Rare Extraintestinal Manifestation Research Articles

2102 Crohn’s Disease With Rare Extra-Intestinal Manifestation and Unusual Great Outcome!!

INTRODUCTION: Crohn’s disease (CD) is a chronic relapsing inflammatory disorder that most commonly affects the small and large bowel. Prolonged diarrhea with abdominal pain, with or without gross bleeding, fatigue, and weight loss are the hallmarks of Crohn’s disease. Extraintestinal manifestations, such as joints, eye and skin disorders, biliary tract involvement, and kidney stones, may occur. Tonsillar involvement in CD has been very rarely described in the literature. CD is diagnosed with endoscopic and imaging studies of the bowel in a patient with a compatible clinical history. We present a 29-year-old female patient who had a rare extra-intentional manifestation of Crohn's disease which is granulomatous tonsillitis and had an unexpectedly favorable outcome post-tonsillectomy being off treatment for 9 years now without any complaints. CASE DESCRIPTION/METHODS: A 29-year-old with no past medical history presented with frequent episodes of tonsillitis followed by prolonged diarrhea with abdominal pain. Physical exam showed enlarged tonsils and generalized lower abdominal tenderness. Labs showed elevated C-reactive protein, Sedimentation rate, and fecal calprotectin. The patient underwent colonoscopy that showed scattered ileocolitis and biopsy results were compatible with Crohn’s disease. Patient’s tonsillitis failed multiple courses of antibiotic therapy. Accordingly, tonsillectomy was performed, and biopsy showed granulomatous lesion consistent with Crohn’s disease. The patient has been off treatment and never required maintenance therapy for 9 years now without any complaints. DISCUSSION: The first case of CD with granulomatous tonsillitis was reported in 1984. CD patients with an oropharyngeal manifestation of their disease are rare, with an estimation of only 0.5%–20% of patients, with a higher incidence in younger patients. A systematic review and meta-analysis did by Sun et al. demonstrated that tonsillectomy in healthy individuals is associated with an increased risk of developing Crohn’s Disease. Koutroubakis et al. did a case-control study that also showed the same results. Our case presented with recurrent tonsillar pain initially which is a rare extra-intestinal manifestation and underwent tonsillectomy with biopsy confirming Crohn’s Disease and had a remarkably unusual recovery from all Crohn’s Disease symptoms and never had any complaints in 9 years off medical treatment.

2045 Linear IgA Bullous Dermatosis Is an Unusual Skin Extra-Intestinal Manifestation in IBD

INTRODUCTION: Linear IgA bullous dermatosis (LABD) is a rare extraintestinal manifestation of inflammatory bowel disease (IBD). This autoimmune subepidermal disease presents with bullae, erosions and vesicles. LABD is characterized by the immunopathologic finding of linear IgA deposits in the basement membrane along the dermo epidermal junction. There is an uncommon association of LABD with IBD and PSC. CASE DESCRIPTION/METHODS: A 36-year-old male, with a history of Ulcerative Colitis (UC) presented for initial evaluation. For 6 years, the patient has been treated for UC with combinations of steroids and mesalamines. He always discontinued therapy due to what he perceived were adverse drug reactions: pruritic blisters affecting his chest, abdomen, back, and arms. He describes multiple bloody bowel movements, pain with defecation, and a 12-pound weight loss over 2 months. His most recent colonoscopy revealed pancolitis. He has never been in clinical remission. He was referred to a dermatologist for evaluation of his skin lesions, to determine if the dermatitis reaction was a true adverse drug reaction. Hyperpigmentation on his chest and back were noted and appeared to be due to a resolved bullous dermatosis (Figure 1). Skin biopsies identified Linear IgA bullous dermatosis. Dapsone 100 mg daily resulted in resolution of the rash. He was subsequently treated for UC with infliximab and achieved a deep remission. Persistently elevated alkaline phosphatase was investigated with MRI. This revealed intrahepatic duct dilation, and liver biopsy confirmed comorbid PSC. DISCUSSION: LABD is defined by three criteria: vesicles/bullae, subepidermal vesicles with a neutrophil predominance and the linear pattern of IgA basement membrane deposits. This case identifies a rare extra-intestinal skin manifestation of IBD. The exact pathophysiology of the association between these distinct, yet inter-related diseases, has yet to be elucidated.

2048 A Rare Manifestation of a Not so Rare Disease

INTRODUCTION: Pyostomatitis vegetans (PV) is a rare mucocutaneous disease characterized by miliary pustules and ulcerations affecting mucous membranes. PV is classified as a subtype of pyoderma gangrenosum, which affects oral mucosa. The majority of PV cases are associated with inflammatory bowel disease. We present an interesting and a rare case of PV associated with ulcerative colitis (UC) that was successfully treated with infliximab. CASE DESCRIPTION/METHODS: A 60-year-old female with history of ulcerative proctitis who had been off therapy for one year presented with abdominal pain, bloody diarrhea, painful oral ulcers, and a 25-pound weight loss over one month. She first noticed the oral ulcers two weeks prior to presentation, and they worsened to a point that she could not eat. Physical examination revealed confluent ulcerations of the buccal mucosa and diffuse abdominal tenderness. Blood work showed hemoglobin of 8 gm/dl, albumin of 2.5 gm/dl, ESR of 101 mm/hr, and CRP of 17 mg/L. C diff was negative. Oral lesions were negative for HSV-1 and HSV-2 by PCR. CT-abdomen showed findings consistent with active left sided colitis. The patient was started on PO and rectal mesalamine and IV methylprednisolone. Colonoscopy revealed severe deep ulcers extending from anus to proximal sigmoid colon with spontaneous bleeding and complete loss of vascular pattern (Figure 1a). During EGD large confluent ulcerations on bilateral buccal mucosa were visualized (Figure 2a–c). Biopsy of the oral lesions revealed ulcerated squamous mucosa and underlying skeletal muscle with mixed, predominantly chronic inflammation. Immunostains for HSV and CMV were negative. Due to minimal response to IV steroids, patient was started on infliximab 10 mg/kg, resulting in dramatic improvement of oral lesions and GI symptoms. Azathioprine was added for dual therapy for the first year. Endoscopic and clinical remission of colitis was achieved (Figure 1b) in addition to complete resolution of PV (Figure 2d). Patient continues to be in remission on infliximab monotherapy two years from initial presentation. DISCUSSION: To the best of our knowledge, this is the second reported case of UC with PV successfully treated with infliximab. Our unique case demonstrates a rare extraintestinal manifestation of UC, namely PV, that highlights the importance of early and aggressive anti-TNFα therapy to achieve remission of PV and colitis.

P096 Successful vedolizumab therapy in an elderly patient with ulcerative colitis and refractory immune thrombocytopenic purpura

BACKGROUND: Extraintestinal manifestations (EIM) are common in inflammatory bowel disease (IBD) patients and may occur before or after the diagnosis of IBD. In general, the most common EIM usually affect joints, skin and eyes. However, less often other systems can also be affected. The occurrence of immune thrombocytopenic purpura in IBD patients is rare and poorly described in the literature. CASE: A 61-year-old man was admitted with a history of diarrhea. Admission laboratory tests showed anemia and iron deficiency. There were also mild thrombocytopenia. The patient reported a history of ulcerative colitis (UC) diagnosed 7 years ago (pancolitis). There were several clinical recurrences, with episodes of severe anemia, requiring hemotransfusion in some occasions. He used 5-ASA without improvement. He reported different courses of prednisone 40 mg per day, with clinical remission. He used azathioprine for about 6 months, but there was no clinical remission free of corticosteroids. Colonoscopy showed a suggestive aspect of UC (pancolitis), with severe endoscopic activity (Mayo ES 3). The patient underwent corticosteroid therapy. He presented clinical remission and was discharged in order to initiate biological therapy on an outpatient basis. After discharge, the patient suspended steroid therapy on his own and did not return to the office for follow-up. Two months later, he developed a new UC relapse and was hospitalized again. Laboratory tests showed a significant worsening of thrombocytopenia (5,000 per mm3 platelets). Prednisone was performed again. The patient achieved clinical remission but maintained severe thrombocytopenia. The diagnosis of Immune thrombocytopenic purpura (ITP) refractory to corticosteroids was considered. Then, the patient underwent different immunoglobulin infusion sessions. He presented a temporary improvement in platelet count (20,000 to 50,000 per mm3), but in a few weeks he returned to severe thrombocytopenia (< 5,000 per mm3). Patient was then treated with rituximab. After the third dose, he developed septic shock. It improved after meropenem use. Due to the severe infectious episode, rituximab therapy was discontinued and romiplostim treatment was initiated. There was partial improvement of the thrombocytopenia and the patient maintained the weekly use of the medication. In August 2017, the patient started vedolizumab. After 14 weeks he evolved with clinical remission free of corticosteroids. The patient remained in clinical remission without corticosteroids in the first 12 months of treatment with vedolizumab. Colonoscopy performed 11 months after the beginning of the biological therapy showed mucosal healing (Mayo ES 1). He was maintained throughout this period with progressive improvement of the platelet count, with weekly doses of romiplostim. After the eleventh month of treatment with vedolizumab, there was a reduction in the dose of romiplostim due to improved thrombocytopenia (>200,000 per mm3). DISCUSSION: Physicians should be alert to the occurrence of severe thrombocytopenia in a IBD patient. ITP is considered a rare extraintestinal manifestation and appears to be related to the activity of IBD. In the case reported, clinical and endoscopic remission induced by vedolizumab seems to have been a decisive factor for the gradual improvement of thrombocytopenia, including the partial weaning of romiplostim.

P100 INCREASING RATES OF INFECTIVE ENDOCARDITIS IN PATIENTS WITH INFLAMMATORY BOWEL DISEASE

Infective endocarditis (IE) is a rare but reported extraintestinal manifestation of inflammatory bowel disease (IBD). In addition to several case reports, there are small level retrospective studies that have suggested that IBD may be an independent risk factor for the development of IE. Patients with IBD are often immunosuppressed and undergo multiple procedures that render them susceptible to bacteremia and, subsequently, endocarditis. Despite this, there is a lack of evidence regarding the association of IE and IBD and no estimates of disease burden. We aim to estimate the burden of infective endocarditis in patients with inflammatory bowel disease using a national level database. We queried the National Inpatient Sample (NIS) for all hospitalizations of patients with a primary or secondary diagnosis of IBD [Crohn’s disease (CD) or ulcerative colitis (UC)] with or without a diagnosis of infective endocarditis using the International Classification of Diseases Ninth Edition Clinical Modification (ICD-9 CM) codes from 2003-2014. We then conducted a retrospective cohort study and performed statistical analysis using SPSS (version 25.0). Trend analysis was tested using Mann-Kendall trend test and associations were evaluated using multivariate logistic regression. From 2003-2014, there were a total of 5215 hospitalizations (3165 in CD and 2050 in UC) for IE in patients with IBD. The patients were primarily Caucasians (69.3% in CD and 70.8% in UC) with an age greater than 61 (49.6 % in CD and 60.1% in UC). In patients with CD and IE, they were primarily female (56.5%) while those with UC and IE were primarily males (52.2%). The rate of IE in IBD was found to be significantly rising (12.9 cases per 10000 hospitalizations in 2003 to 20.5 in 2014; p <0.05). Multivariate logistic regression showed age (aOR 1.02; CI 1.02-1.03; p<0.0001) and female gender (aOR 1.15; CI 1.03-1.28; p<0.01) to be a significant contributor to the presence of IE while comorbid hepatitis C, HIV, drug use, dialysis use, congenital or pre-existing cardiac abnormalities and the presence of cardiac devices were noted to be non-significant. The initial results of our study demonstrate that there is a rising rate in the number of hospitalizations for infective endocarditis in patients with IBD in the United States. The lack of an association with various comorbidities that are typically associated with IE suggest that such a rise in IBD patients is potentially related to disease flares itself. While it remains a rare extraintestinal manifestation, providers must continue to be aware of this potential complication especially given the increasing use immunosuppressant agents. Table 1. Demographics of IBD patients with and without infective endocarditis. Figure 1. Trend of infective endocarditis incidence in IBD hospitalizations from 2003-2014.

A Rare and Unusual Case of Healing Pyoderma Gangrenosum in Uncontrolled Crohnsʼ Disease

Pyoderma gangrenosum (PG) is a rare extraintestinal manifestation of inflammatory bowel disease (IBD). A literature review suggests that 6 to 40% of patients with IBD will experience extraintestinal manifestations. Respectively, Erythema nodosum and pyoderma gangrenosum are the first and second extraintestinal manifestations of IBD. PG is seen in an estimated 5% of Ulcerative Colitis patients and even less in Crohn's disease. PG is the more rare extraintestinal manifestation for Crohn's disease. Although PG is a neutrophilic dermatosis, the cause of PG remains not well understood due to its low incidence. The general axiom for these cutaneous manifestations is to control the inflammatory disease flare in order to obtain resolution of this extraintestinal manifestation. For our case report, we present a 39-year-old African American male inmate who presented with a year of intermittent uncontrolled Crohn's disease with healing Pyoderma gangrenosum. Prior to his incarceration the patient well controlled with a mesalamine regimen without any symptoms of abdominal pain or bloody diarrhea. After incarceration, he became noncompliant with therapy and developed intermittent flares. Patient's physical exam consisted of gross bloody diarrhea associated with a 30-40 pound weight loss over two years and intermittent abdominal pain, most severe in his epigastric region. Patient also presented with healing maculopapular lesions circumferentially extending from his knees to feet. Literature review suggests that pyoderma gangrenosum resolves when the underlying condition, in this case Crohn's disease, is managed. However, we present this rare and unique case due to the patient's pyoderma gangranosum presenting in a healing phase amidst uncontrolled Crohn's disease. There has only been one other reported case study where a patient presented with intractable PG amid controlled Crohn's disease activity. These two cases raise more questions about the molecular mechanism that drive the disease activity. Futhermore, it underscores potential weaknesses to the axiom that disease activity and extraintestinal manifestations run parallel to each other prompting further need for research to understand the molecular drivers of inflammatory bowel diseases.2060 Figure 1 No Caption available.

Thrombotic Cutaneous Gangrene: A Rare Dermatologic and Hematologic Extraintestinal Manifestation of Ulcerative Colitis

Ulcerative colitis (UC) is associated with many extraintestinal manifestations, which can include dermatologic and hematologic involvement. Thrombotic cutaneous gangrene (TCG) is a rare dermatologic and hematologic complication with potentially poor prognosis, often needing colectomy in addition to anti-coagulation for complete resolution. A 33-year-old healthy female presented with chronic bloody diarrhea and was diagnosed with left sided UC. She was initiated on steroids and discharged on a prednisone taper. Three days after discharge, she presented with fever and progressive and extensive racemose and retiform purpura on her trunk and extremities. She continued to have bloody stool despite adherence to prednisone. Areas of purpura evolved to tense bullae which erupted. She had an elevated D-dimer, prolonged PT, and reduced anti-thrombin III activity. Complete antiphospholipid syndrome panel was negative. A skin punch biopsy showed thrombotic vasculopathy involving small dermal vasculature without vasculitis. No other end-organ complications of hypercoagulability were identified. MRE showed inflammatory changes limited to the descending colon, sigmoid colon, and rectum, with no involvement of the small bowel. Anti-thrombin III levels were replenished, high dose heparin drip was started, and her hypercoagulable state resolved. The patient's UC was refractory to steroids; she was subsequently started on Infliximab, which controlled her luminal symptoms. She was discharged on Lovenox, which significantly improved her skin manifestations. Our case underlines the importance of appreciating a hypercoagulable state in UC and suspecting TCG in UC cases presenting with rapidly progressing purpura. Immediate hypercoagulability work up, skin biopsy, and anticoagulation should be initiated to prevent further complications. Furthermore, we demonstrate that trial of biologic immunosuppressive therapy may prevent need for colectomy to treat TCG.2098_A Figure 1. Retiform purpura on shoulder and back2098_B Figure 2. Areas of purpura evolved to become tense bullae which eventually erupted.2098_C Figure 3. Mayo 3 UC

Crohn's disease of the vulva: A prospective study.

Vulval Crohn's disease (VCD) is a rare extra-intestinal cutaneous manifestation of Crohn's disease. VCD is often unrecognized and misdiagnosed and can be difficult to treat. The aim of the study was to describe the clinical presentation, associated features, and response to treatment modalities in patients with VCD. A prospective review of patients with VCD who were referred to our specialist dermatology clinic from 2003 to 2017 was performed. Data on age at diagnosis, presenting signs, associated features, and response to treatment were collected. Thirty-one patients with a median age of 40years (range 13-66years) were identified. VCD manifested most frequently with vulval edema (77%), ulceration (35%), and fissures (39%). Eighty-one percent had active gastrointestinal disease. Oral involvement and pyoderma gangrenosum were present in 13% and 19%, respectively. Topical therapy was effective in mild VCD. Tumor necrosis factor-α inhibitors were the most effective second-line therapy, with 53% achieving complete clinical remission. Additionally, we have found compression garments and shorts to be useful adjuncts in treating vulval edema. Vulval Crohn's disease is a diagnostic and therapeutic challenge. We recommend consideration of tumor necrosis factor-α inhibitors at an early stage.

A case of ulcerative colitis presenting with cerebral venous thrombosis.

Patients with inflammatory bowel disease (IBD) have been reported to have an increased risk of thromboembolism. Cerebral venous thrombosis (CVT) is a rare but serious extraintestinal manifestation of IBD. Due to its highly variable manifestation and low incidence, CVT is not usually readily recognized by physicians. Herein, we report a case of a 35-year-old male presenting with CVT associated with ulcerative colitis (UC). The patient was admitted with chief complaints of bloody diarrhea that had started 3 days prior. Sigmoidoscopy showed hyperemic and edematous mucosa, friability, and shallow ulcers from the sigmoid colon to the rectum suggestive of IBD. Three days later, the patient started complaining of a headache, and gradually developed a decreased level of consciousness. Magnetic resonance imaging of the brain revealed CVT with hemorrhagic infarctions. An angiogram was obtained to evaluate the extent of CVT, and anticoagulation therapy was initiated with intravenous heparin. During hospitalization, he was diagnosed with UC and treated with 5-aminosalicylic acid. After discharge, the patient was recovered without neurological deficit, and remission of UC was also obtained. The presence of headache or acute worsening of neurological status in a patient with IBD should alert the health professionals about the possibility of CVT.

Incidentally detected lung lesions in a patient with crohn's disease.

THE CASE A 57-year-old man, nonsmoker, recently diagnosed as having Crohn's disease was referred to us for evaluation of an incidentally detected radiological lesion. As part of the workup for Crohn's disease, a computed tomography (CT) scan of the abdomen was taken, which revealed a few infiltrates in the visualized portion of both lower lobes. He denied having any significant respiratory symptoms apart from an infrequent dry cough. Clinical examination of the respiratory system revealed the presence of random, inspiratory crackles at both lung bases. A high-resolution CT (HRCT) scan was taken [Figure 1].Figure 1: Name the radiological sign seen in this image? What is the most likely diagnosis?QUESTIONS Question 1: Name the radiological sign seen in this image? Question 2: What is the most likely diagnosis? ANSWERS The (Reverse Halo sign) Cryptogenic organizing pneumonia. DISCUSSION The “Reverse halo sign” (also referred to as “reversed halo sign”) is characterized by the presence of a central ground-glass opacity surrounded by a ring [Figure 2, white arrow] or crescent [Figure 3, white arrow] of dense airspace consolidation. It is also called atoll sign since it resembles a coral atoll. Although it was first described in cryptogenic organizing pneumonia (COP) and was thought to be diagnostic of the condition, it has been subsequently described in a variety of other conditions including fungal infections and adenocarcinoma of the lungs. But still, COP remains the most common condition in immunocompetent patients where the reverse halo sign is seen.[1] Table 1 lists the various conditions in which reverse halo sign is seen.[2]Figure 2: Axial section of high-resolution computed tomography scan of the thorax showing ground glass density surrounded by a ring of consolidation, in the superior segment of left lower lobe (white arrow)Figure 3: Axial section of high-resolution computed tomography scan of the thorax showing ground glass density surrounded by a crescent of consolidation, in the anterior segment of left upper lobe (white arrow)Table 1: Differential diagnosis of the reverse halo signCorrelating the clinical details with the CT findings will help to narrow down the diagnosis.[2] For instance presence of this sign in a patient detected to have HIV is suggestive of Pneumocystis jirovecii infection, whereas the same in a patient who has undergone a solid organ transplant would mean pulmonary zygomycosis. If there are coexisting tree-in-bud lesions and centrilobular nodules, it points toward a diagnosis of tuberculosis. Its presence in a patient with Crohn's disease, as in our case would narrow down the differential diagnosis to COP. Lung involvement is uncommon in Crohn's disease. It could either be due to the disease or sometimes due to treatment with drugs such as sulfasalazine and methotrexate. Pulmonary involvement could be upper airway involvement like glottic or subglottic stenosis, and tracheal stenosis, or small airway diseases like COP and diffuse panbronchiolitis or lung parenchymal involvement like interstitial lung disease or necrobiotic nodules.[3] COP is a rare extraintestinal manifestation of Crohn's disease. This disease, which used to be previously referred to as bronchiolitis obliterans organizing pneumonia, is a subacute process characterized by the presence of granulation tissue in the bronchioles and alveolar ducts with extension into the alveoli. The “shared antigen theory” has been postulated to explain the occurrence of organizing pneumonia in Crohn's disease.[4] Patients commonly present with fever, cough, or chest pain. HRCT is the investigation of choice in the evaluation of diffuse lung diseases. The common findings in CT are ground glass opacities and parenchymal consolidation. Fleeting consolidation and the reverse halo sign are sometimes present, which are all the most suggestive of the diagnosis. Since COP is always a diagnosis of exclusion, a flexible bronchoscopy, and lavage to exclude infections is mandatory. Sometimes, surgical lung biopsy is needed to confirm the diagnosis. This case is being presented to highlight the radiological finding of the “Reverse halo sign.” A knowledge of the various etiologies where it occurs would give a clue to make the appropriate diagnosis, avoid unwanted investigations, and institute timely management. As illustrated in our case, the presence of this radiological sign in patients with Crohn's disease should make one think about the possibility of COP. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

Gut-Lung Connection: Pulmonary Necrobiosis in Inflammatory Bowel Disease

SESSION TITLE: Pulmonary Manifestations of Systemic Disease 1 SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 31, 2017 at 01:30 PM - 02:30 PM INTRODUCTION: Inflammatory bowel disease (IBD) is an idiopathic disease that causes inflammation of the gastrointestinal tract and includes ulcerative colitis and Crohn’s disease. Overt respiratory manifestations of IBD are unusual, but bronchiectasis, organizing pneumonia, and eosinophilic pneumonia have been reported. Cavitary lung nodules are rarely seen in IBD. In this report, we present a case of Crohn’s disease-associated pulmonary necrobiosis. CASE PRESENTATION: A 67-year-old woman with Crohn’s disease on adalimumab presented with four weeks of cough, hemoptysis, and dyspnea. Computed tomography (CT) of the chest revealed nodular cavities with consolidation (Fig. 1). Laboratory evaluation for Cryptococcus, Aspergillus, Coccidioides, Histoplasma, and Mycobacterium tuberculosis was unrevealing. Autoimmune serologies (antinuclear antibody, anti-neutrophil cytoplasmic antibodies, rheumatoid factor, and SSA/SSB) were negative. A bronchoscopy with bronchoalveolar lavage was performed with negative bacterial, fungal and mycobacterial cultures. An initial CT-guided biopsy did not reveal any organisms or malignant cells. Video-assisted thoracoscopic surgery (VATS) biopsy of the right middle and lower lobes was performed, with pathology showing airway-centered inflammation with secondary granulomas, abscess formation, and necrosis (Fig. 2), consistent with a diagnosis of pulmonary necrobiosis. She was started on infliximab, which caused an infusion reaction. She was then placed on prednisone and mycophenolate mofetil, with resolution of the cavitary lesions. DISCUSSION: Extra-intestinal manifestations of IBD are not commonly seen in the lung, and pulmonary necrobiosis has only been described in isolated case reports. Necrobiotic lung nodules are sterile nodules that can cavitate and histologically are composed of abscesses filled with necrotic debris and inflammatory cells. The association between bowel disease activity and the lung lesions has been controversial in the literature, as the nodules have been reported to precede, be coincident with, or follow the diagnosis of IBD. Pulmonary necrobiosis responds well to corticosteroids though the duration of treatment remains unknown. CONCLUSIONS: There should be a high index of suspicion for the development of pulmonary disease in the setting of IBD, and IBD should be considered in the differential of cavitary lung nodules. Reference #1: El-Kersh K., et al. Pulmonary necrobiotic nodules in Crohn’s disease: a rare extra-intestinal manifestation. Respir Care 2014;59(12):e190-e192. DISCLOSURE: The following authors have nothing to disclose: Nancy Hsu, Yusaku Shino No Product/Research Disclosure Information

Inflamed Bowels, Now Inflamed Lungs: A Case Report of Pulmonary Necrobiotic Nodules in Crohn's Disease

SESSION TITLE: Pulmonary Manifestations of Systemic Disease 3 SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 31, 2017 at 01:30 PM - 02:30 PM INTRODUCTION: Pulmonary necrobiotic nodules have been described as a rare extraintestinal manifestation of Crohn’s disease. We report imaging and pathological findings of this rare disease entity. CASE PRESENTATION: A 21-year-old gentleman with a longstanding history of Crohn’s disease presented with 2 weeks of right-sided pleuritic chest pain, associated with a dry cough. He did not report fevers, night sweats, abdominal pain or diarrhea. The patient had achieved remission of his Crohn's disease for 11 years with adalimumab, which was discontinued 2 months prior to presentation due to concerns of developing secondary lymphomas. He was then transitioned to mesalamine, after which he experienced a self-limited episode of Crohn’s disease exacerbation. On presentation, he was hemodynamically stable. Physical examination was notable for right-sided chest tenderness. Laboratory investigations showed an elevated erythrocyte sedimentation rate of 33 mm/hr and D-dimer of 535 µg/mL. A computed tomography (CT) scan of the chest showed multiple nodular appearing opacities bilaterally (figure 1). Lung biopsy revealed areas of parenchymal necrosis with necrotizing granulomas. The necrosis was basophilic, which contained nuclear debris and eosinophils with the surrounding inflammatory infiltrates (figure 2). Gram stain, acid-fast bacilli stain, fungal stain and tissue culture were all negative. Angiotensin-converting enzyme level was normal. Cytoplasmic antineutrophil antibodies titer was 1:40, myeloperoxidase and proteinase-3 antibodies were not detected. The differential diagnosis was primary vasculitis versus Crohn’s disease associated necrobiotic nodules. The lack of systemic manifestations lowered the suspicion for active vasculitis. The patient had an uneventful recovery. Mesalamine was changed to vedolizumab. Repeat chest CT scan 2 months later showed marked reduction in size and number of the pulmonary nodules. 5 months after presentation chest CT showed complete resolution of the previously noted pulmonary nodules. DISCUSSION: Our case demonstrates a rare pulmonary complication related to Crohn’s disease exacerbation, which is the development of pulmonary necrobiotic nodules. We suspect that this was the consequence of therapy de-escalation. Our hypothesis is supported by the complete resolution of pulmonary nodules following therapy re-escalation. CONCLUSIONS: Even after achieving Crohn's disease remission for a long period of time, therapy de-escalation still carries the risk of triggering unexpected pulmonary manifestations. Physicians should consider risks versus benefits of alternate therapy. In clinical practice, it is important to maintain a broad differential diagnosis, especially in patients with conditions affecting multi-organ systems. Reference #1: Warwick, G., Leecy, T., Silverstone, E., Rainer, S., Feller, R., & Yates, D. H. (2009). Pulmonary necrobiotic nodules: a rare extraintestinal manifestation of Crohn's disease. European Respiratory Review, 18(111), 47-50. DISCLOSURE: The following authors have nothing to disclose: Mahmoud Ahmed, Laurie Lerner No Product/Research Disclosure Information

Genital Granulomatosis in Male and Female Patients With Crohn's Disease: Clinical Presentation and Treatment Outcomes.

Genital granulomatosis [GG] is a metastatic form of Crohn's disease [CD], characterised by granulomatous inflammation of the genital skin without contact with the gastrointestinal tract. Little is known about GG, as most publications are case reports or small series, and only sporadic in male cases. Cases of GG were retrospectively collected through the Collaborative Network For Exceptionally Rare case reports project of the European Crohn's and Colitis Organisation. A total of 43 patients [9 males, 34 females] were diagnosed as having GG, mostly as oedema and/or ulcers. Histological confirmation of granulomas was obtained in 70% of the cases. CD location was colonic or ileocolonic in 97% and perianal disease was documented in 57%. There was no significant difference between males and females in CD phenotype or genital lesions. GG was the first manifestation of inflammatory bowel disease [IBD] in one-third of the patients; these patients were younger at the time of GG occurrence and they all were non-smokers. GG occurred in the absence of gastrointestinal disease activity in 30% of the cases. Ten out of 11 patients [91%] responded to systemic corticosteroid treatment, 5/9 patients responded to immunomodulators, and 9/11 patients responded to anti-tumour necrosis factor alpha [TNF-α] agents. GG is a rare extraintestinal manifestation of CD. It mainly occurs among women, in the setting of colonic involvement of CD, and perianal disease is often associated. Most cases are successfully managed with systemic corticosteroids or anti-TNF agents.

Clinical experience and diagnostic algorithm of vulval Crohn's disease.

Vulval Crohn's disease (VCD) is a rare extraintestinal cutaneous manifestation of Crohn's disease. No consensus on the diagnostic workup and therapeutic management of this condition has been provided in the current literature. Retrospective, multicentre descriptive case series of female patients diagnosed and treated with VCD. By chart review, data on initial symptoms, clinical courses, histologic findings and therapeutic management were collected. Fifteen female patients with a median age of 28 years (interquartile range: 28-44 years) suffering from Crohn's disease of the ileum (27%), colon (33%) and ileocolon (40%) were included. VCD manifested most frequently with vulval swelling (93%), pain (80%) and erythema (73%). Histologic analysis demonstrated granulomatous inflammation in 78% and a mixed inflammatory cell infiltrate in 67% of cases. In eight (53%) cases, topical therapy resulted in temporary reduction of vulval symptoms. Combotreatment with immunosuppressive agents and tumour necrosis factor α inhibitors was the most effective second-line therapy: five (33%) patients achieved sustained clinical remission with this therapeutic strategy. The diagnostic workup of VCD is challenging and should be approached in a multidisciplinary manner. Histopathologic analysis of the vulva supports the diagnosis. Topical therapy and systemic treatment with immunosuppressive agents and tumour necrosis factor α inhibitors are advised to treat this condition.

HEMATOLOGIC MANIFESTATIONS OF CROHN'S DISEASE: TWO CLINICAL CASES

Inflammatory bowel diseases (IBD) are commonly associated with extraintestinal manifestations, hematological disorders being the most special among them. In some cases, they dominate the clinical picture masking the intestinal manifestations of the underlying disease. Aplastic anemia is an extremely rare extraintestinal IBD manifestation. There are only two clinical cases of aplastic anemia associated with ulcerative colitis and non with Crohn's disease reported in the literature. Combination of Crohn's disease and В₁₂-deficient anemia is more prevalent, but is seen usually only after more than 20 cm of the ileus has been resected. The first clinical case presented in this paper is a combination of severe fistula-forming Crohn's disease with a constriction in the terminal part of the ileus and profound pancytopenia as an outcome of aplastic anemia. This profound pancytopenia is associated with an extremely high risk of life-threatening complications both of surgical treatment, as well as of several chemotherapeutic agents, which made the management of this patient difficult. The second clinical case demonstrates the manifestation of Crohn's disease as ileocolitis starting from the symptoms of cobalamin deficiency: severe В₁₂-deficient anemia, funicular myelosis and sensory ataxia, with blunted intestinal symptoms. This made the initial diagnosis and timely treatment difficult. Replacement therapy with cobalamin injections and treatment with glucocorticoids and antibacterials led to endoscopically confirmed remission of Crohn's disease and normalization of hematological parameters, with persistent polyneuropathy. Thus, management of patients with Crohn's disease should be multidisciplinary. In the case of anemia, leucopenia and/or thrombocytopenia in IBD patients it is necessary to exclude potential myelodysplasia and bone marrow aplasia. In the event of megaloblastic anemia and/or progressive polyneuropathy one should bear in mind potential cobalamin deficiency. However, in severe anemia it is important to perform diagnostic assessment for IBD, first of all, for Crohn's disease, especially, if any intestinal symptoms are present.

Organizing Pneumonia: A Rare Extra Intestinal Manifestation of Ulcerative Colitis

Respiratory involvement of ulcerative colitis is exceptional. The prevalence is estimated to 0.2%. It may develop during the course of the disease particularly after colectomy, but can also occur without any correlation with the activity of the inflammatory bowel disease and can affect numerous organs most notably lungs. Chronic bronchitis and bronchiectasis are the main respiratory manifestations. Lung disease involving pleural and parenchymal damage is relatively uncommon among patients with inflammatory bowel disease. We report the case of an organizing pneumonia associated to a pleural effusion as an extra intestinal manifestation of ulcerative colitis in a 28-year-old man with a longstanding quiescent history of an ulcerative colitis. He was successfully treated with steroids without relapse during the follow-up period.

La gangrène cutanée thrombotique : une manifestation extradigestive rare de la rectocolite hémorragique

Thrombotic cutaneous gangrene is a rare extra-intestinal manifestation of ulcerative colitis with a severe prognosis. A 35-year-old woman with a 7-year history of ulcerative colitis presented with extensive ecchymotic lesions that began a few hours earlier. On examination, she was febrile with multiple necrotic lesions. Skin biopsy showed multiple microthrombi in the dermal vessels. A diagnosis of thrombotic cutaneous gangrene was established. The patient was treated with heparin and systemic corticosteroids. The majority of cutaneous lesions showed improvement after 1month. Thrombophlebitis of the left lower limb occurred subsequently. Thrombotic cutaneous gangrene is attributed to microvascular thrombosis, which arises from the hypercoagulability observed in ulcerative colitis. Complete blood and coagulation tests must be performed and early anticoagulation with heparin must be considered in order to prevent the progression of cutaneous infarction.

Use of Infliximab for the Treatment of Sweetʼs Syndrome in a Patient with Inflammatory Bowel Disease

INTRODUCTION: Sweet's Syndrome is characterized by fever, neutrophilia, painful erythematous skin lesions (papules, nodules, plaques) and a diffuse neutrophilic infiltrate in the upper dermis of the skin. It can be idiopathic, malignancy-associated or drug-induced. CASE REPORT: A 47 year old female with Ulcerative Colitis presented with 3 days of nausea, vomiting and diarrhea. She was receiving Azathioprine 100mg daily for 3 years. Laboratory studies were significant for WBC 18.4 (89% neutrophils), ESR 20, CRP 14. Stool cultures and clostridium difficile toxin were negative. CT scan showed diffuse colonic, gastric antral and duodenal wall thickening. Upper endoscopy revealed diffuse gastric erythema, and shallow duodenal ulcerations (pathology showing acute and chronic duodenitis). Flexible sigmoidoscopy showed confluent ulcerations and mucus exudates in the recto-sigmoid colon (pathology showing chronic active colitis). 3 days after admission, she developed high grade fevers (1020 F), and new erythematous tender papules on the torso and extremities. Biopsy of the papules showed interstitial neutrophilic infiltrate in the upper dermis consistent with Sweet's Syndrome. She declined steroid therapy due to a prior adverse reaction of elevated intraocular pressures, but agreed to treatment with Infliximab at a dose of 5mg/kg. Her skin lesions and fever resolved within 2 days, and she had improvement of her gastrointestinal complaints. She was discharged, continued on outpatient Infliximab therapy and is currently in clinical remission. DISCUSSION: We report a case of Sweet's Syndrome in a patient with inflammatory bowel disease (IBD) successfully treated with Infliximab. Sweet's Syndrome is a rare extra-intestinal manifestation of IBD, presenting independent of its activity and should be considered in the differential diagnosis of a patient with IBD who develops skin lesions. The skin manifestations can last for weeks to months without treatment. Systemic corticosteroids are the gold standard therapy. The success of Infliximab in our patient's treatment supports the role of tumor necrosis factor (TNF) in the pathogenesis of Sweet's Syndrome. Few case reports have shown the treatment of Sweet's Syndrome associated with IBD with infliximab at doses of 5mg/kg and 10mg/kg. Our case suggests that Infliximab and other anti-TNF agents can be successfully used as a second line therapy for Sweet's Syndrome in those with IBD.Figure 1Figure 2

CT and MRI of Rare Extraintestinal Manifestations of Inflammatory Bowel Disease in Children and Adolescents.

Inflammatory bowel disease (IBD) is associated with a spectrum of extraintestinal manifestations (EIMs) affecting many organ systems. EIMs can occur in more than 40% of patients with IBD and are associated with significant morbidity. They occur at any time point in the course of disease, often during an active phase of bowel inflammation, but sometimes preceding bowel disease. Prompt recognition of EIMs enables timely and more effective therapy. Physicians who image patients with IBD should be aware of the myriad extraintestinal conditions that may be detected on imaging studies, both within and outside of the abdomen, as they may predate the diagnosis of IBD. Cross-sectional imaging of unusual conditions associated with IBD will be presented, including pathology in the hepatobiliary, pancreatic, genitourinary, musculoskeletal, mucocutaneous, vascular, neurologic, and pulmonary systems.

Crohn's colitis-induced myocarditis

Myocarditis can be idiopathic or arise in response to numerous systemic insults. Myocarditis occurring in the setting of an exacerbation of inflammatory bowel disease is a rare extra-intestinal manifestation of both ulcerative and Crohn's-related colitis. Here, we present a unique case of a 56-year-old female patient presenting with an acute Crohn's colitis flare that was eventually complicated by myocarditis. Our case is unique in that we clearly delineate the clinical course and development of myocarditis in a patient with focal myocardial inflammation in a pattern that is atypical for myocarditis.<Learning objective: To illustrate the clinical course and resolution, along with cardiac magnetic resonance imaging and echocardiographic findings, of Crohn's-induced myocarditis.>