Sharpe’s syndrome is a systemic polysyndromic disease that combines individual manifestations of systemic lupus erythematosus, dermatomyositis, and scleroderma. Pathology occurs with joint, muscle, skin, visceral symptoms, Raynaud’s and Sjogren’s syndromes. The diagnosis can be established if autoantibodies to ribonucleoprotein, an immunological marker of the disease, are present in the blood of patients. The treatment lends itself well to glucocorticosteroid therapy. The disease is relatively benign and usually does not lead to severe destructive lesions.
Meaning
Sharpe syndrome mainly affects women. The determining factors in the development of Sharpe syndrome can be considered the interaction of hereditary predisposition, endocrine disorders, provoking environmental agents (viral infection, stress, trauma, hypothermia, etc.).
Violation of immune homeostasis in Sharpe syndrome is expressed by an imbalance of immunoregulatory functions of T-lymphocytes, an extremely high titer of autoantibodies to ribonucleoprotein (RNP), circulation of immune complexes and further development of systemic inflammation.
Symptoms
The Sharpe syndrome clinic is characterized by a combination of moderately pronounced signs of Raynaud’s syndrome, polymyositis, polyarthritis, skin and visceral lesions. In 85% of patients, the early phase of the disease is accompanied by Raynaud’s syndrome, occurring with dense swelling of the hands, however, without severe ischemic and necrotic changes in the distal phalanges of the fingers and the development of sclerodactyly.
Articular manifestations in the form of recurrent multiple arthritis and arthralgia in Sharpe syndrome in a third of patients cause erosive bone changes. The formation of rheumatoid nodules and the detection of rheumatoid factor are noted. Polymyositis in Sharpe syndrome is characterized by pain, muscle asthenia of the proximal extremities, muscle seals and responds well to therapy with glucocorticosteroids.
Visceral symptoms occur with dysphagia caused by hypotension of the esophagus and pulmonary hypertension. In connection with the progression of pulmonary hypertension, attention is drawn to the appearance of shortness of breath at rest and its intensification with minimal exertion; unproductive paroxysmal cough, thoracalgia. Skin manifestations in Sharpe syndrome include scleroderm-like changes, erythematous spots, telangiectasia, alopecia, discoid lupus, periorbital pigmentation. In some cases, Sharpe syndrome develops kidney damage (diffuse glomerulonephritis), neurological syndromes (aseptic meningitis, trigeminal neuralgia), anemia, lymphadenopathy, moderate hepatomegaly and splenomegaly.
Complications
The aggravated course of Sharpe’s syndrome may be associated with severe progressive visceral lesions and their complications: esophagitis, esophageal strictures, myocardial infarction, renal failure, stroke, perforation of the colon.
Diagnostics
Diagnosis of Sharpe syndrome is based on serological and clinical signs. A reliable serological marker of Sharpe syndrome is the receipt of a positive antiribonucleoprotein reaction with a hemaglutination titer of 1:600 and higher. Nonspecific biochemical signs are an increase in globulins (a2 and γ), fibrin, CK, AST, seromucoid, aldolase, sialic acids, CRP.
Objective clinical symptoms include swelling of the hands, myositis, synovitis, acrosclerosis, Raynaud’s syndrome. The presence of a serological marker and three clinical symptoms is required for the undoubted diagnosis of Sharpe syndrome. If the patient has three dominant sclerodermic signs (Raynaud’s syndrome, swelling of the hands and acrosclerosis), the diagnosis is confirmed by the presence of myositis or synovitis.
Treatment and prognosis
To choose a therapeutic tactic for Sharpe syndrome, a rheumatologist’s consultation is necessary. Depending on the nature of the lesions of the internal organs, additional consultation with a cardiologist, neurologist or gastroenterologist may be required. Recurrent polyarthritis and pleurisy in Sharpe syndrome requires the appointment of NSAIDs, antimalarial drugs, low-dose courses of prednisone, sometimes methotrexate.
Inflammation in polymyositis is stopped with prednisone, less often with methotrexate and azathioprine. When Raynaud’s syndrome dominates, patients should beware of provoking agents (freezing, vibration, inhalation of cigarette smoke). For therapeutic purposes, vasodilators of the Ca antagonist group (nifedipine) are used.
Treatment of esophageal disorders in Sharpe syndrome consists in the appointment of antacid drugs and diet. With myocarditis and early signs of pulmonary hypertension, therapy with corticosteroids and cytostatics (cyclophosphamide) is indicated. The progressive course of hypertension of the small circle requires the introduction of prostacycline, the appointment of ACE inhibitors and calcium antagonists.
Usually, the course of Sharpe’s syndrome is conditionally favorable. Severe lesions of the lungs and kidneys, leading to a fatal outcome, rarely develop. Prevention of the syndrome has not been developed.