Clinical manifestations of anti-synthetase syndrome positive for anti-alanyl-tRNA synthetase (anti-PL12) antibodies: a retrospective study of 17 cases

Abstract

The severity of anti-PL12 ASS varied because of the constant pulmonary involvement. ILD was the predominant prognosis factor, which was notable in cases associated with pulmonary hypertension.

An official European Respiratory Society/American Thoracic Society research statement: interstitial pneumonia with autoimmune features

“…Raynaud's phenomenon, palmar telangiectasia, distal digital tip ulceration and digital oedema are specific physical findings that are often seen in systemic sclerosis [23,24] but rarely seen in IIP. Similarly, the features of digital fissuring ("mechanic hands") ( figure 1) and a fixed rash on the digital extensor surfaces (Gottron's sign) (figure 2) are hallmarks of the anti-synthetase syndrome or systemic sclerosis-myositis overlap associated with PM-Scl antibody positivity [16,[25][26][27][28][29][30][31][32]. The use of nailfold microscopy in the evaluation of individuals with Raynaud's phenomenon is encouraged as capillary loop abnormalities can be predictive of developing a CTD such as systemic sclerosis or dermatomyositis [33][34][35][36].…”

The Antisynthetase Syndrome

“…In one study (20), CK levels increased to up to twice the normal level in only two individuals. Interestingly, two of these cases were diagnosed with PAH.…”

“…Among PL-12 positive ASS, the histological features of ILD is predominantly of the NSIP pattern (20), while myositis is mostly mild (34). In one study (20), CK levels increased to up to twice the normal level in only two individuals.…”

Polymyositis/Dermatomiositis: Differential Diagnosis

“…Наличие ИПЛ определяет прогноз болезни [28,29]. Примечательна фенотипическая вариабельность и внутри АСС [30,31]. Клиническая картина пациентов, позитивных по anti-PL-12 и аnti-PL-7 антителам, в боль-шей степени ассоциирована с тяжелым ИПЛ и высоким риском развития легочной гипертензии, чем с миозитом [32][33][34].…”