We report a case of a 54-year-old man who developed an atypical systemic syndrome involving Raynaud’s phenomenon, pulmonary fibrosis and skin thickening. These features were initially suggestive of newly diagnosed scleroderma. However, he displayed atypical clinical features of same, antinuclear antibody was negative and symptoms were refractory to various immunosuppressive therapies. CT imaging revealed a gastric mass, which later proved to be a gastrointestinal stromal tumour (GIST). Resection of the GIST leads to minimal symptomatic improvement. Surveillance imaging 1 year later revealed metastatic deposits. He was subsequently initiated on imatinib therapy, which led to a rapid improvement in fibrotic changes within weeks. While there have been previous descriptions of paraneoplastic fibrotic disorders, this is the first description of a scleroderma mimic in the setting of a GIST. It highlights an important potential overlap in the pathogenesis of these disease processes and the potential efficacy of tyrosine kinase inhibitors for scleroderma-like fibrotic disorders.

Background/Aims  Gastro-intestinal stromal tumour (GIST) is recognised as the most prevalent mesenchymal tumour of the gastro-intestinal (GI) tract. The vast majority of GIST display activating mutations in receptor tyrosine kinases, such as platelet-derived growth factor receptor alpha (PDGFRA) and c-kit. This characteristic has lead to the development and approval of targeted therapies such as Imatinib for metastatic GIST. Scleroderma is an idiopathic fibrotic syndrome which involves excess collagen deposition in multiple tissues and organs. There is emerging evidence that dysfunctional fibroblasts are central to the underlying pathogenesis of this disease. An increasing awareness of the complex interplay of these fibroblasts with other cells and inflammatory mediators, such as platelet-derived growth factor (PDGF), is being recognised. This novel case serves to further develop our understanding of these pathological interactions, and to highlight a prospective role for Imatinib in disrupting them. Methods  We present a case report below. Results  A 54-year-old Caucasian male was referred to our rheumatology clinic due to worsening Raynaud's syndrome, arthralgia, and dry cough. He subsequently developed weight loss, pyrosis and skin thickening. This clinical picture was suggestive of a new diagnosis of systemic sclerosis. However, fibrotic skin changes did not extend to the distal finger, and anti-nuclear antibody (ANA) was negative. Symptoms were also refractory to various vasodilator and immunosuppressive therapies, including nifedipine, repeated iloprost infusions, azathioprine, mycophenolic acid, prednisolone, and sildenafil. Computed tomography (CT) imaging revealed interstitial pulmonary fibrosis and a mass within the gastric fundus. Histopathologic features and immunological staining of this mass were consistent with a GIST. Resection of the tumour did not improve fibrotic symptoms. Surveillance imaging one-year later was highly concerning for metastatic recurrence, which was later confirmed with tissue biopsy. He was subsequently initiated on Imatinib therapy, which led to a rapid improvement in fibrotic changes within weeks. Conclusion  While there have been previous descriptions of para-neoplastic fibrotic syndromes, this is the first reported case of a scleroderma-mimicking disorder associated with GIST. It hints at an important potential overlap in the pathogenesis of these disease processes, and a potential role for tyrosine-kinase inhibitors in the treatment of scleroderma-like fibrotic disorders. Disclosure  Z.A. Butt: None. W. Ng: None. D. Howard: None. K. Osman: None.

Background High-sensitivity troponin-T (HS-cTnT) levels are often measured in patients presenting with atrial fibrillation (AF), with many subjected to unnecessary invasive assessments. The significance of a normal or mildly raised HS-cTnT in this context is poorly understood. This study aimed to determine the predictive value of HS-cTnT for significant coronary artery disease (CAD) in new AF with rapid ventricular response. We also compared the discriminative ability of HS-cTnT to suspected angina for significant CAD. Methods We examined patients presenting with new AF to two tertiary Irish centers in a defined period. Those included had HS-cTnT taken at presentation and subsequent ischemic evaluation. Results Of 5350 cases screened for inclusion, 281 were deemed eligible. Of these, 148 and 133 patients had a positive and negative index HS-cTnT, respectively. Of those with negative HS-cTnT, 13 (9.8%) had significant CAD versus 51 (34.5%) with positive HS-cTnT (P < 0.001). Positive Hs-cTnT status remained significant upon multivariate analysis (OR, 2.9; 95% CI, 1.37–6.14; P = 0.005). A similar model where HS-cTnT was replaced with suspected angina produced an OR of 1.64 (95% CI, 0.75–3.59; P = 0.213). A logistic model determined optimal cutoff value for HS-cTnT to be less than 30 ng/l, producing a negative predictive value of 91.8% and area under the receiver operative curve of 83.36. Conclusion HS-cTnT exhibits potential as an effective screening biomarker to predict nonsignificant CAD in new rapid AF, allowing more targeted and rationalized ischemic testing. HS-cTnT may also be a more accurate predictor of significant CAD than clinically suspected stable angina. Graphical abstract: http://links.lww.com/MCA/A540

An 18-year-old male subject presented with recurrent strokes 3 years after percutaneous patent foramen ovale (PFO) closure with an PFO occluder device for presumed PFO-related stroke. A transesophageal echocardiogram revealed apparent device displacement during dynamic cardiac cycle. This case highlights an unusual but important delayed complication of transcatheter PFO closure. ( Level of Difficulty: Intermediate. )