Syndrome of Sharp

The syndrome of Sharp or mixed connectivity is an association at a patient of the signs from at least two Connectivites (systemic diseases , auto-immune Maladie).

It was described for the first time in 1972 by Gordon Sharp and collaborators as an autonomous clinico-biological table, associating demonstrations belonging at the same time to the érythémateux Lupus disseminated, with the systemic Sclérodermie, the Polymyosite, and the Polyarthrite rhumatoïde. In this princeps description, it from the start is made state of antinuclear antibodies with mouchetée fluorescence, whose antigen was quickly identified with a Ribonucleoprotein.

Thereafter the distinction was made (in particular by the French school of internal medicine) between the authentic syndromes of Sharp, whose evolution is slow and benign, and other cases where the evolution is done in the gravity of traditional complications of one or the other of quoted connectivities. One admits now that these severe cases are rather forms of lupus, of polymyosite or scleroderma authentic whose signs of beginning could préter with confusion with Sharp.

As a specific entity the syndrome of Sharp is a rare affection which touches mainly the young women. As in all connectivities one can find antecedents family of other diseases of system or autoimmune.

Signs

There exist criteria of diagnosis defined by the team of Alarcon Segovia in a very important series.
the beginning is slowly progressive, with appearance of a Syndrome of Raynaud.
There exists often an aspect of " large fingers boudinés" enough evocative, as well as authentic a Syndrome of the carpel tunnel.
the capillaroscopy when it is practiced can give the exchange with a systemic Sclérodermie beginner.
There exist often polyarthraigies, even of true inflammatory arthritis, especially of the ends.
One can see myalgias, with evocative biological anomalies of a myosite.

Diagnosis

the absence of visceral complication (Digestive, renal, pulmonary, cardiac) is evocative authentic syndrome of Sharp.
There exists a great inflammatory, complete syndrome.
Especially, the existence of nuclear anti antibodies with fluorescence mouchetée with a title raised, of anti type RNP, is characteristic.

Treatment

It is all the more effective (relative with that of other connectivities) that the evolution is slow and benign.
It is based on the use of Aspirine, or Antiinflammatoires not stéroïdiens (after checking of the renal and digestive state).
the syndrome of Raynaud profits from anticalcic or prostaglandins vasodilatatrices.
a basic treatment by antipaludéens of synthesis (Nivaquine) is often recommended (in the absence of against indication).
the use of corticoids seldom essential, is even disadvised.

References

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