Anti-Synthetase Syndrome with Severe ARDS Masquerading as Severe Covid Pneumonia

Medical History

The patient had a history of 8-10 admissions in 4-5 months at various hospitals in various regions for the aforesaid
complaints. She has been receiving treatment for complicated pneumonia, non-resolving pneumonia, organising
pneumonia, long-haul COVID, complicated pneumonia & septicemia. The treatment protocols included broad-spectrum IV and nebulised antibiotics, nebulisation, steroids, antifibrotics, oxygen therapy, BIPAP, anticoagulants, antitussives, and symptomatic medication.

However, she remained unrelieved of her symptoms, and her clinical condition continuously worsened. She was referred to us for further management in a critical state.

Patient Profile

A 39-year-old female patient from Madhya Pradesh was admitted to the Department of Pulmonology at the CK Birla Hospital. The patient complained of severe breathlessness – Grade 5 MRC even on high flow oxygen support at 15 lit/min. She was also affected with severe Type 1 respiratory failure and severe paroxysmal dry cough, headache, severe generalised body ache and high grade fever for the last four to five months. Patient autoimmune profile was strongly positive for anti jo antibody which is specific for patients suffering from inflammatory myopathies which is seen very rarely and commonly misdiagnosed.

About Anti-Synthetase Syndrome

Anti synthetase syndrome is a rare autoimmune disorder that can affect multiple body parts. In this condition, certain antibodies target specific proteins in the body that lead to the abnormal functioning of the immune system. As a result, this condition causes diverse symptoms, including inflammation of the muscles and interstitial lung disease.

About ARDS

ARDS stands for acute respiratory distress syndrome. In this condition, excess fluid fills up in the air sacs (alveoli) of the lungs causing less oxygen intake. Common symptoms of ARDS include severe shortness of breath, low blood pressure and abnormally rapid breathing.

Investigations and Intervention

• On admission, the patient was severely hypoxic (having an extreme decline in oxygen level in blood cells) on high flow, diaphoretic (having abnormal sweating), tachycardiac (affected with irregular and rapid heartbeat), tachypneic (having abnormal and shallow breathing) with excruciating body ache and unable to move lower limbs with severe work of breathing.
• For precise insights, preliminary tests were performed, including arterial blood gas (ABG) analysis, doppler ultrasound of lower limbs, and CT pulmonary angiogram.
• After reviewing the symptoms and test reports, the pulmonary experts, led by Dr Kuldeep Kumar Grover, decided to admit the patient to the ICU.
• The patient was kept in ICU on high flow for a few hours and then electively intubated because of worsening ARDS and unrelieved of her symptoms.
• She was kept ventilated, sedated and paralysed on ARDS net ventilation. All blood and urine cultures were negative.
• Chest X-ray showed diffuse white-out lungs that are classical of ARDS.
• High-resolution CT scan showed diffuse dense ground glassing with interlobular septal thickening in all the segments of lungs.
• The patient was treated with pulse dose of steroids and IV monthly doses of cyclophosphamide by which patient recovered well.

Post-treatment findings

• The patient responded to the pulse steroids and gradually weaned. She was extubated from the ventilator on the 7th day and is doing fine.
• Chest X-ray showed markedly improvement, and the patient went back home happily on room air.