A78-06 A Rare Conundrum: The Chest Under Siege

Abstract Introduction Eosinophilic fasciitis (EF) is a rare connective tissue disorder, also known as “Shulman Syndrome” typically characterized by progressive induration and thickening of the skin and soft tissues. While pulmonary involvement is exceptionally uncommon, Eosinophilic fasciitis may externally restrict chest expansion, leading to a diagnostic dilemma when severe breathlessness and restrictive respiratory physiology are present despite absence of intrinsic lung pathology. Case Presentation A 65-year-old man presented with a 12-month history of exertional breathlessness and significant weight loss (23kg), with rapid progression to wheelchair dependency. He had biopsy-proven EF, previously managed with methotrexate, prednisolone, and hydroxychloroquine. Physical examination revealed marked thickening and induration of the torso and limbs, sparing hands and feet, with reduced chest expansion.Multiple visits to GP and seen by acute medicine, infections disease and cardiology, treated for Asthma and Heart Failure, given diuretics and Inhalers with very little to no benefits. Laboratory studies were within normal limits, and autoimmune and myopathy screens were negative. Radiographically, bi-basal atelectasis was observed on CXR (Fig 1.A), while CT pulmonary angiography excluded embolism. (Fig 1.B.C) Physiological data highlighted a restrictive pattern: FVC 1.51L (38%), FEV1 0.99 (32%), KCO 64%, DLCO reduced. Respiratory muscle studies and Echocardiogram was unremarkable. MRI was done which showed fascial thickening, hyperintense signal with in the fascia on fluid sensitive sequences helping with the diagnosis. After extensive workup the diagnosis of respiratory impairment secondary to extrinsic chest restriction was made. Management involved a multidisciplinary approach, including dermatology and plastic surgery, with extracorporeal photopheresis improving skin symptoms and proposals for surgical release of chest wall restriction. Fig 1.A 1.B 1.C Discussion Eosinophilic Fasciitis presenting as severe extrinsic restriction of chest movement and secondary respiratory impairment is a rare but important diagnostic challenge. While most literature focuses on fascia and joint involvement, a handful of cases report marked respiratory symptoms due to hidebound chest restricting expansion. The primary learning point for respiratory assembly clinicians is to consider extrinsic chest wall restriction, not just intrinsic parenchymal processes, in patients with unexplained restrictive physiology and a background of autoimmune or connective tissue disease. Early and collaborative multidisciplinary assessment—including dermatology, respiratory medicine, and surgical evaluation—can streamline management and offer novel therapeutic options. Fig 2.A 2.B 2.C Fig 2 (A).(B) Orange peel skin with longitudinal depression ‘groove sign’ along the ventral surface of limbs are characteristic. (C) Showing marked fascia infiltration and thickening. This abstract is funded by: None