Dr.Babur Salim Student in Rheumatology deptt. FFH

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Presentation transcript:

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

TAKE HOME MESSAGE

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

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

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

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

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)

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…

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

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

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

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

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( 0.1-0.5%)-----annual monitoring recommended.

Thank you