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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
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
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
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
42. Diagnosis
SLE with Lupus Nephritis Class II
with Secondary Sjogren’s Syndrome
with Type 1 Renal Tubular Acidosis
with Bilateral Medullary
Nephrocalcinosis
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
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
56. • Pericarditis, with or
without an effusion
• Verrucous(Libman-Sacks)
endocarditis
• Myocarditis
• Heart block in neonatal
lupus
• Atherosclerosis
Cardiac involvement and vascular
manifestations
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
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
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
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
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
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
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
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