SHARP syndrome

Unknown prevalence, except in Japan where it is estimated at 1/37 000 inhabitants. The female/male ratio is about 10:1. Rare connectivitis where clinical signs of systemic lupus erythematosus, scleroderma, polymyositis and/or rheumatoid arthritis are entangled. The etiology is unknown, but abnormal responses of lymphocytes B and T to cryptic autoantigens have been highlighted.

Clinical picture:

-        onset most often between 15 and 35 years; moderate fever

-        Raynaud's phenomenon

-        arthralgias or polyarthritis, podgy fingers, sclerodactyly and/or moderate muscle weakness.

-        sometimes, disorders of esophageal motility, skin rash,  trigeminal neuralgia, pleuritis, pericarditis and/or signs of pulmonary hypertension.

A high level of anti-U1-RNP antibodies (> 1/1000) is almost always present, without the autoantibodies linked to other connectivites (with the exception of anti-SSA and/or anti-SSB antibodies in case of Sjögren's syndrome).

Most minor forms respond to treatment with NSAIDs, hydroxychloroquine, or low doses of corticosteroids. Moderate to severe forms usually require medium to high doses of corticoids  (approximately 0.5 to 1 mg/kg/d), associated, in case of severe intestinal involvement, to another immunosuppressive therapy.


Anesthetic implications:

check renal function; avoid hypothermia in presence of Raynaud phenomenon


References :


Updated: February 2017