moderator: DR. C R BHAT presentor: DR. TAMPHASANA. W

1. SYSTEMIC LUPUS
ERYTHROMATOSUS MANIFESTING
AS NEPHROTIC SYNDROME
BY DR.TAMPHASANA WAIROKPAM,
1Yr Resident
DEPT. OF INTERNAL MEDICINE,
KVGMCH, SULLIA

2. • Herewith I am presenting an interesting case-
22 yrs old female patient ,married from
Periyapattna, who was referred to us from
Dermatology dept for e/o edema & facial
puffiness .

3. • Her primary problem, which brought her to
dermat. dept was facial rash of 1 yr duration,
the rash used to aggravate & cause burning
pain on exposure to sun- suggestive of
photosensitivity- malar rash .
• However she did not had rashes over other
sun exposed area.

4. • She developed Facial puffiness & edema of legs
for 1 month, apparently more in the morning
hours, but there was no oliguria or haematuria.
• On further enquiry she had chronic mild
Intermittent ,on & off fever, headache, easy
fatiguability , weight loss ,anorexia, recurrent oral
ulcers, & alopecia.
• She had bilateral wrist and knee joint pains for
last 1 year.

5. • She did not had similar problems prior to 1yr.
• She was not on any regular medication & there was no
history of drug allergy .
• She was on mixed diet , she had decreased appetite &
decreased sleep.
• Her Bowel and bladder habits regular.
• She attained menarche at 13 yrs , cycles regular(4/30)
&there was no h/o menstrual irregularity.
• She is married & there was no h/o pregnancy loss
• No similar complaints in the family

6. To summarise
• Young married lady with – Fever, Arthralgia,
Malar rash, photosensitivity, & other
constitutional symptoms for ~ 1 yr , now
presenting with Edema & facial puffiness.

7. • D/D for Prolonged fever & constitutional symptoms
– Infections
• SBE
• TB
– CT Disease
• SLE
• RA
• MCTD
• Vasculitis
– Malignacy
• Lymphoma
• Chr Leukemia

8. • Edema & Facial puffiness on the background
of prolonged illness
– Renal
• Nephrotic syn
• Immune mediated AGN
• Renal failure
– Malabsorption
– Severe Anemia & hypoprotenemia of chr. Disease

9. • In this patient we considered CT disease SLE
– Arthralgia
– Typical rash
– Photosensitivity
– Oral ulcers
• Complicated by
– ? Nephrotic syndrome
– ? Renal failure

10. General physical examination
• General examination:
• Patient is conscious, cooperative, comfortably sitting on bed, well
oriented to time, place & person.
• Her BMI was 16.06
• Her temp 100`F,
• PR 98b/m, regular, normal volume, character , all peripheral pulses
felt.
• BP :170/100 mm Hg.
• RR 16/min.
• She was Pale & had Malar rash, Facial puffiness, periorbital edema
& b/l Pitting pedal edema .
• There was no icterus, cyanosis, clubbing, lymphadenopathy or
orogenital ulcers .
• Her Fundus showed grade 2 htn retinopathy.
• There was no skeletal or joint deformity or e/o active arthritis.

11. Systemic examination
• All systemic examinations were normal except
for a short systolic murmur in the mitral area.
• CVS: apex in the 5th ICS in the MCL ,S1,S2
heard, systolic murmur in the mitral area.
• P/A-soft ,no tenderness, no organomegaly,
BS+.
• CNS-NFND, plantars are b/l flexors.
• RS- Clear VBS b/l

12. SUMMARY
• 22 yrs old lady with
• Malar rash
• facial puffiness & B/L Leg edema
• Pallor
• Hypertension & Gr II retinopathy
• Systolic murmur at apex.

13. Investigations revealed :
• Anemia(hb- 5.1 gm%)
• Thrombocytopenia ( 70,000)
• Leucopenia (2600)
• ESR: 77 (raised)
• 24 hrs urinary protein: 5.1 gm
• Normal GFR
• Hypoalbuminemia( S alb-: 2 gm/dl)
• ANA : +
• Anti ds DNA : +
• Anti histones +
• USG ABD/Pelvis – mild hepatosplenomegaly, & b/l
mild pleural effusion.
• Other investigations were normal.

14. Diagnosis - SLE
• Young lady with Fever, Arthritis, Malar rash,
Photosensitivity, oral ulcer
• Anemia, Thrombocytopenia, leukopenia
• High ESR
• Pleural effusion
• + ANA & Ds DNA
• Nephrotic proteinuria

15. • Management:
• Inj. Methyl prednisolone 1g iv OD for 3 days.
• followed by oral prednisolone 40 mg/day in
divided doses.
• Diuretics, anti- hypertensives, hematinics &
blood transfusion.
• At discharge, oral prednisolone 40 mg in
divided doses was advised to be tappered
after 1 month over a peroid of 1 month.

16. • At discharge: Pt is comfortable, stable, edema &
facial puffines had reduced markedly.
• P.R- 82 bpm.
• B.P- 150/90mm hg
– Hb- 7.2 gm%
– TLC- 4,400
– DLC-N75,L 18, M 7
– PLT – 92,000.
In view of development of renal involvement(NS) , pt
was referred to higher centre for renal biopsy and
further management with immunosuppressive drugs.

17. Review on SLE
• Systemic lupus erythematosus (SLE) is an
autoimmune disease in which organs and cells
undergo damage mediated by tissue-binding
autoantibodies and immune complexes.
• Ninety percent of patients are women of
child-bearing years.
• Prevalence of SLE is 15–50 per 100,000.

18. Causes
DEFINITE FACTOR : Ultraviolet B light
PROBABLE FACTOR : Sex hormones; F:M ratio is 9:1 ;
3:1 in young and old.
POSIBLE FACTOR :
1)Dietary -high intake of saturated fats
2)Infectious agent -Bilateral DNA human retroviruses,
endotoxins, bacterial toxins.
3)Medication –Hydralazine, Procainamide, INH,
Hydantoins, Chlorpromazine, Methyldopa,
d-penicillamine, minocycline, TNFα-antibodies.

19. Triggering agents
Abnormal immune regulation
T cells
Excessive cytokine release
B cells
Autoantibody formation
Immune complexes build up in the tissues and can cause
inflammation, injury to tissues, and pain.

20. Criteria for diagnosis of lupus
A person shall be said to have SLE if any 4 or more of the 11
criteria are present, serially or simultaneously, during any
interval of observation
S.N Criteria Definition
Fixed erythema, flat or raised, over the malar
1 Malar Rash
eminences, tending to spare the nasolabial folds
Erythematous raised patches with adherent
2 Discoid Rash keratotic scaling and follicular plugging; atrophic
scarring may occur in older lesions.
Skin rash as a result of unusual reaction of
3 Photosensitivity
sunlight.
Oral or Nasopharyngeal ulceration, usually
4 Oral Ulcers
painless.
Nonerosive arthritis involving 2 or more peripheral
5 Arthritis joints, characterized by tenderness, swelling, or
effusion.

21. S.N. Criteria Definition
a) Pleuritis –convincing history of pleuritic pain or
rubbing heard by a physician or evidence of pleural
effision.
6 Serositis
OR
b) Pericaditis-documented by ECG or rub or evidence
of pericardial effusion.
a) Persistent proteinuria greater than 0.5 gms. per day
or greater than 3+ if quantitation not performed.
Renal
7 OR
Disorder
b) Cellular casts- may be red cell, hemoglobin,
granular, tubular, or mixed.
a) Seizures –in the absence of offending drugs or
known metabolic derangements; e.g.-uremia,
Neurologic ketoacidosis, or electrolyte imbalance.
8
Disorder b) Psychosis –in the absence of offending drugs or
known metabolic derangements; e.g.-uremia,
ketoacidosis, or electrolyte imbalance.

22. a) Hemolytic Anemia –with reticulocytosis
OR
b) Leukopenia –less than 4,000/mm
Hematolo OR
9 gic
Disorder c) Lymphopenia –less than1,500/mm
OR
d) Thrombocytopenia –less than 100,000/mm
in the absence of offending drugs
Immunolo Anti- dsDNA, Anti-Sm and/ or anti phospholipid
10 gic
Disorder
Antinuclea Abnormal titre of ANA at any point in time in the
11 r absence of drugs known to induce ANAs.
antibodies

23. Lupus nephritis
• Proteinuria
• Nephrotic syndrome
• Granular casts
• Red cell casts
• Hematuria
• Decreased renal function
• HTN
• ARF
• Accelerated atherosclerosis, hyperlipidemia,
hyperglycemia.

26. Management
1) Musculoskeletal
? NSAIDs
Hydroxychloroquine + steroid -5-10mg/day
Methotrexate 10-20mg/week
Persistent pain in one joint only- ischaemic necrosis of bone
2) Cutaneous Lupus
Protective clothing
Sunscreen lotion
Local glucocorticoids
Hydroxychloroquin 400mg/day
Quinacrine 100mg/day
Etiretinate 1mg/kg/day in divided doses
Dapsone, Thalidomide
Cytotoxic drugs

27. 3) Serositis-
• NSAIDs + Steroids 10-15mg/day
4) Aggressive Therapy
Manifestation usually responsive to high dose glucocorticoids
 Vasculitis
 Severe dermatitis of SCLE or SLE
 Polyarthritis
 Polyserositis – Pericarditis, pleurisy, peritonitis
 Myocarditis
 Lupus pneumonitis
 Glomerulonephritis – proliferative forms
 Hemolytic anemia
 Thrombocytopenia
 Diffuse CNS syndrome – acute confusional state, demyelinating
syndromes, intractable headache, serious cognitive defects
Myelopathies .
Peripheral neuropathies
Lupus crisis – high fever and prostration

28. Cytotoxic Drugs
Initial Maint.
Drug Advantage Adverse side effect Inci.
Dose Dose
Azathiopri-- 1-3 1-2 Probably Bone marrow <5
ne mg/kg/ mg/kg/ reduces suppression,
Day Day flares, Leukopenia 15
requires reduces renal Infection (herps 10
6-12 scarring, zoster),
months reduces Malignancies, <5
to work glucocorticoid Infertility, 15
Well dose Early menopause 10
Requirement Hepatic damage <5
Nausea 15
Cyclophop- 1-3 0.5 As for Bone marrow <5
-hamide mg/kg/ mg/kg/ azathioprine suppression
day Day probably
Requires orally or Orally or effective in
2-16 8-20mg/ 8-20mg/kg higher
weeks to kg IV IV Every proportion of
work well once a 4-12 Wk + patient
month Mesna
+ mesna

29. Combination Therapy
Azathio- 1.5-2.5 1-2 Possibly more Infections 40
-prine mg/kg/ mg/kg/ effective than
day day one drug
orally Orally
plus Cystitis 15
Cyclophpspo- 1.5-2.5 1-2
mide mg/kg/ mg/kg/
day day
orally orally

30. Prognosis of SLE
• Survival in patients with SLE in the United States, Canada,
Europe, and China is approximately 95% at 5 years, 90% at
10 years, and 78% at 20 years.
• In the United States, African Americans and Hispanic
Americans have a worse prognosis than Caucasians,
whereas Africans in Africa and Hispanic Americans with a
Puerto Rican origin do not.
• Poor prognosis (~50% mortality in 10 years) in most series
is associated with (at the time of diagnosis)
– high serum creatinine levels [>124 mol/L (>1.4 mg/dL)
hypertension
– nephrotic syndrome (24-h urine protein excretion >2.6 g)
– anemia [hemoglobin <124 g/L (<12.4 g/dL)]
– hypoalbuminemia
– hypocomplementemia
– aPL

31. • This case is presented to showcase most of
the common clinical manifestation of a rare
disease.

32. THANK YOU