Pt was empirically started on intravenous methylprednisolone (2?mg/kg) due to issues for ILD, and rheumatology and thoracic surgery services were consulted. Investigations Autoimmune work up was sent and the patient underwent video-assisted thoracoscopic surgery -guided biopsy of the Telatinib (BAY 57-9352) right lung. is usually characterised by the presence of autoantibody against aminoacyl-transfer RNA synthetase, named antisynthetase antibody, and of which anti-Jo-1antibody is the most common, predominantly found in 15C30% of patients with polymyositis and 60C70% of those with interstitial lung disease (ILD).1 2 ASS patients present with various clinical manifestations, including myositis, arthralgia or arthritis, ILD, Raynaud’s phenomenon and mechanic hands. ILD is very frequent in ASS and reported in 60C70% of cases and drives the prognosis. Although ASS is included in the spectrum of inflammatory myopathies, myositis may be absent or delayed after lung involvement.1C4 Here, we statement a first case of a woman aged 61 years who presented with acute respiratory distress syndrome (ARDS) and was subsequently diagnosed with ASS without any myositis features. Case presentation A woman aged 61?years with a medical history of hypertension, heart burn, paroxysmal atrial fibrillation and diastolic heart failure presented to the emergency room (ER) with a 1-week history of shortness of breath on exertion and dry cough in December 2015. Pt was on aspirin, omeprazole and amlodipine at home and denied any fever, chills, chest pain, leg swelling, palpitations, joint swellings, muscle mass weakness, skin rash or any sick contacts. The patient has normal X-ray of the chest 2?weeks prior to admission (physique 1). Family history was significant for ILD in child per the patient, but we were not able to verify the specific type. The patient denied any history of smoking or any other drug use. Open in a separate window Figure?1 X-ray of the chest showing normal lungs few weeks prior. The patient was placed on oxygen as she was hypoxic on room air flow in the ER. Initial abnormal laboratory findings included elevated white cell count (WCC) of 13?330 with no left shift, mild normocytic anaemia Telatinib (BAY 57-9352) with a haemoglobin level of 10.0?g/dL, elevated creatinine of 1 1.6?mg/dL and initial X-ray of the chest showed diffuse interstitial opacities (physique 2). Pt was admitted to the cardiology support for possible acute heart failure exacerbation and pneumonia. Open in a separate window Physique?2 X-ray of the chest showing bilateral interstitial opacities in the lung on admission. She was started on antibiotics and furosemide, and despite these steps, patient’s respiratory status worsened, prompting Telatinib (BAY 57-9352) transfer to the rigorous care unit (ICU) and subsequent endotracheal intubation. X-ray of the chest showed worsening infiltrates bilaterally with WCC elevated to 19?000 and the patient became febrile. Antibiotics were broadened after obtaining cultures and CT scan of the chest without contrast was ordered for further evaluation of the worsening infiltrates (physique 3). CT scan showed diffuse bilateral ground glass opacities in the right middle and bilateral lower lobes. She underwent bronchoscopy with bronchoalveolar lavage (BAL) and trans-bronchial biopsy from the right lower lobe. Diffuse alveolar haemorrhage was ruled out based on BAL and all the Telatinib (BAY 57-9352) cultures from your blood, urine and lung tissue were unfavorable. Trans-bronchial biopsy from your lung showed chronic bronchitis. Open in a separate window Physique?3 CT scan of the chest showing bilateral ground glass opacities on admission. The patient continued to require ventilator support, despite the broad-spectrum antibiotics and unfavorable cultures and underwent tracheostomy. Trans-thoracic echocardiogram (ECHO) was obtained that showed normal ejection portion with stage 1 diastolic dysfunction. A repeat CT scan of the chest without contrast was obtained to evaluate the previous opacities and showed worsening ground glass opacities in the lower lobes bilaterally with reticulation. Pt was empirically started on intravenous methylprednisolone (2?mg/kg) due to issues for ILD, and rheumatology and thoracic surgery services were consulted. Investigations Autoimmune work up was sent and the patient underwent video-assisted thoracoscopic surgery -guided biopsy of the right lung. Autoimmune labs were remarkable for normal creatinine phosphokinase, positive anti-JO-1 and RO-52 antibodies AKT1 (table 1) and lung biopsy showed nonspecific interstitial pattern (NSIP) with fibrosis (physique 4). Table?1 Antibody profile in the patient thead valign=”bottom” th align=”left” rowspan=”1″ colspan=”1″ Antibodies /th th align=”left” rowspan=”1″ colspan=”1″ Result /th /thead Anti-JO-1PositiveRO-52 AbPositiveRNP AbPositiveSS-A (Ro) AbPositiveCCP antibodyPositiveCRPElevatedANAPositiveCPKNormal Open in a separate window Open in a separate window Determine?4 Lung biopsy showing non-specific Telatinib (BAY 57-9352) interstitial pneumonia pattern with fibrosis. Differential diagnosis At this time, differential diagnosis we had for this individual was: Idiaopathic non-specific interstitial pneumonia (NSIP). NSIP due to underlying connective tissue disease. Acute interstitial pneumonia. Drug-induced pneumonitis. Organising pneumonia. Treatment The patient was diagnosed as having ASS with pulmonary involvement (ILD) as the cause of her acute respiratory.