1. Dr.Babur Salim Student in Rheumatology deptt. FFH
Assistant Professor Rheumatology
FCPS(med), FCPS(Rheuma), FACR(USA),MSc(Pain Medicine)

2. TAKE HOME MESSAGE

3. Objectives Basic Concept of Over lap Syndrome
Mixed connective Tissue Disorder
Undifferentiated connective tissue disorder
Role of HCQ in SLE

4. Two full blown flavors----diseases in one patient
Over lap syndrome
Two full blown flavors----diseases in one patient

5. OVERLAP(LUPUS+RA=RUPUS)
30,years , F, has;
polyarthritis
oral ulcers
raynauds
photosensitivity
O/E:
swollen and tender PIPS, MCPS
swan neck deformities
Labs : RA factor +ve ACCP anti +ve , ANA and anti dsDNA +ve, X rays: periarticular erosions, periarticular osteopenia

6. Close your eyes and confirm the flavor …..sure?
MCTD
Close your eyes and confirm the flavor …..sure?

7. MCTD 35 years , F, presented with; raynauds phenomenon, polyarthritis
proximal muscle weakness
mild thickening of skin of hands
Labs: ANA +ve,
antiDsDNA negative, RF and ACCP negative, anti RNP Positive , CPK is 150(normal 50)

8. MCTD vs. Overlap Syndrome
Anti RNP antibodies have a protective role in autoimmune diseases.
MCTD patients do not get renal or cerebral disease(most of the times)
MCTD has better prognosis---may develop into only one disease over a period of years…

9. But don’t take MCTD as granted ……remember two important complications of MCTD
erosive arthritis
Pulmonary hypertension

10. Undifferentiated Connective Tissue Disorder
Patients presenting in one or few common symptoms without fulfilling any disease diagnostic criterion

11. Secondary raynauds -----no other symptom/sign or lab evidence
Asymmetrical polyarthritis with no other symptom/sign or lab evidence
Follow-up the pts as they will differentiate to any one disease over a period of months to year

12. Why to differentiate? All these three diseases are different;
Geneticallay
Serologically
Clinical presentation
Treatment wise
prognostically

13. Role of HCQ in SLE Sort of DMARD in Lupus ( prevents flares)
Anti-thrombotic
anti-atherosclerotic
in joint/skin
Safe in Pregnancy
Stop if intolerant /complication like maculopathy( %)-----annual monitoring recommended.

14. Thank you