Rare Extraintestinal Manifestation Research Articles

Ulcerative colitis complicating pneumomediastinum, subcutaneous emphysema and pneumothorax.

A 17-year-old man with ulcerative colitis (UC) presented to our hospital with neck pain and fever after vomiting. On examination, a snowflake sensation was noted in the neck. A chest radiograph showed extensive subcutaneous emphysema in the chest. CT scans showed extensive subcutaneous emphysema in the neck, shoulders and axilla, as well as pneumomediastinum and pneumothorax. A diagnosis of pneumomediastinum with exacerbation of UC was made, and he was fasted and treated with antibiotics. Intensive granulocyte and monocyte adsorption apheresis (GMA) was started for exacerbation of UC. His symptoms and the radiological findings of pneumomediastinum improved. He remained in remission on azathioprine. UC is a chronic inflammatory bowel disease (IBD) associated with extraintestinal manifestations (EIM). Very few cases have been complicated by pneumomediastinum. The increase in alveolar pressure due to vomiting and systemic inflammation-related pleural or esophageal damage may cause pneumomediastinum in this case. Prevention of progression to mediastinitis and treatment of exacerbated UC are contradictory. GMA was successful because it was not an immunosuppressive therapy. Our case highlights that rare EIM may complicate exacerbation of UC.

Incidence, Predictors, and Outcomes of Acute Pericarditis in Patients with Inflammatory Bowel Disease: A 10-Year Nationwide Analysis

BackgroundInflammatory bowel disease (IBD) is a chronic condition characterized primarily by inflammation of the gastrointestinal tract. Pericarditis is a rare but important extraintestinal manifestation of IBD that is poorly understood yet is associated with significant morbidity. The objectives of this study were to identify the factors associated with pericarditis in IBD and associated complications. MethodsHospitalized adult patients (aged ≥ 18 years) with a diagnosis of acute pericarditis in the IBD cohort, 2011-2020, were identified from the National Inpatient Sample using codes from the International Classification of Diseases (revision 9 or 10). Multivariable logistic regression was performed to identify clinical factors associated with pericarditis among IBD patients and in-hospital complications. ResultsDuring the period 2011-2020, among 3,236,747 IBD patients, 9113 (0.28%) had pericarditis, with a mean patient age of 54.08 ± 0.48 years, and 53.1% females. Patients with IBD and coexisting diagnoses of systemic lupus erythematosus (odds ratio [OR] 1.49, 95% confidence interval [CI] 1.03-2.15, P = 0.033), rheumatoid arthritis and/or collagen vascular disorders (OR 1.75, 95% CI [1.41-2.17], P < 0.001), or postcardiotomy syndrome (OR 67.13, 95% CI [30.08-149.80], P < 0.001), were each associated with a higher risk of pericarditis. Compared to IBD patients without pericarditis, patients with IBD and pericarditis had an increased associated incidence of inpatient mortality (OR 1.65, 95% CI [1.25-2.18], P < 0.001). ConclusionsPericarditis is an uncommon but important manifestation of IBD. The presence of a concomitant autoimmune condition led to a higher likelihood of developing pericarditis among IBD patients, and IBD patients who develop pericarditis had a higher incidence of inpatient mortality compared to IBD patients without pericarditis. Providers should be aware of the connection between IBD and pericarditis to identify individuals at risk of adverse complications.

A rare occurrence of myocarditis with acute heart failure as an extraintestinal manifestation of Crohn&amp;rsquo;s disease in a young male

Crohn’s disease (CD) is an idiopathic inflammatory bowel disorder, which can be associated with various extraintestinal manifestations. Cardiac and pulmonary manifestations of CD are rare with only a few cases being reported. Myocarditis is an unusual and rare extraintestinal manifestation of CD. Only a few case reports associating CD with the above manifestation are found in the literature. Here, we report a case of an 18-year-old man diagnosed with CD for two years, on optimum treatment, presenting with the symptoms of myocarditis with acute heart failure, as extraintestinal manifestations in the absence of bowel symptoms, while being negative for infective and non infective aetiological screening. This case highlights the importance of knowing the rare extraintestinal manifestations of CD, which will ultimately guide the treating physicians.

Gastrointestinal: A case of ulcerative colitis complicated by Tolosa-hunt syndrome and pyoderma gangrenosum and featuring rare extraintestinal manifestations.

Gastrointestinal: A case of ulcerative colitis complicated by Tolosa-hunt syndrome and pyoderma gangrenosum and featuring rare extraintestinal manifestations.

Metastatic crohn’s disease in a child: rare extraintestinal manifestation

Extraintestinal manifestations occur in every second child with Crohn’s disease, can affect any organ system, and determine the type of therapy and prognosis of the disease. The skin is the most frequent “target organ” of all extraintestinal manifestations, the spectrum of skin lesions is extremely clinically and pathogenically heterogeneous. Metastatic Crohn’s disease (MBC) is a rare extraintestinal manifestation of the disease, approaches to the diagnosis and management of which are not standardized in either adults or children. The most frequent description of MBC in the literature is skin damage, although interstitial lung damage, bone and muscle damage, and ENT organs, could be destructive. MCD lesions vary in morphology and can arise anywhere on the skin, rarely undergo reverse development, require special approaches to therapy. The primary diagnosis of such elements is extremely difficult, often delated, biopsy is required for a definitive diagnosis - the detection of epithelioid granuloma and its elements outside the gastrointestinal tract. Therapeutic approaches to MCD are not standardized now, they are carried out according to the protocols of CD management, and the effect of the use of oral steroids, a-TNFa drugs is shown. A case of metastatic Crohn’s disease in a 16-year-old teenager with an unusual localization - the nasal septum is described. The destructive nature of the lesion with the formation of perforation of the nasal septum determined the difficult path of differential diagnosis with Wegener’s granulomatosis. Morphological examination of the biopsy of the nasal mucosa revealed epithelioid cells of sarcoid-like granuloma. Systemic glucocorticosteroids use (GCS) led to epithelialization of the nasal septum defect and a decrease in the activity of CD in the intestine mucosa.

P490 Prevalence and clinical features of chronic nonbacterial osteomyelitis in a nationwide cohort of children with inflammatory bowel diseases

Abstract Background Among musculoskeletal extraintestinal manifestations (EIM), the association between IBD and a rare autoinflammatory condition, chronic nonbacterial osteomyelitis (CNO), has been reported. The primary objective of our study was to report the clinical characteristics and the disease course of a cohort of patient with concurrent diagnosis of CNO and IBD. The secondary outcome was to define the prevalence of CNO in a nationwide cohort of patients with IBD. Methods This is a retrospective, multicenter study based on the Italian Society of Gastroenterology, Hepatology, and Pediatric Nutrition (SIGENP) IBD National Registry. The diagnosis of CNO was defined as the presence of uni- or multifocal bone inflammatory lesions with suggestive radiological or histopathological characteristics, having excluded infectious, oncological, or other inflammatory conditions. All patients under 18 years of age with a combined diagnosis of IBD and CNO identified in the registry were included. Results 18 patients with a combined diagnosis of CNO and IBD out of 4229 patients with IBD were identified, with an overall prevalence of 0.4%. 15 (83.3%) patients were male. Median age at IBD diagnosis was 10.1 years (IQR: 6.16 years), whereas median age at CNO diagnosis was 11.7 years (IQR: 6.31 years). The most frequently reported IBD phenotype was CD (13 patients, 72.2%), followed by IBD-U (3 patients, 16.7%) and UC (2 patients, 11.1%). The diagnosis of concurrent CNO was more common in patients affected by CD than in those affected by either UC or IBD-U (13/1843, 0.7% vs 5/2886, 0.2%, p=0.01). Similarly, the occurrence of CNO was more frequent in male gender when compared to female one (15/2312, 0.6% vs 3/1917, 0.1%, p=0.01). All the patients had multifocal bone involvement; 2 (11.1%) developed subsequently dermatological manifestations consistent with SAPHO syndrome. In 8 patients (44.4%) IBD onset preceded CNO diagnosis, in 6 (33.3%) IBD onset followed CNO diagnosis, whereas in the remaining 4 patients (22.2%) the diagnosis of IBD and CNO was concomitant. Table 1. summarizes therapeutic approach to both conditions in each of the patient identified in our case series. At the end of follow-up, 14 (77.8%) patients had achieved IBD clinical and biochemical remission. Ten (55.6%) patients achieved clinical and radiological remission of bone lesions, 6 (32.3%) had achieved clinical remission but showed persistence of bone lesions at magnetic resonance imaging (MRI), 2 (11.1%) had persistence of both clinical and radiological activity. Conclusion Our findings support the hypothesis that CNO disease spectrum can be considered a rare extraintestinal manifestation of IBD, with a low but still non-negligible prevalence.

Orbital myositis in a patient with Crohn's disease: A case report of two episodes

To describe a case report of a 34 years-old patient with Crohn's Disease and two episodes of Ocular Myositis. The research methodology employed in this study consisted of a retrospective review of the patient's complete medical history. Crohn's Disease is a chronic inflammatory bowel disorder known to be associated with a wide range of extraintestinal manifestations. Ocular abnormalities, such as episcleritis and uveitis, are commonly observed. However, orbital myositis is an extremely rare ocular extraintestinal manifestations characterized by acute ocular pain that worsens with eye movements and is often accompanied by diplopia. In this case report, we present the case of a 34-year-old woman with a confirmed diagnosis of Crohn's Disease, who experienced two episodes of acute orbital pain exacerbated by ocular movements and diplopia. The diagnosis was established through clinical evaluation and radiologic imaging, with confirmation after a good response to systemic corticosteroids. She responded favorably to systemic corticosteroid therapy on both episodes, and no additional treatment was required. As of now, she remains stable without any ocular sequelae. It is important to note that orbital myositis is an uncommon ocular manifestation associated with Crohn's Disease, and prompt recognition and management are crucial to achieve successful outcomes.

Long-term remission achieved in a rare case of pyoderma gangrenosum and ulcerative colitis with surgery and postoperative infliximab.

Background Pyoderma gangrenosum (PG) is a rare extraintestinal manifestation of inflammatory bowel disease. In recent years, the use of biologics in PG has been on the rise and has shown promising results. The surgical treatment of PG remains a topic of debate, with limited reports on the use of postoperative biologic therapy. Case reprt: This case report describes a 52-year-old woman who presented with multiple skin ulcers, pus discharge, and bloody diarrhea. The patient was diagnosed with PG with ulcerative colitis based on medical history, ulcer appearance, histopathology, treatment response, and the presence of ulcerative colitis. Surgical intervention was performed to repair the ulcers and amputate the fourth finger and fourth toe of both feet. Additionally, infliximab induction therapy was initiated two weeks after the surgery. The patient's intestinal symptoms demonstrated improvement, and after 10 months of treatment, the lesions were completely healed with no recurrence of skin ulcers. Conclusions This case report highlights a rare instance of successful treatment for PG with ulcerative colitis through a combination of surgery and postoperative infliximab.

S120 Beyond the Bowels: Immune Thrombocytopenic Purpura Presents as a Rare Extraintestinal Manifestation of Ulcerative Colitis

S120 Beyond the Bowels: Immune Thrombocytopenic Purpura Presents as a Rare Extraintestinal Manifestation of Ulcerative Colitis

S1056 Clinical Characteristics of Metastatic Crohn’s Disease, a Rare Cutaneous Extra-Intestinal Manifestation: A Systematic Review

S1056 Clinical Characteristics of Metastatic Crohn’s Disease, a Rare Cutaneous Extra-Intestinal Manifestation: A Systematic Review

Sweet Syndrome Associated with Active Inflammatory Bowel Disease: A Case Series of a Rare Extra-intestinal Manifestation.

Cutaneous extra-intestinal manifestations (EIM) occur in up to 20% of patients with IBD. Information about Sweet syndrome (SS)'s clinical course as a rare cutaneous EIM in IBD is limited to case reports. We present the largest retrospective cohort on the occurrence and management of SS in IBD. Electronic medical records and paper charts since 1980 were retrospectively reviewed at a large quaternary medical center to identify all adult IBD patients with histopathology-proven SS. Patient characteristics and clinical outcomes were evaluated. 25 IBD patients with SS were identified; 3 patients were assessed to have AZA-induced SS. The majority of SS patients were female. Median age at diagnosis was 47years (IQR 33-54years) and SS appeared at a median of 6.4years after IBD diagnosis. IBD patients with SS had a high rate of complicated IBD phenotypes (75% extensive colitis in UC and 73% stricturing or penetrating disease in CD, with 100% colonic involvement), as well as frequent co-occurring EIMs (60%). SS correlated with global IBD disease activity. Corticosteroids were an effective therapy for SS in IBD. Recurrence rate of SS was 36%. Contrary to previous case reports, SS was a cutaneous EIM occurring late after diagnosis of IBD in our cohort, with occurrences paralleling global IBD disease activity. Although AZA-induced and IBD-associated SS were both effectively treated with corticosteroids, distinguishing them is relevant for future IBD treatment strategies.

Managing metastatic Crohn’s disease: a single center experience, review of the current evidence, and treatment algorithm

Background Crohn’s disease (CD) is an inflammatory bowel disease (IBD) that, besides gastrointestinal symptoms, may encompass extra-intestinal symptoms, such as dermatological manifestations. Of those, metastatic CD (MCD) is a rare extra-intestinal manifestation for which the management is uncertain. Methods We conducted a retrospective case series of patients with MCD seen at the University hospital Leuven, Belgium, combined with an overview of the recent literature. Electronic medical records were searched from January 2003 till April 2022. For the literature search, Medline, Embase, Trip Database, and The Cochrane Library were searched from inception to April 1, 2022. Results A total of 11 patients with MCD were retrieved. In all cases noncaseating granulomatous inflammation was found on skin biopsies. Two adults and one child were diagnosed with MCD prior to their diagnosis of CD. Seven patients were treated with steroids (intralesional, topical or systemic). Six patients needed a biological therapy to treat MCD. Surgical excision was performed in three patients. All patients reported a successful outcome and most cases achieved remission. The literature search yielded 53 articles, including three reviews, three systematic reviews, 30 case reports and six case series. A treatment algorithm was generated based on literature and multidisciplinary discussion. Conclusion MCD remains a rare entity and diagnosis is often difficult. A multidisciplinary approach including skin biopsy is necessary to diagnose and treat MCD efficiently. Outcome is generally favorable, and lesions respond well to steroids and biologicals. We propose a treatment algorithm based on the available evidence and multidisciplinary discussion.

Multiple ulcerative colitis-associated aseptic abscesses successfully treated with infliximab: a case report.

Aseptic abscesses are rare extraintestinal manifestations of inflammatory bowel disease. Herein, we present the case of a 69-year-old female patient with ulcerative colitis in whom multiple aseptic abscesses were successfully treated with infliximab. Aseptic abscesses associated with ulcerative colitis are difficult to differentiate from infectious abscesses. In the present case, we reached a diagnosis of aseptic abscesses associated with ulcerative colitis as antibiotics were ineffective and repeated Gram stains and cultures of blood and abscess were negative. Aseptic abscesses are commonly found in the spleen, lymph nodes, liver, and skin; however, in the present case, the periosteum was the major site. Prednisolone is often effective for aseptic abscesses; however, the present patient was initially treated with a combination of 40mg/day of prednisolone and granulocyte and monocyte adsorption apheresis, with inadequate effect. Infliximab was administered as the patient was steroid-resistant, with strong effect. Subsequently, infliximab treatment has been continued, with no recurrence after 2years. However, as there have been reports of cases of recurrence even after remission with treatment, careful follow-up in the future is therefore necessary.

AN UNUSUAL MANIFESTATION OF METASTATIC CROHN’S DISEASE: A CASE REPORT

Crohn’s Disease (CD) is a chronic granulomatous inflammatory disease involving any section of the gastrointestinal (GI) tract. Metastatic Crohn’s Disease (MCD) is a rare extraintestinal manifestation of CD defined as cutaneous granulomatous lesions non-adjacent to the GI tract. We present a case of suppurative lymphadenitis as a presentation of MCD. This has not yet been reported in the literature.

AN UNUSUAL MANIFESTATION OF METASTATIC CROHN’S DISEASE: A CASE REPORT

Abstract INTRODUCTION Crohn’s Disease (CD) is a chronic granulomatous inflammatory disease involving any section of the gastrointestinal (GI) tract. Metastatic Crohn’s Disease (MCD) is a rare extraintestinal manifestation of CD defined as cutaneous granulomatous lesions non-adjacent to the GI tract. We present a case of suppurative lymphadenitis as a presentation of MCD. This has not yet been reported in the literature. CASE REPORT A 22-year-old female with a history of iron deficiency anemia and left calf abscess, secondary to Methicillin-Resistant Staphylococcus Aureus (MRSA) infection, was diagnosed with ileocolonic CD at 18. Her CD was complicated by strictures and abdominal and left tubo-ovarian abscesses with a fistula to the sigmoid. She underwent Ileocolonic resection, sigmoid colectomy and left salpingo-oophorectomy. Histology of the ovarian abscesses unveiled granulation tissue with pseudocysts. Azathioprine and Infliximab were started. At 20, the patient reported acute onset of swelling and pain on the left side of her neck. She denied any constitutional symptoms, sick person or animal contacts, travel, use of recreational drugs and sexual activities. Physical exam was remarkable for a tender, rubbery, 2-centimetre left cervical lymph node. The neck Ultrasound showed cervical lymphadenitis. Antibiotic was started and later extended to target resistant infections. Despite this, she reported worsening symptoms. Computed Tomography of the neck noted a collection suggestive of suppurative lymphadenitis. Incision and Drainage (I &amp; D) of the abscess was done; MRSA was detected. Histology exposed necrotizing granulomata encircled by palisading histocytes and giant cells. There was no evidence of dysplasia, fungal, acid-fast bacilli or spirochetes. Further, Human Immunodeficiency Virus serology, Interferon-Gamma Release Assay and chest radiograph were unremarkable. Later, she underwent surgical excision revealing a fistula to the lower mandible. A drain was placed and then removed upon recovery. Meantime, her CD stayed stable; Infliximab was held till the completion of antibiotics. A year later, she reported a tender, swollen lump on the left side of her groin with an ultrasound confirming inguinal suppurative lymphadenitis. Antibiotics were started, and I &amp;D was done for lasting symptoms. The culture and histology were similar to the previous. Considering the history of CD and histology findings, MCD was concluded as the etiology of this patient’s recurrent MRSA abscesses. CONCLUSION Patients on biologic therapy are immunocompromised and presumed high risk for super-infections. In a setting of a persistent infection, despite antibiotics, the possibility of MCD as the cause of lymphadenitis should be raised to cue the clinician to alter therapy and address the MCD within a multidisciplinary setting.

Severity of acute pancreatitis in patients with inflammatory bowel disease in the era of biologics: A propensity-score-matched analysis using a nationwide database in Japan.

Acute pancreatitis (AP) is a rare extraintestinal manifestation of inflammatory bowel disease (IBD). Several studies from Western countries have reported that the severity of AP in patients with IBD is similar to that in the general population; however, its severity in patients from Eastern countries in the era of biologics remains unclear. This study aimed to investigate the severity of AP in patients with IBD and the effect of biologics on the severity of AP using a nationwide database. We divided 1138 eligible AP admissions from the Diagnosis Procedure Combination database system into IBD and non-IBD groups after propensity score matching, and compared the severity of AP. We divided the IBD group into ulcerative colitis (UC) and Crohn's disease (CD) subgroups and compared each with the non-IBD group. Logistic regression analysis was conducted to identify the clinical factors affecting acute pancreatitis. IBD and UC groups had lower rate of severe AP compared to the non-IBD group (13.7% vs 28.3%, P < 0.0001 and 11.0% vs 28.3%, P < 0.0001, respectively). There were no differences in the rates of severe AP between the CD and non-IBD groups. Multivariate analysis showed that biologics did not affect the severity of AP. The severity of AP in patients with IBD may be lower than that in the general population; biologics for IBD may not worsen its severity. Further prospective studies are required to clarify the severity of AP in patients with IBD.

Autoimmune Hemolytic Anemia in Inflammatory Bowel Disease-Report of a Case and Review of the Literature.

A wide spectrum of extraintestinal manifestations (EIMs) can burden patients with inflammatory bowel disease (IBD). EIMs contribute fairly to morbidity and mortality rates in IBD patients. Moreover, EIMs in IBD patients are so frequent that some suggest that IBD should be approached as a systemic disorder. Anemia is very common in IBD patients. The two most common types of anemia in IBD, iron deficiency anemia and anemia of chronic disease, are extraintestinal complications. Autoimmune hemolytic anemia (AIHA) is a rare extraintestinal manifestation of IBD, more frequent in ulcerative colitis (UC) than in Crohn's disease (CD). In this case-based review of the literature, we present a 36-year-old female patient diagnosed with Crohn's disease (CD) and Coombs positive AIHA, complicated by pulmonary thromboembolism and successfully treated with anti-tumor necrosis factor (anti-TNF) therapy. The underlying pathophysiological mechanism of AIHA in IBD is unclear. Treatment options for AIHA in IBD patients before biologic therapy included corticosteroids alone or in combination with azathioprine (AZA), methotrexate, and surgical treatment (colectomy and/or splenectomy). Currently, biologic therapy is a promising therapeutic option, especially in corticosteroid refractory or corticosteroid-dependent IBD patients with AIHA.

S2756 Not So Sweet: A Rare Extraintestinal Manifestation in Crohn's

Introduction: Acute febrile neutrophilic dermatosis (Sweet’s syndrome) is a rare extraintestinal manifestation (EIM) of inflammatory bowel disease (IBD). Other neutrophilic dermatoses include pyoderma gangrenosum and erythema nodosum. Only 40 cases associated with Crohn’s disease have been reported in the literature. Characteristics include abrupt onset of painful erythematous nodules with fever, arthralgias, and leukocytosis which typically resolve with systemic corticosteroids. Case Description/Methods: We describe a case involving a 22-year-old male with presumed UC associated fulminant colitis and perforation requiring ostomy. During staged proctocolectomy with plans for a J pouch, he underwent flexible sigmoidoscopy with biopsies consistent with UC. He was started on high dose Infliximab. One month after being placed in continuity as the final stage of J pouch creation, he developed fevers and wound dehiscence. Imaging revealed fistula formation giving rise to the concern for Crohn’s colitis. Despite antibiotics, his fevers persisted. During this hospitalization, he developed red papules and pustules with peripheral scaling on his face spreading across his body (picture 1) with evidence of pathergy at IV sites. Dermatology diagnosed him with acute febrile neutrophilic dermatosis. Despite the concern for poor wound healing, he was treated with IV corticosteroids 1mg/kg for 5 days with resolution of his lesions. He remains stable on Infliximab. Discussion: Most commonly, Sweet's Syndrome is reported in women (87%) and associated with active disease (67-80%). It is more common in Crohn's disease than in UC. Early recognition is important in order to start corticosteroids quickly in concert with traditional IBD therapy. Not only does this case demonstrate a rare finding of Sweet’s syndrome in a male with Crohn’s, it brings up an important discussion about acute systemic corticosteroid therapy initiation during the perioperative period. It is known that corticosteroids affect the process of wound healing. This case highlights a favorable outcome despite use of high dose steroids in this patient who was critically ill requiring treatment. In review of existing literature, 1 randomized double-blind study evaluated the effect of acute administration of steroids in the perioperative period. This study discovered no wound infections and only 1 patient with wound dehiscence. Sweets is a rare EIM and in severe presentations, systemic steroids are indicated, despite presenting in the immediate perioperative period.Figure 1.: Picture 1.

S2706 Orofacial Granulomatosis in Crohn's Disease

Introduction: Extra-intestinal manifestations (EIMs) of inflammatory bowel disease (IBD) are rare complications that affect roughly 6-25% of IBD patients. Orofacial granulomatosis (OFG) is a rare manifestation that does not have reliable epidemiologic data and is characterized by orofacial swelling with non-caseating granulomas seen on pathology. Case Description/Methods: A 23-year-old male with a history of Crohn’s colitis presented with loose stools and hematochezia for 7 months and 1 year of left sided lip swelling. He was diagnosed with Crohn’s disease at age 15 and was maintained on oral mesalamine and 6-mercaptopurine (6-MP). At age 16, the patient was evaluated for supraglottic edema. Urgent tonsillectomy was performed, with pathology revealing necrotizing and non-caseating granulomas. He recovered without complication. He achieved endoscopic remission at age 18 and his medications were discontinued. Unfortunately, he was lost to follow-up. During this subsequent presentation, his vitals were within normal limits. Exam was notable for left-sided lip and cheek swelling without mass or lesion. Bloodwork was notable for an ESR of 19 and CRP of 7.5. Lip biopsy showed normal squamous epithelium and non-caseating granulomas, consistent with OFG. Patient was treated for a Crohn’s flare with oral prednisone as a bridge to 6-MP. His OFG was treated with intralesional corticosteroids without relief and has on-going outpatient follow up. His current treatment plan includes further cycles of steroid injections before considering alternative therapies. His most recent colonoscopy showed endoscopic and histologic remission of his luminal Crohn’s while on infliximab. (Figure) Discussion: OFG is frequently seen as a manifestation of a systemic condition such as IBD. OFG is diagnosed by biopsy and clinical suspicion. OFG specific treatments include topical steroids and calcineurin inhibitors. Recent data suggests that a cinnamon/benzoate free diet may provide modest benefit to patients. Adequate treatment of underlying IBD is equally important. This patient has the classic symptoms and histopathologic findings of OFG and likely had undiagnosed OFG for many years given his tonsillar pathology years prior. Despite treatment, this patient has persistent and refractory symptoms. This case highlights a classic presentation of a rare IBD extra-intestinal manifestation and demonstrates how EIMs can occur even with well controlled IBD.Figure 1.: Orofacial Granulomatosis: Lip and Cheek Swelling.

S1055 Crohn’s Disease Patients Treated With Anti-TNFs Have Lower Rates of Myocarditis

Introduction: Myocarditis is a rare extraintestinal cardiac manifestation of IBD. It is more commonly reported in Ulcerative Colitis than in Crohn's disease (CD) and consequently not frequently reported. Moreover, the prevalence of myocarditis in CD patients on immunosuppressive and immunomodulatory therapy is not well known. Our study aims to assess the prevalence of myocarditis and the effect of immune-modifying therapy in CD. Methods: In multi-institutional database (Explorys Inc, Cleveland, OH) which includes electronic health record data from 26 major integrated US healthcare systems. Based on the Systematized Nomenclature of Medicine – Clinical Terms (SNOMED-CT), we identified all patients (age >18 years) with a concomitant diagnosis of myocarditis and CD between 1999 to 2022. We investigated the prevalence of myocarditis in CD compared to patients with no IBD. Also, we compared the prevalence between CD patients with and without immune-modifying agents. Results: Of the 70, 301,380 individuals in the database from 1999 to present, we identified 249,300 (0.3%) patients with CD, of whom 40,840 (16.4%) patients received anti-TNFs. CD patients were 59.4% females, 76% Caucasian, and 70% in 18-65 years age group. The prevalence of myocarditis was 0.12% for CD compared to 0.04% in individuals without IBD, p< 0.001. Compared to the general population, patients with CD had higher association risk of myocarditis diagnosis [OR: 3.33, p< 0.0001]. Among CD, predictors of having myocarditis included being elderly ( >65 y/o), African American, smokers and has history of type 2 diabetes (P< 0.0001). The prevalence of myocarditis was significantly lower CD patients on anti-TNF agents [OR: 0.08, p < 0.0001], and thiopurines [OR: 0.11, p < 0.0001] whereas methotrexate and 5-aminosalicylates (5-ASA) had higher rates [OR: 2.78, p < 0.0001] and [OR: 1.63, p =0.0001], respectively. No significant effect was noted with Vedolizumab [OR: 1.30, P= 0.4103] (Figure). Conclusion: In this large database, we found a higher risk association between CD and myocarditis. Anti-TNF agents and thiopurines were less likely to be associated with myocarditis, while methotrexate and 5-ASA were more likely to be associated with myocarditis in CD. The difference in the association between immune-modifying agents is unclear. Hence, further prospective studies are required to evaluate this association. Our findings may have significant implications for patients with cardiac risk factors and underlying myocardial dysfunction.Figure 1.: Overall Risk and Predictors of Myocarditis Among Crohn's disease. Univariate analysis used to calculate OR. OR; odds ratio, CI confidence interval. Anti-TNF; anti-tumor necrosis factor. 5-ASA; mesalamine.