Short case -2
28 year old married woman, mother of two , from nalgonda ,who does maggam work on blouses ,
came for followup ,
patient was apparently asymptomatic till july 2021, when she first developed low grade fever ,which was present daily,increasing during evening time, for which she took tab dolo 650mg everday for one month, after which she started having joint pains(b/l knee, ankle, elbow, metacarpophalangeal and proximal interphalangeal joints) associated with swelling , with slight restriction of movement for one month.
later she developed oral ulcers,which were painful .
she also had increased hair fall,but no alopecia .
h/o rash over cheeks ? photosensitive
h/o pedal edema pitting type upto ankle.
Her first consultation with a physician was in September 2021 ,where she was adviced to get ana profile done, and was diagnosed with pancytopenia , and started on hcq 200 mg and prednisolone 10 mg twice per day , azathioprine 50 mg which she used for 2 months and discontinued as her joint pains and swelling subsided.
On Jan 1st , she had sudden onset weakness of left upperlimb , which resolved within 6 hours ,not a/w seizures,loss of consciousness.she was started on mycofenolate mofetil 1gm/day which was tapered to 500mg od within 2 weeks, (SLEDAI-14)with followup every two months.
since feb 2022 she has been skipping doses of mycofenolate ,due to financial issues and is taking it three times a week.
Personal history: She has a normal appetite ,consumes grains, Vegetables,meat, and has resumed her work in blouse designing.
menstrual history:
regular cycles 3-4 days /30, 3 pads per day, no clots, missed period for 2-3 months during fever episodes.
General examination:
built:thin
Skin:no hyperpigmentation currently.
pallor: absent
icterus:absent
cyanosis:absent
clubbing :absent
lymphadenopathy:absent
edema :absent
PR: 96/min ,regular
BP:110/70 mmhg
January 2022
Musculo skeletal examination:
Axial skeleton:
1)Cervical spine:normal
2)Thoracic spine:normal
3) Sacro-iliac joint:normal
Appendicular skeleton:
1)Shoulder joint: no swelling
No tenderness
Range of movements: Normal
2)elbow joint: no swelling
No tenderness
Range of movements: Normal
3)elbow joint: no swelling
No tenderness
Range of movements: Normal
4) wrist joint:normal
5) hand: metacarpophalangeal joint:normal
Interphalangeal joint :normal
6) knee joint:no swelling
No tenderness
Range of movements: Normal
7) ankle joint: no swelling
No tenderness
Range of movements: Normal
8) metatarsophalangeal joint:Normal.
Systemic examination
CNS: conscious
Oriented to time ,place,person
Speech: normal
Memory:intact
Intelligence:normal
Cranial nerves:normal
Sensory system:normal
Motor system:normal.
Cerebellum :normal
CVS: Apex beat in 5th ics ,mid clavicular line
S1 and S2 heard in all areas
R.S: bilateral airway entry present,
normal vesicular breath sounds in all areas
GIT:no oral ulcers currently
no tenderness,free fluid , organomegaly.
Investigations
5/8/21--------------------10/10/21--------may 2022
Hb-7.4 gm/dl. 8.9. 11.7
Tlc-3600 cells/cm3. 3400. 6600
platelets -1.9lakh/cm3. 1.84. 3.3lakh
esr-110mm/hr. 10
crp-9.0. negative
RF-negative
peripheral smear -normocytic ,normochromic.
12/12/2021
ana-positive
anti ds dna-strongly positive
anti sm-negative
c3-76.8 mg/dl (low )
c4-normal
As per SLICC criteria (6) in November 2022
clinical-leukopenia, thrombocytopenia,oral ulcers,synovitis
lab criteria-anti dsdna, low complement ,
current treatment
tab.mycophenolate mofetil 500mg od
Tab Prednisolone 20mg od
Tab hcq 200 mg od
Tab.Aspirin 75mg od
Final diagnosis: connective tissue disorder, likely systemic lupus erythematous in remission
(SLEDAI=0)
Criteria for remission
SLEDAI
Critical appraisal:
Enteric-coated mycophenolate sodium versus azathioprine in patients with active systemic lupus erythematosus: a randomised clinical trial
Ordi-Ros J, et al. Ann Rheum Dis 2017;0:1–8. doi:10.1136/annrheumdis-2016-210882
P-A total of 240 patients were enrolled between May 2010 and December 2013. Of the patients in this intention-to-treat population, 120 were randomised to each treatment group.
Eligible patients were aged ≥18 years, had an SLE according to the revised ACR classification criteria and moderate-to-severe active disease defined as: a SLE Disease Activity Index 2000 (SLEDAI-2K)26 total score ≥6 or at least 1 British Isles Lupus Assessment Group (BILAG) A or 2 BILAG B domain scores at screening.
exclusion criteria were immunosuppres-sant therapy 12 weeks before randomisation; active nephritis or non-lupus-related significant laboratory abnormalities.
I-Eligible patients were randomised (1:1) to receive EC-MPS (target dose: 1440 mg/day) or AZA (target dose: 2 mg/kg, per thiopurine methyltransferase levels (TPMT)) in addition to background oral prednisone and antimalarial agents.
O-The primary efficacy endpoints were the proportion of patients achieving at 3 and 24 months, at least 8 consecutive weeks of clinical remission (CR), defined as a clinical SLEDAI-2K=0.
Primary endpoint Clinical remission rates were higher in the EC-MPS group by month 3 (32.5% (39/120 patients)) compared with the AZA group (19.2% (23/120); percentage difference 13.3% (95% CI 2.3 to 24), p=0.034) and sustained throughout the study to month 24 (71.2% (84/118) vs 48.3% (57/118); percentage difference 22.9% (95% CI 10.4 to 34.4), p<0.001)
Secondary endpoints included: the overall proportion of patients in CR and partial clinical response (PR) (≥50% reduction in the total SLEDAI-2K score with a BILAG C score or better, without new BILAG A/B scores); treatment failure (premature discontinuation necessitated by protocol-prohibited rescue therapy due to worsening or persistent disease activity
BILAG A/B flares were more common in the AZA group (71.7% (86/120 patients)) compared with the EC-MPS group (50% (60/120)) (p<0.001).
In the AZA and EC-MPS groups, 34.2% and 35% patients had 1 disease flare; 21.7% and 13.3% had 2 flares; and 16.7% and 5% had >2 flares, respectively.
Mucocutaneous and renal flares were more frequent in the AZA group (p=0.003 and p=0.031, respectively)
Flares were associated with medication reduction in 38 patients (31.7%) of the AZA group and 29 (24.2%) of the EC-MPS group.
Rates of new BILAG A flares were low, but significantly higher in AZA (21.7% (26/120) vs 8.3% EC-MPS (10/120), p=0.004)
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