤ Creatinine urine random=61.32 mg/dl

32. FURTHUR PLAN:
➤ Nephrologists- opinion for renal involvement
➤ Renal biopsy done-Report awaited
➤ INDICATION: Any patient ‘with SLE’ who has clinical or
laboratory evidence of active nephritis, especially with the
first episode of nephritis, requires Renal Biopsy.
➤ Child seen by rheumatologist advised oral HCQ + oral
steroid
➤ On D3-D4- symptoms of pain decreased, fever subsided,
well being improved
➤ Planned for Discharge on oral steroids[prednisolone &
hydroxycholoroquine]

33. KIDNEY BIOPSY
1. GBM is of normal thickness with patent capillary lumens which are not
bloodless or sclerosed.-
2.Mesangium - Shows increased cellularity. - Marked endocapillary
proliferationwith polymorphs and occasional neutrophilic
karyorrhectic debris seen.
3. No organized deposits,extracapillary proliferation or fibrinoid necrosis is
seen.
4. Tubulo-interstitium:- Tubules - Show protein reabsorption droplets.
5. Interstitium - No significant infiltrate seen.

34. IMMUNOFLUORESCENCE
Following immunostaining pattern is noted -
1.IgA =2 + mesangial and capillary wall;
2.granular IgG =3 + mesangial and capillary wall;
3.coarse granular IgM =2+mesangial and capillary wall;
4.granular C3 =2 + mesangial and capillary wall;
5.granular C1q =2 + mesangial and capillary wall;
6.granular Kappa lightchains=3 + mesangial and capillary wall;
7.granular Lambda light chains =3 +mesangialand capillary wall;
IMPRESSION: -Lupus Nephritis - Class IV-G

35. ACR -SLE DIAGNOSTIC CRITERIA

38. ➤ FINAL DIAGNOSIS:
➤ SLE with secondary
grade-IV Lupus
Nephritis
➤ Discharged on day 6

39. THA
NKS
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