Antisynthetase antibody syndrome

Disease has a huge impact on life. It not only brings physical pain, but also seriously hinders the rhythm of life and undermines the patient's physical health and hope for life. There are many types of diseases, among which various internal diseases have the greatest impact on the body, and some of them are rarely heard of. For example, what is antisynthetase antibody syndrome? Let’s take a look at the explanation below, I hope everyone can understand it.

Among the antibodies associated with polymyositis/dermatomyositis (PM/DM), there is a type of anti-synthetase antibody. Patients who are positive for this type of antibody have a group of special syndromes, namely myositis, interstitial lung disease, symmetrical polyarthritis, acute fever, mechanic's hands, and Raynaud's phenomenon, which is called anti-synthetase antibody syndrome (ASS). Such patients rarely have malignant tumors and respond well to treatment, but the disease is prone to relapse. So far, people have discovered 13 types of such antibodies, among which the most widely studied and best understood is the anti-Jo-1 antibody, which has the highest positive rate of about 20%. Therefore, ASS is often called anti-Jo-1 antibody syndrome.

Clinical manifestations

1. Myositis

The incidence rate is 95%. Some patients have mild myositis symptoms, and a few even have no myositis.

2. Interstitial lung disease

Most patients have interstitial lung disease, which is the most prominent manifestation of ASS. They may experience chest tightness, dry cough, and shortness of breath after activity. The disease progressively worsens and is often the first symptom of ASS. Auscultation of both lungs revealed crackles, mainly in the lower lungs. Pulmonary function tests showed restrictive ventilation dysfunction accompanied by diffusion dysfunction. Interstitial lung lesions are very likely to be complicated with opportunistic infections and rapidly develop into respiratory failure, which is the leading cause of death in ASS.

3. Fever

Most patients have fever at the onset of the disease, and the incidence of fever is higher when the disease worsens or when there is infection.

4. Technician's hands

The skin on the radial side and palmar surface of the fingers is highly keratinized, rough, cracked and pigmented, resembling the hands of a mechanic.

5. Symmetrical polyarthritis

It accounts for 70%, most commonly affecting the wrists, metacarpophalangeal joints and proximal interphalangeal joints, and is easily misdiagnosed as rheumatoid arthritis. A small number of patients may develop joint deformities, but X-rays do not show bone erosion.