Systemic lupus erythematosus (SLE)

এটা একটা chronic autoimmune disease.
SLE এর ব্যাপারে একটা কথা প্রচলিত আছে :

Lupus can do anything but not everything is lupus.🤔

আমরা বুঝতে পারতেছি body তে এমন কোন system নাই যা Lupus attack করতে পারে না।

Diagnostic criteria 4/11 feature আগের মতোই আছে।
যদি ও EULAR/ACR মিলে research purpose এ SLE কে নতুন ভাবে classify করছে।

Patient presentation :🌿
অনেক কিছু নিয়ে(any system) present করতে পারে কিন্তু যেই symptoms সাধারণত থাকেইঃ
Fatiguability (80-100%)
Arthralgia(90%)
Fever 50%
Raynauds phenomenon upto 50%
Skin lesion(20-50)%
Renal involvement (40-70)%

Pathogenesis :
Intracellular অরগানেল্লে(Neucleic acid) এর Against এ antibody formation হয়। eg:(ANA)
Autoimmune process. Etiology unknown
F:M ratio- 9:1

Investigation :
Autoantibodies SLE clinical feature develop করার অনেক বছর আগেই blood এ পাওয়া যেতে পারে।

ANA Laboratory hallmark of disease.
ANA (-) is unlikely to have/develop SLE 🙂
Anti ds-DNA is highly specific for SLE

Management :

Mild SLE: (constitutional symptoms /mild arthritis/rash <9%BSA /platelet 50-10000/SLEDAI<6)

Rx:

For mucocutaneous lesion:

-Use sunscreen SPF 50
-Topical tacrolimus.0.1% cream BD for 2-3 weeks
If not controlled :
Low potency steroid(hydrocortisone 1%/desonide 0.05%/betamethason)-14 days not controlled gap 14days then again use 14 days

For joint pain/headache /fever:
NSAID
Hydroxychloroquine(HCQ) <5mg/kg/day maximum upto 400mg/day
If no improvement after 8week of HCQ add prednisolone 5- 20mg for 2 weeks

Moderate SLE:(RA llike arthritis/rash 9-18% of BSA/platelet 20-50 thousands /serositis/SLEDAI(7-12)

Rx:
Induction of remission:
0.5mg/kg/day 2 week then gradually taper over 6months
HCQ-as mild case
Steroid sparing agent: MTX/Azathioprin

Severe SLE:(Nephritis /myelitis/myositis/cerebratis/m.vasculitis/platelet<20000/eash>18%bsa)

Rx:
HCQ-as mild case
Prednisolone:. 5-1mg/kg/day for 2-4weeks gradually taper to 7.5mg @ 12-24 weeks taper slowly over 8 month
Life threating হলে IV Methyl prednisolone pulse therapy 1g/day for 3 day
Cyclophosphamide: 500mg @15 days interval for 6 pulses

Renal lupus and CNS lupus (stage and stepwise) এর management এখানে আলোচনা করা হয়নি।

©️ Digest medicine ☘️

Ref:
1.Davidson principle 24th
2.Rheumatology secrets
3.Oxford handbook of Rheumatology
4.Rheumatology by Marc c. Hochberg
5.EULAR guidelines