Diffuse Cutaneous Systemic Sclerosis presenting With High Grade Distal Small Bowel Obstruction: A Case Report

Summary: Background Systemic sclerosis (SSc) is a rare autoimmune disease characterized by fibroproliferative microvasculature alterations leading to fibrosis and loss of function of the skin and internal organs. Intestinal motility dysfunction is commonly reported in SSc, with a 40-88% prevalence rate. However, 65% of the SSc patients are asymptomatic, leading to an under-diagnosis or delayed diagnosis of small bowel involvement. Small bowel involvement in SSc can manifest as dysmotility secondary to poor smooth muscle activity and stiffness, leading to complications such as pseudo-obstruction, pneumatosis cystoides intestinalis, small intestinal bacterial overgrowth, and diverticula. Case Presentation A 71-year-old female with diffuse cutaneous systemic sclerosis and a heavy burden of calcinosis presented to the emergency room with increasing abdominal distension, non-bloody diarrhea and emesis that started four days before admission. The patient mentioned having similar prior symptoms of abdominal distension, causing her recurrent emesis leading to hospitalization due to small bowel obstruction. She denied any associated symptoms, such as fever, chills, diarrhea, acute abdominal pain or prior abdominal surgeries. Patient was found hemodynamically stable with increased abdomen distention in no acute distress. Labs were remarkable for WBC 11.6, lipase 692, UA +2 proteins, +hyaline and granular casts 15-19, Specific gravity 1.030. CT abdomen revealed a suspected internal hernia within the right lower quadrant, two different/distinct transition points and diffuse proximal small bowel dilatation suspicious for complete small bowel obstruction. CT enterography redemonstrated severe diffuse small bowel dilation, gastric distention and severe esophageal dilation. Additionally, extensive diffuse subcutaneous calcification was seen within the anterior abdominal wall. Manage with IV fluid for hydration, electrolyte replacement, clear liquid diet, and nasal-gastric tube placement with suction for decompression. Over the following days, her small bowel obstruction resolved, and diet was subsequently advanced. Upon discharge the patient continued to have intermittent bloating and distention, prompting her gastroenterologist to try domperidone, octreotide and antibiotics for small intestinal bacterial overgrowth. Discussion Despite the propensity of SSc to lead to pseudo-obstruction, patients can still develop mechanical small bowel obstruction, making the use of neostigmine contraindicated. Dietary and lifestyle changes and medications should be the mainstay of treatment for SSc-related GI dysmotility. The choice of prokinetic agent is dependent on the patient’s condition and the extent of GI dysmotility. Surgery is recommended with serious, rare complications such as bowel perforation or ischemia. Conclusion We aim to increase awareness and avoid the morbidity and mortality associated with developing mechanical small bowel obstruction and pseudo-obstruction in patients with SSc. We also want to emphasize the importance of a multidisciplinary approach involving gastroenterologists, rheumatologists, and surgeons to optimize the care of individuals with SSc. References • Edoardo Savarino, Manuele Furnari, Nicola de Bortoli, Irene Martinucci, Giorgia Bodini, Massimo Ghio, Vincenzo Savarino,Gastrointestinal involvement in systemic sclerosis, La Presse Médicale, Volume 43, Issue 10, Part 2,2014,Pages e279-e291,ISSN 0755-4982, https://doi.org/10.1016/j.lpm.2014.03.029. • J. Philip, N. Lickiss, P.T. Grant, N.F. Hacker,Corticosteroids in the Management of Bowel Obstruction on a Gynecological OncologyUnit,Gynecologic Oncology, Volume 74, Issue 1, 1999,Pages 68-73,ISSN 0090-8258, https://doi.org/10.1006/gyno.1999.5406. • Kanyakorn Jaovisidha, M.E. Csuka, Urias A. Almagro, Konrad H. Soergel, Severe gastrointestinal involvement in systemic sclerosis: Report of five cases and review of the literature,Seminars in Arthritis and Rheumatism,Volume 34, Issue 4,2005,Pages 689-702,ISSN 0049-0172, https://doi.org/10.1016/j.semarthrit.2004.08.009. • Massat B, McCarthy J. Systemic Sclerosis, Malnutrition, and Small Bowel Obstruction: Why Clinicians Should Consider Early Total Parenteral Nutrition in Systemic Sclerosis With Severe Gastrointestinal Involvement. Cureus. 2022 Aug 3;14(8):e27638. doi: 10.7759/cureus.27638. PMID: 36072159; PMCID: PMC9438553.