E1121. Scleroderma: Head to Toe Review for the Body Radiologist
  1. Stephen Smith; University of Rochester Medical Center
  2. Devanshi Mistry; University of Rochester Medical Center
  3. Ravinder Sidhu; University of Rochester Medical Center
Scleroderma is an autoimmune disease characterized by vasculopathy and fibrosis which leads to dysfunction of multiple organ systems. The GI tract is the most commonly affected organ system involved in progressive scleroderma. The severity of GI system involvement is correlated with poor clinical outcomes. Therefore, pathology identified by radiologists can serve as a prognostic decision-making tool for referring clinicians. Additionally, the knowledge of scleroderma is still evolving and the disease is extremely heterogeneous. Radiologists can serve a powerful clinical role in helping to guide patient treatment in such a heterogeneous disease. The purpose of this presentation is to educate on scleroderma as a disease process, discuss important clinical features within the GI tract, and educate on common extraintestinal manifestations which can increase confidence in a scleroderma diagnosis.

Educational Goals / Teaching Points
1. Provide an overview on scleroderma pathophysiology and diagnosis 2. Describe the spectrum of GI tract involvement in scleroderma 3. Discuss the relevance of unique imaging findings in the management of scleroderma patients 4. Demonstrate extraintestinal manifestations which can increase confidence on a scleroderma diagnosis

Key Anatomic/Physiologic Issues and Imaging Findings/Techniques
To meet these goals, scleroderma involvement will be discussed throughout the GI tract including salivary gland involvement (fibrous replacement of glands and increased risk of squamous cell carcinoma), esophagus (gastroesophageal reflux, strictures, Barrett’s esophagus, and esophageal cancer), stomach (gastroparesis and gastric antral vascular ectasisa), small bowel (pneumatosis cystoides intestinalis, chronic intestinal pseudo-obstruction, hide-bound bowel sign, jejunal diverticula), large bowel (chronic constipation, loss of haustra, and wide mouth diverticula), and liver disease (most commonly primary biliary cirrhosis). Modalities will include abdominal CT, abdominal ultrasound, fluoroscopy, and nuclear medicine gastric emptying exams. The presentation will then focus on a variety of imaging findings outside of the GI tract which can increase diagnostic certainty and may be less familiar to a body radiologist. To meet this goal, a variety of additional sites of organ involvement will be discussed including the skeletal system (acro-osteolysis, periarticular osteopenia, soft tissue calcifications, erosions, etc.), lungs (scleroderma-related interstitial lung disease), the heart (myocarditis), and vascular involvement (vessel narrowing due to fibrosis). Modalities will include cardiac MR, chest CT, hand radiography, and angiography.

Radiologists can serve a pivotal role in the diagnosis of scleroderma and identifying the broad range of organ system involvement in patients with scleroderma. These valuable roles can ensure patients receive prompt and appropriate clinical treatment. A better knowledge of scleroderma can also help to avoid unnecessary medical imaging and clinical consultations. Finally, radiology interpretations can be used by referring clinicians to help stratify patients and provide information on patient prognosis.