Anti-Ha anti-synthetase syndrome presenting as rapidly progressive interstitial lung disease: a case of high confidence autoantibody testing

Alina Giovanna Liedtke^1*Thomas Daikeler²Stefan Gherca³Matthias Josef Herrmann¹Spasenija Savic Prince⁴Sarah Louise Tansley⁵Ingmar A. F. M. Heijnen⁶Katrin Esther Hostettler^1*

• ¹Clinic of Respiratory Medicine, University Hospital Basel, Basel, Switzerland
• ²Department of Rheumatology, University Hospital of Basel, Basel, Switzerland
• ³Clinic of Radiology and Nuclear Medicine, University Hospital of Basel, Basel, Switzerland
• ⁴Pathology, Institute of Medical Genetics and Pathology, University Hospital Basel, Basel, Switzerland
• ⁵University of Bath Department of Life Sciences, Bath, United Kingdom
• ⁶Division Medical Immunology, Laboratory Medicine, University Hospital Basel,, Basel, Switzerland

We report on a 38-year-old patient who presented with rapidly progressive interstitial lung disease (ILD), without any signs of muscular involvement. Antinuclear antibody testing by indirect immunofluorescence revealed a nuclear titer of 1:320 with a fine speckled and a cytoplasmic titer of 1:1'280 with a fine speckled pattern. Subsequent myositis-specific and myositis-associated antibody tests with commercial multiplex dot-immunoassays showed a strong positive result for anti-Ha antibodies, also confirmed by protein immunoprecipitation, establishing the diagnosis of anti-synthetase syndrome with associated ILD. Despite initial improvement after treatment with intravenous cyclophosphamide and high dose steroids, he relapsed shortly after, with additional muscular symptoms. Subsequent escalation of therapy with rituximab resulted in sustained remission. Considering the scarcity of data about the clinical presentation and prognosis of patients with anti-Ha antibodies, our report provides additional information on diagnostic challenges and therapeutic response in these patients.