- Mrs. Shogota Khatun, a 31-year-old housewife, presented with multiple joint pain for 1.5 years and weakness of limbs for 1 year. On examination, she had a butterfly rash and muscle weakness. - Investigations revealed positive autoantibodies, renal involvement, and secondary Sjogren's syndrome. A renal biopsy showed lupus nephritis class II. - She was diagnosed with systemic lupus erythematosus, lupus nephritis class II, secondary Sjogren's syndrome, and type 1 renal tubular acidosis. She was started on treatment including hydroxychloroquine and azathioprine.

1. Presented by:
BA-102160
Major Md Imran Jewel
Traineein Aerospace Medicine(InternalMedicine)
A 31 Years Old Female with
Multiple Joints Pain and
Weakness of Limbs

2. Particulars of Patient
Name: Mst Sharifa Khatun, W/O Snk MdRokunuzzaman
Age: 31 years
Sex: Female
Religion: Islam
Marital status: Married
Occupation: Housewife
Address: 112/3, Kachukhet,Dhaka
Place of admission: CMH Dhaka
Date of admission: 11.10.2023

3. 1. Multiple joints pain for one and a half years.
2. Weakness of both upper and lower limbs for last one
year.
Chief complaints

4. According to statement of the patient, she was reasonably
well about one and a half years before.
Then she developed pain in multiple joints of both upper
and lower limbs involving small joints of both hands,
wrists, elbows, ankles and knee joints which was insidious
in onset, symmetrical, mild to moderate in severity, present
through out day and night but more marked in the
morning with stiffness lasting for less than an hour,
improves with pain killers and some activities without any
joint swelling and deformity.
History of present illness

5. She also complained of progressive weakness of both
upper and lower limbs for one year which was insidious in
onset and causing her difficulty in standing from sitting
position and raising hands above heads for combing,
dressing or undressing.
She noticed gradual loss of hair, dryness of eyes and mouth
and reddish, raised rash on her cheek which is not itchy
but increases with exposure to sunlight for last few
months.
History of present illness

6. She has no history of fever, oral ulcer, muscle pain, change
of fingers color in exposure to cold, tightening of skin,
weight gain or loss, cold or heat intolerance, palpitation,
chest pain, body swelling, diurnal variation of weakness,
convulsion, unconciousness, cough and shortness of
breath.
Bowel and bladder habit is normal.
Normotensive and non-diabetic.
History of present illness

7. History of past illness:
UTI 2 month back.
No history of spontaneousabortion and significant disease.
Drug history:
Took pain killers occassionally.
Received a course of oral antibioticfor 7 days 2 month back for UTI.
History

8. Family history:
Married for 12 year and has a son and a daughter and lives
with her husband.
No other family members are suffering from similar
illness.
Menstrual history:
Menstrual Period: 4-5 days
Menstrual Cycle: 30 days, regular
Flow: Average
History

9. Personal history:
Non smoker and non alcoholic.
Socio-economic history:
She belongs to a lower middle class family, lives in small
apartment in Dhaka and has access to safe drinking water.
Immunization history:
Immunized in childhood as per EPI schedule, received 03
doses of covid-19 vaccine
History

10. Appearance: Ill looking
Body-built: Average
Nutritional Status: Normal
Behavior: Co-operative
Decubitus: On choice
Anemia: Absent
Jaundice: Absent
Cyanosis: Absent
Clubbing: Absent
General examination

11. Koilonychia: Absent
Leukonychia: Absent
Oedema: Absent
Dehydration: Absent
JVP: Not raised
Lymph node: Not palpable
Thyroid gland: Not enlarged
General examination

12. Pulse: 76b/min, regular
Blood Pressure: 120/80mm Hg
Temperature: 98.4 F
Respiratory Rate: 14/min
Skin condition: Butterfly Rash, sparing naso-labial fold. Any
other rash or skin changes were absent.
Hair Distribution: Alopecia
Bedside Urine Test: negative for protein and glucose in
dipstick urinary test
General examination

13. Musculoskeletal system:
• Gait: Myopathic
• Limbs Examination: No joint swelling, redness,
tenderness, deformity, muscle wasting, muscle
tenderness and restriction of joint movement
observed.
Systemic examination

14. Nervous system:
• Higher cerebral function: Normal
• Speech: Normal
• Cranial Nerves function: Intact
• Muscle bulk: Muscle wasting absent
• Muscle tone: Normal
Systemic examination

15. Nervous system:
• Muscle power:
• Jerks:
• Plantar response: Flexor
• Coordination: Normal
• Sensory function: Intact
Reflex Ankle Knee Supinator Biceps Triceps
Right N N N N N
Left N N N N N
Systemic examination

16. Respiratory system:
• Breath sound: Vesicular
Cardiovascular system:
• Heart rate: 76b/min, regular
• S1,S2 loud, audible in all area
• No added sound
Gastrointestinal system:
• Abdomen is normal in shape, umbilicus centrally placed,
soft, non-tender, no organomegaly, no ascites present
Systemic examination

17. • Mrs Shogota Khatun, 31 years old normotensive, non-
diabetic hailing from Katchukhet, Dhaka was admitted to
CMH Dhaka on 11/10/2023 with the complaints of
multiple joints pain for one and a half years and weakness
of both upper and lower limbs for one year.
Salient feature

18. • According to statement of the patient, about one and a
half years ago she developed pain in multiple joints of
both upper and lower limbs involving small joints of both
hands, wrists, elbows, ankles and knee joints which was
insidious in onset, symmetrical, mild to moderate in
severity, present through out day and night but more
marked in the morning with stiffness lasting for less than
an hour, improves with pain killers and some activities
without any joint swelling and deformity. She also
Salient feature

19. gradually developed progressive weakness of both upper
and lower limbs for one year and has difficulty in standing
from sitting position and raising hands above heads for
combing, dressing or undressing. She also has history of
malar rash , hair fall, dry eyes and dry mouth. She has no
history of joint swelling, Raynauds phenomenon, skin
tightening, muscle pain, fever, weight gain or loss.
Salient feature

20. • On examination, her vitals were within normal limit.
There was butterfly rash on the face and no other rash or
skin changes were observed in the body. Muscle power
was MRC grade 4/5 in all four limbs. All reflexes were
normal and planter was bilaterally flexor. No sensory
abnormality was found. All other systemic examination
revealed no abnormality.
Salient feature

21. • System Inc Lupus Erythematosus and polymyositis
overlap syndrome with Secondary Sjogrens Syndrome
• Dermatomyositis with Secondary Sjogrens Syndrome
• Mixed Connective Tissue Disease
Provisional diagnosis
Differential diagnosis

22. POINTS IN FAVOR POINTS AGAINST
Joint Pain
None
Butterfly rash on face
Alopecia
Dry eyes and dry mouth
Proximal myopathy
Systemic Lupus Erythematosus and polymyositis
overlap syndrome with Secondary Sjorens
Syndrome

23. POINTS IN FAVOR POINTS AGAINST
Proximal myopathy
Butterfly rash sparing
nasolabial fold and absence
of any other rash in the
body
Joint pain
Dry Eyes and Dry mouth
Dermatomyositis with Secondary Sjogrens
Syndrome

24. POINTS IN FAVOR POINTS AGAINST
Joint pain
Absence of Raynaud’s
Phenomenon, Sclerodactyly
Proximal Myopathy
Dry Eyes and Dry mouth
Butterfly rash on face
Mixed Connective Tissue Disease

25. CBC with ESR
CRP- Negative
Date Hb TLC Platelet ESR
23/10/23 12.5 6.30 195 20
12/10/23 12.9 5.00 161 18
Investigations

26. Investigations
Investigations Results
Liver function test
S. Bilirubin 0.3 mg/dl
ALT 12 U/L
ALP 56 U/L
S. Creatinine 0.8 mg/dl
Fasting Blood Glucose 4.8 mmol/L
2 hrs AFB 6.7 mmol/L
S. Lipid Profile Normal
S. Uric Acid Normal
Urine R/E Normal

27. S. Electrolytes:
Investigations
Tests Reports Reference range
08/10/23 14/10/23
Serum
Sodium
(Na)
137 140 136-148
(mmol/L)
Serum
Potassium
(K)
3.1 2.9 3.6-5.2
(mmol/L)
Serum
Chloride (Cl )
107 110 95-105
(mmol/L)
Serum
Calcium(Ca)
8.5 8.1-10.5 mg/dl
Serum
Phosphate
2.9 2.7-4.5 mg/dl

28. • 24 hrs Urinary Electrolytes:
Result Ref
Na 58 mmol/l 20-110mmol/l
K 22 mmol/l 12-62 mmol/L
Cl 63 mmol/l 55-125mmol/l
• Urinary Electrolytes(Spot Sample) :
Investigations
Urine Volume 3000ml Result Ref
Na 153 mmol/l 40-220mg/L
K 57 mmol/l 25-125mmol/L
Cl 159 mmol/l 110-250 mmol/l
Ca 63mg 80-300mg

29. • Urine PH- 6.0
08/10/23 23/1023
Urinary Protein 317.1.4 mg/L 172,9 mg/L
Urinary Creatinine 3.1 mmol/L 3.1 mmol/L
Protein Creatinine Ratio 102.4:1 55.7:1
• Protein Creatinine Ratio (PCR) :
Investigations
• 24 Hours Urinary Protein- 155 mg
(Ref value:< 140mg)

30. Suggestive of Normal anion Gap Metabolic Acidosis
• Arterial Blood Gas Analysis :
Investigations
Test Report Reference range
PH 7.34 7.35-7.45
HCO3 ( mmol/L) 15.6 22-28 mmol/L
PCO2 (mmHg) 27 35-45 mmHg
Anion Gap 15 8-16 mEq/L

31. • Acid Load Test :
Suggestive of RTA type 1
Investigations

32. Antibody Value Interpretation
RA test 24 IU/ml > 15 IU/ml
Positive
Anti CCP Antibody 2.3 U/ml <5 U/ml
Negative
Anti Nuclear Antibody 4.6 IU/ml >1.5 IU/ml
Positive
Anti ds-DNA 240 IU/ml >25 IU/ml
Positive
Anti Smith Antibody 1.2 U/ml <15 U/ml Normal
Investigations
• Antibody Profile

33. Antibody Value Interpretation
Anti Phospholipid Antibody 1.6 U/ml <12U/ml
Negative
Anti RNP Antibody 1.1 U/ml <15 U/ml Normal
Anti SS-A 1.4 U/ml <15 U/ml Normal
Anti SS-B 1.3 U/ml <15 U/ml Normal
Anti Jo-1 antibody 0.7 U/ml <15 U/ml Normal
Investigations

34. Investigations
Complement value ref
C3 1.3 g/L 0.9-1.8 g/L
C4 0.3 g/L 0.1-0.4 g/L
Enzyme value ref
S.CPK 54 U/L 24-190 U/L
S. Aldolase 3.4 U/L Upto 7.6 U/L
• Complement
• Enzyme

35. • S. Cortisol : Normal
• S. Aldosterone: Normal
• S.TSH, FT4, FT3: Normal
• S.PTH: Normal
Investigations

36. USG of Whole Abdomen:
Bilateral Medullary Nephrocalcinosis
Investigations

37. Plain X Ray Abdomen AP view Erect Posture:
Bilateral Medullary Nephrocalcinosis
Investigations

38. • ECG: Normal Finding
Investigations

39. • Chest xray : Normal
Investigations

40. • Schirmer’s test : Positive
Investigations

41. Investigation
Renal Biopsy and Histopathology-
Mesangial Proliferative Lupus Nephritis
ISN Class II

42. Diagnosis
SLE with Lupus Nephritis Class II
with Secondary Sjogren’s Syndrome
with Type 1 Renal Tubular Acidosis
with Bilateral Medullary
Nephrocalcinosis

43. • Patient education, counseling and reassurance
• Avoid Sun and ultraviolet light exposure
Treatment
DRUG DOSE DURATION
Tab HCQ (200) 0+0+1.5 Cont
TAB NaHCO3 (665) 1+0+1 Cont
Tab Azathioprine(50) 0+0+1 Cont
Tab Pilocarpine(5) 1/2+1/2 +1/2 Cont
Tab. Ramipril (2.5) 1+0+0 Cont
Tab Omeprazole(20) 1+0+1 1 month

44. Treatment
DRUG DOSE DURATION
Tab. Vit C 250 2+0+2 1 Month
Tab. Vit E 200 1+0+1 1 Month
Tab. Fexofenadine120 0+0+1 1 Month
Cream Mometasone Apply locally Once
Daily
21 days
Sunscreen Cream SPF 50
+
Apply Locallyfrom
0730 hrs to 1630 hrs
regularly
Cont
E/D Carboxymethyl
cellulose + Glycerine
1 drop B/E twice daily Cont
Hypromellose eye gel 1 drop B/E at night Cont

45. Discussion on SLE

46. Definition
Chronic, multisystem
inflammatory disease
characterized by
autoantibodies directed
against self-antigens, immune
complex formation, and
immune dysregulation
resulting in damage to
essentially any organ.

47. Epidemiology
• Prevalence ranges from about 0.03% in people of
European descent to 0.2% in people of African Caribbean
origin.
• Female to male ratio: 9:1
• Peak age at onset lies between 20 to 30 years

48. • Multiple factors are associated include :
• Genetic
• Environmental factors
Aetiology

49. Genetic factors:
• Strongest region of association lies
with alleles in the HLA-DR and DQ
regions.
• The overall heritability of SLE has been
estimated to be about 40%–60%.
• SLE is associated with inherited
mutations in complement components
C1q, C2 and C4, in the
immunoglobulin receptor FcγRIIIb and
in the DNA exonuclease
Aetiology

50. Environmental:
– Ultraviolet light
– Viruses
– Drugs.
– Silica dust.
– Cigarette smoking
Aetiology

51. • The characteristic feature of SLE is autoantibody production
which are directed against antigens present within the cell or
within the nucleus.
• Defects in apoptosis or in the clearance of apoptotic cells,
causes inappropriate exposure of intracellular antigens on
the cell surface, leading to polyclonal B- and T-cell
activation, and autoantibody production.
Pathophysiology

52. • Joints 90%
• Skin
-Rashes 70%
-Discoid lesions 30%
-Alopecia 40%
• Pleuropericardium 60%
• Kidney 50%
• Raynaud’s 20%
• Mucous membranes 15%
• CNS (psychosis/convulsions) 15%
Organ Involvement in SLE

53. • Fatigue
• Fever
• Myalgia
• Weight change
Constitutional Symptoms

54. • Arthralgia often with morning stiffness
• Commonly polyarticular
• Symmetrical
• Usually non-erosive
MSK Symptoms

55. • Malar rash
• Discoid Rash
• Diffuse Alopecia
• Urticaria Eruptions
• Livedo Reticularis
Mucocutaneious

56. • Pericarditis, with or
without an effusion
• Verrucous(Libman-Sacks)
endocarditis
• Myocarditis
• Heart block in neonatal
lupus
• Atherosclerosis
Cardiac involvement and vascular
manifestations

57. • Raynaud phenomenon
• Vasculitis
• Thromboembolic disease
Vascular Phenomenon

58. •Lupus nephritis
•Tubulointerstitial disease
•Vascular disease including thrombotic nephropathy
Kidney Involvement

59. • Oral Ulcer
• Esophagitis
• Protein losing enteropathy
• Lupus hepatitis
• Acute pancreatitis
• Mesenteric vasculitis
• Peritonitis
GIT involvement

60. • Pleuritis (with or without effusion)
• Pneumonitis
• Interstitial lung disease
• Pulmonary hypertension
• Alveolar hemorrhage
Pulmonary Involvement

61. • Stroke
• Seizures
• Cognitive dysfunction
• Delirium, psychosis
• Peripheral neuropathies
Neurologic and Neuropsychiatric
involvement

62. • Anemia
• Leukopenia
• Thrombocytopenia
• Lymph node enlargement
Hematological Involvement

63. 1st Line investigations
• CBC with ESR
• ANA
• Anti ds DNA
• Urine R/E
• S. creatinine,S. Urea
• LFT
• Complement C3, C4
2nd Line investigations
• S. electrolytes
• Chest X-ray P/A view
• C reactive protein
• MRI of brain
• Renal biopsy
• 24 hours UTP,PCR,ACR
• Direct, indirect Coombs test
• Anti phospholipid antibody
Investigation

65. 2019 ACR
criteria of
SLE

66. General Considerations:
• Patient education , Counseling , Reassurance
• Taking nutritious diet
• Regular exercise and lifestyle modification
• Avoid sun and ultraviolet light exposure
• Control of hypertension, hyperlipidemia and obesity
• Stop Smoking and reduce excess alcohol intake
Treatment

67. Mild to Moderate Disease-
Mild Disease restricted to skin and joints:
1. Analgesics
2. NSAIDS
3. HydroxyChloroquine
4. Glucocorticoid
5. Methotrexate, Azathioprine or MMF
Treatment

68. Severe and Life Threatening Disease-
Renal, CNS and Cardiac Involvement:
1. High dose Glucocorticoid
2. Cyclophosphamide
Targeted therapy(biological) :
1. Rituximab
2. Belimumab
Treatment

69. Maintenance Therapy-
1. Oral Prednisolone, Azathioprine, MMF, Methotrexate
2. Life long Warfarine in SLE and antiphospholipid Syndrome
with Thrombosis history
Treatment

70. 5 yr after diagnosis 92% of patients are alive, the prognosis
falls to 82% survival at 10 yr, 76% at 15 yr and 68% at 20
yrs with treatment.
Bad prognostic factors-
• Renal disease
• Hypertension
• Male sex
• Low socioeconomic status
• Presence of antiphospholipid antibodies
• High overall disease activity
Prognosis

71. Discussion On Lupus Nephritis

72. Lupus Nephritis

73. Lupus Nephritis

74. Class Lupus
Nephritis
Light Microscopy Immunofluorescence
Study
I Minimal
mesangial
Normal Mesangial immune
deposits (IgG and C3,
C4)
II Mesangial
proliferative
Mesangial
hypercellualirity
Mesangial immune
deposits (IgG and C3,
C4)
III Focal Involve <50 % of all
glomeruli
Sub endothelial
immune deposits with
or without mesengial
alteration
Classification of Lupus Nephritis

75. Class Lupus
Nephritis
Light Microscopy Immunofluorescence
Study
IV Diffuse Involve >50 % of all
glomeruli, global or
segmental
Sub endothelial
immune deposits with
or without mesengial
alteration
V Membranous Global or Segmental
involvement of
>50% of all
glomeruli
Sub epithelial immune
deposits
VI Advanced
sclerosing
> 90% glomeruli
globallysclerosed
Sub epithelial immune
deposits
Classification of Lupus Nephritis

76. 1. Class I and II not require any treatment
2. Class III ,IV and V is treated with mycofenolate
mofetil or cyclophosphamide with high dose steroid
3. An alternative is tacrolimus, a calcineurin inhibitor
4. Class VI progresses to ESRD and requires renal
replacement therapy
Treatment of Lupus Nephritis

77. Discussion On Sjogren’s Syndrome

78. Chronic autoimmune disease
characterized by symptoms of
oral and ocular dryness,
exocrine dysfunction,
lymphocytic infiltration,
fibrosis and destruction of the
exocrine glands.
Sjogren’s Syndrome

79. Types Of Sjogrens Syndrome

80. Revised International
Classification Criteria
Four of these six criteria are required for positive case
classification of Sjögren’s syndrome
Diagnosis

81. 1. Ocular Symptoms ▪ Dry eyes for more than 3 months
▪ Sensation of foreign body in the eye
▪ Use of tear substitutes more than thrice daily
2. Oral symptoms ▪ Dry mouth for more than 3 months
▪ Recurrent or persistent swollen salivary glands
▪ Need liquids to aid swallowing
3. Ocular signs ▪ Schirmer’s test (<5mm in 5 mins)
▪ Rose Bengal Test
4. Histopathology ▪ In salivary gland biopsy, focal lymphocytic
sialadenitis with focus score >1 per 4 mm2
5. Salivary signs
positive result from
one of following
▪ Unstimulated whole salivary flow
▪ Parotid sialography showing diffuse sialectasis
▪ Salivary scintiography showing delayed uptake,
reduced concentration & delayed excretion of
tracer.
6. Autoantibodies ▪ Anti Ro (SSA)
▪ Anti La (SSB) or both

82. • Lymphoma most commonly ▪ MALTOMA
▪ DLBCL
Complication

83. Routine Investigations
– CBC
– LFT
– FBS, 2HABF
– CRP
– Urine R/E
– Serum Urea, Creatinine , Electrolytes
– X-RAY Chest P/A view
Investigation

84. Specific investigations
Antibody
• ANA
• RF
• Anti Ro ab/ Anti SSA ab
• Anti La ab/ Anti SSB ab
Sialography
Scintigraphy
USG of Salivary gland
Biopsy
Investigation

85. • Artificial Tears
• Softcontact
lenses
• Tinypunctal
plug
• Cyclosporine
▪ Oral rinses
and gels
▪ Mouth
washes
▪ Artificial
saliva and
water
sipping
▪ Pilocarpine
• NSAIDs
• MTX
• HCQ
• Rituximab
Treatment

86. ▪Renal tubular acidosis
(RTA) is a disease that
occurs when the kidneys
fail to excrete acids into
the urine, which causes a
person’s blood to remain
too acidic
▪It is characterized by
normal anion gap
metabolic acidosis
Renal Tubular Acidosis

87. Types of RTA

88. PATHOPHYSIOLOGY

89. NH4CL loading test : (confirmatory)
Investigation

90. ▪ Confusion or decreased alertness
▪ Muscle weakness
▪ Increased or irregular heart rate
▪ Muscle cramps or pain
▪ Abdominal pain
▪ Bone pain
Clinical Feature

91. )
▪Nephrocalcinosis, nephrolithiasis
▪Hypercalciuria
▪Hypokalemia
▪Hyperkalemia
▪Osteomalacia
Complication

92. ▪ Correction of acidemia with oral sodium bicarbonate.
▪ Hypokalemia ,urinary stones formation and
nephrocalcinosis can be treated with potassium citrate
tablets
Treatment

93. 1. SLE may present in isolation or may be associated
with other autoimmune disease and complication.
2. Early detection and treatment can give a favorable
outcome.
3. Regular follow up is needed to monitor the disease.
Take home message

94. THANK YOU