CME INDIA Case Presentation by Dr Mritunjay Kumar Singh, Nephrologist Physician, Secretary, National CCDSI, HQ, Gaya.
CME INDIA Case Study:
Knowledge is a process of piling up facts, wisdom lies in their simplification. – Martin H Fischer
Two cases of SLE nephritis, second case is MCTD associated SLE
CASE 1:
What you think for this Presentation?
- A 26-year female presented with c/o
- Fever – 4 months
- Arthralgia – 1 months
- She was on ATT for last 3 months (previous c-xray showing right sided mild pleural effusion)
On Examination
- Patient is conscious, cooperative, lying comfortably on bed, well oriented to time, place and person
- Weight: 40 kg., height:158cm.
- Facial puffiness+
- Pallor++, no icterus, no cyanosis, no clubbing
- No lymphadenopathy, pedal edema+
- JVP-N, BP-110/70, P-96/min. regular fair in volume
- CHEST- BS diminished on right base
- CVS-S1&S2 heard, no murmur
- P/A – no organomegaly
- CNS- NONE, Neck-soft, Plantar-B/L down going
- Musculo skeletal system – swollen and tender joint, no deformity
Tests Done
- CBC – Hb-9.3g/dl, TLC-2900Cell/cumm., N-69, L26, M4
- PLT-58000/cu mm, ESR-40
- LFT-WNL,
- RFT-WNL,
- Na-137, k-3.87, cl 102, stp/alb-7.7/3.2
- U\R\M-alb1+, rbc-6to8, pus cell-plenty
- 24 hr. Urinary Protien-1200mg/day
So, we get these things in this case
- Prolonged Pyrexia
- Arthralgia
- Active urinary sediment and proteinuria
- Anemia
- Leucopenia
- Mild right sided Pleural effusion
ANA Test
Double Stranded Antibody – ds DNA test
So, Now you think it is SLE
- Chronic multisystem disease, involving joints, skin, serosal membranes, CNS and kidney.
- Predominantly affects women of child bearing age.
- Lupus nephritis occurs in 50% patients of SLE, usually within the 1st year of disease onset and is major cause of morbidity and mortality.
Would You Like to go for Renal Biopsy in this case?
Final diagnosis:
Focal lupus nephritis
Activity index-7/24, Chronicity index-0/12
How You Know about Activity Index?
NIH activity/chronicity index
Activity index
- Glomerular capillary hypercellularity
- Glomerular leukocyte infiltration
- Glomerular fibrinoid necrosis, karyorhexis
- Glomerular sub endothelial deposit (wire-loop lesion)
- Glomerular cellular crescents
- Interstitial inflammation
Chronicity index
- Glomerular sclerosis
- Glomerular fibrous crescent
- Tubular atrophy
- Interstitial fibrosis
What are RISK factors for Lupus Nephritis(LN)?
- Younger age
- Male sex
- Early course of lupus
- Black, Hispanics and Asians
- Genetic predisposition- apolipoprotein A (APOLI), platelet derived growth factor receptor alpha (PDGFRA), hyluronase synthase 2(HAS2).
- HLA-DR3/15 increases the risk, while HLA-DR4/11 appears to protect.
How You diagnose Lupus Nephritis?
- Urine dip-stick
- Urine microscopy
- Spot UPCR- can give inaccurate result in LN
- 24-hour urinary specimen
- 1st void early morning UPCR
- The gold standard for diagnosis and classification of LN is per-cutaneous renal biopsy.
Indication of renal biopsy in SLE
- Proteinuria > 500mg/day
- Nephritic sediment in urine (dysmorphic RBC,RBC cast or WBC cast) especially associated with proteinuria
- Worsening of renal function attributable to SLE
General principal management of LN
- Disease severity and risk of progressive kidney damage.
- Non-proliferative LN(I/II/Vnon-nephrotic)- conservative management, which include BP control with RAAS and immunomodulation with antimalarials (HCQS)
- Proliferative LN (III/IV OR III/IV+V OR V with nephrotic range proteinuria)- high dose corticosteroids and systemic immunosuppression along with RAAS /HCQS
- Induction phase- 3 to 6months, maintenance phase-several years.
Role of antimalarials in LN
- Immunomodulators act on innate immune system through TLR and reduces production of downstream pro-inflammatory cytokines.
- Anti-inflammatory, antithrombotic and safe in pregnancy.
- In LN it has shown to improve 12months renal response rate, reduce flare risk and delay progression to kidney failure.
- Can lead to pigment changes in macula, if used in higher doses (cumulative dose-1000g), base line eye examination and once a year thereafter.
Prognosis
- The prognosis of LN has improved over years (1970 to 2000), but after 2000 the rate of RRT remains the same.
- In proliferative LN – death rate solely due to kidney is 5-25% within first 5 years of diagnosis.
- ESRD and RRT is required in 10-30% of cases.
CASE 2:
A 30-year old female came with initial c/o
- Multiple joint pain – 3month
- Generalized body swelling – 3month
- Fever on and off for same duration
On Examination
- Facial puffiness+
- Pallor++, no icterus, no cyanosis, no clubbing
- No lymphadenopathy, pedal edema+
- JVP-N, BP-110/70, P-96/min. regular fair in volume
- Swollen and puffy fingers
Lab Exam:
- Hb – 7.7g/dl, TLC-2770, N-449%L45%, PLT-67000
- Scr. – 1.01mg/dl
- BUN – 22mg/dl, Uric acid-8.2
- U/R/M – protein-1+ RBC- 20-22/HPF
- 24-hour urine protein estimation- 2700mg/day
| ANA | POSITIVE |
| RF | NEGATIVE |
| CRP | POSITIVE |
| SERUM C3 | LOW (37) |
ENA
Let us see Kasukawa diagnostic criteria for Mixed Connective Tissue Disorder(MCTD)
Common symptoms:
- Raynaud’s phenomenon
- Swollen fingers/hands
Presence of anti U1 RNP
Mixed findings:
SLE like
- Polyarthritis
- Pericarditis/Pleuritis
- Lymphadenopathy
- Facial erythema
- Leucopenia/Thrombocytopenia
Scleroderma like
- Sclerodactyly
- Pulmonary fibrosis
- Esophageal dysmotility
Polymyositis like
- Muscle weakness
- High CPK
- Myopathic EMG
In view of above tests findings, would you like to go for Renal Biopsy?
Diagnosis in this case:
Mixed Lesion
Membranous lupus nephritis +diffuse lupus nephritis
- ACTIVITY INDEX-9/24
- CHRONICITY INDEX-2/12
Treatment
- RAASI
- HCQS
- Iv pulse MP- oral prednisolone
- MMF
Non-LN kidney disease
- Lupus podocytopathy
- TMA (APLAs, aHUS, TTP)
- Acute interstitial nephritis
- Cryoglobunemic GN
- Acute tubular necrosis
Pregnancy and LN
- Patient with LN who want to have children should be counseled to wait until LN is quiescent for at least 6 months.
- Switched to a “pregnancy friendly” regimen at least 3 months before attempting conception. (MMF to Azathioprine)
- CNIs can be continued throughout pregnancy.
Treatment in Pregnancy Flare
- Occur in 20% of cases (pre-eclampsia or HELLP syndrome in 11%, fetal loss in 8.4% and pre-term baby in 30.8%)
- Challenging
- Renal biopsy (safely done up to 20 weeks)
- Therapeutic options are limited and include hydroxychloroquine, corticosteroids, azathioprine, CNIs, and IV immune globulin
- Hydroxychloroquine use has been shown to reduce the probability of having a small-for-gestational age baby by 85% and reduces the risk for congenital heart block by 50% in babies of mothers who are anti-Ro antibody positive.
CME INDIA Learning Points
- SLE patients may present with a variety of clinical manifestations, these can resemble many differential diagnoses.
- Suspicion of SLE needs to be considered when deducing a diagnosis for PUO.
- A hallmark of SLE is the presence of autoantibodies; immune complexes are potent pyrogens.
- Fever is well recognised in SLE. It may be intermittent, accompanying disease flares or present as a continuous low-grade daily fever
- The titre, as well as the specific patterns of autoantibody positivity correlate to clinical phenotype, and in the context of fever.
- It has been observed that patients with ribonucleoprotein (RNP) positivity are four times more likely to have fever as a manifestation of their disease.
- C-reactive protein levels can be a valuable diagnostic aid, as flares in SLE tend not to cause significant CRP elevation.
- Lupus nephritis is clinically evident in 50%-60% of patients with SLE.
- LN is histologically evident in most SLE patients, even those without clinical manifestations of renal disease.
- Evaluating renal function in SLE patients is important because early detection and treatment of renal involvement can significantly improve renal outcome.
- Laboratory tests for SLE disease activity include the following:
- Antibodies to double-stranded DNA (dsDNA)
- Complement (C3, C4, and CH50)
- ESR
- CRP
- Renal biopsy should be considered in any patient with SLE who has clinical or laboratory evidence of active nephritis, especially upon the first episode of nephritis.
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Superb Discussion
Nice presentation . Thought discussed.
Just like to comment that instead of SLICC criteria for SLE , the newer 2019 EULAR/ACR criteria has more sensitivity and specificity.
In the first case there is significant number of pus cell in urine which might give false urinary albumin level and few rbc may be due to UTI also . There is also possibility of drug induced Lupus as Pt was on ATT for 3 months which should be differentiated also.
In second case oedema of hands can be due to generalised swelling and anaemia in this particular case and there is no history of Raynauds. It may be taken as SLE alone instead of MCTD as it is not fulfilling most of the criteria of Kasukawa for diagnosis of MCTD. Other criteria of Diagnosis of MCTD is Alarcon – Secovia which is simpler.
Throughly discussed. Correction please
Sir regarding 1st case ..24 hour urinary protein is 1200mg/day..so sub nephrotic protein excretion is there..it is not false elevated urinary albumin.pateint is having all features even before ATT started,so chances of drug induced lupus is less..
Regarding 2nd case she is having features of raynaud”s phenomenon as well as sclerosed skin ..Thank you
Nice presentation . thoroughly discussed.
Just like to comment that instead of SLICC criteria for SLE , the newer 2019 EULAR/ACR criteria has more sensitivity and specificity.
In the first case there is significant number of pus cell in urine which might give false urinary albumin level and few rbc may be due to UTI also . There is also possibility of drug induced Lupus as Pt was on ATT for 3 months which should be differentiated also.
In second case oedema of hands can be due to generalised swelling and anaemia in this particular case and there is no history of Raynauds. It may be taken as SLE alone instead of MCTD as it is not fulfilling most of the criteria of Kasukawa for diagnosis of MCTD. Other criteria of Diagnosis of MCTD is Alarcon – Secovia which is simpler.