Title : Primary APLA syndrome presenting as severe CTEPH - A CASE REPORT
Abstract:
INTRODUCTION:
Antiphospholipid antibody syndrome ( APLA syndrome ) is an autoimmune disease, with hypercoagulable state leading to dangerous clotting in arteries and veins.Those with APS make abnormal proteins called antiphospholipid autoantibodies in the blood. This causes blood to flow improperly and can lead to dangerous clotting in arteries and veins. APS occurs as a primary condition or in the setting of an underlying systemic autoimmune disease, particularly systemic lupus erythematosus (SLE). APS affects women five times more commonly than men and is associated with obstetric complication.Patients with APS and catastrophic APS are recognized to have a high risk of recurrent thrombosis that can occur despite anticoagulant therapy. Although antithrombotic therapy remains the mainstay of treatment, bleeding manifestations can complicate management and contribute to increased morbidity.
CASE REPORT:
In this case study we report a 30 year old male who is a known case of CHRONIC THROMBOEMBOLIC PULMONARY HYPERTENSION, B/L BRONCHIECTASIS and COR PULMONALE who presented with recurrent bouts of hemoptysis and Grade 3 Dyspnoea. Patient was managed with BRONCHIAL ARTERY EMBOLISATION on september 2019 and patient was started on T.SILDENAFIL 20mg. Lung perfusion scan findings suggest perfusion defects in both lungs due to chronic pulmonary embolism. Pt recalls frequent childhood respiratory infections and no other known comorbidities. Later he was admitted in the THORACIC MEDICINE department of Madras medical College in 2023 with further episodes of hemoptysis and series of investigations were carried out, On evaluation, CBC implied Polycythemia and ABG values implied TYPE 1 respiratory failure.
Chest Xray showed prominent pulmonary vasculature , straightening of left heart border and a dilated main pulmonary artery. Pt was then assessed with Right heart study and Vasoactivity testing which showed in view of CTEPAH and probable Group 4 PAH and HIGH PVRI . Rheumatological opinion and Laboratory investigation revealed positive for IgG Anticardiolipin antibody with a negative ANA profile suggesting no other underlying autoimmune disorder. Accordingly he was diagnosed as primary APLA syndrome and was treated with long term STEROIDS, HCQ ,ANTI COAGULANTS and VASODILATORS.
PLAN:
patient is in line for planning with pulmonary endarterectomy after further investigations and evaluation from the department of cardiothoracic vascular surgery.
Audience Take away:
- Second most common presentation of APLA syndrome is a pulmonary complication of CTEPH .
- To think of possibilities like APLA in certain cases of hemoptysis with cor pulmonale , and start timely treatment.
- To suspect rare cases beyond common diagnosis.
- To explore timely permanent solutions for patients with chronic long term debilitating diseases.
