What to Know: Seven Extraintestinal Manifestations of Inflammatory Bowel Disease

Author: Li Junchen

This article is published with the author’s authorization, please do not authorize it reproduced.

Introduction

Inflammatory bowel disease (IBD) is an idiopathic intestinal inflammatory disease, including ulcerative colitis (UC) and Crohn’s disease , CD), characterized by chronic, recurrent, and unknown etiology. In addition to abdominal pain, diarrhea, tenesmus, mucus, pus and blood in the stool and other intestinal manifestations, other systemic primary lesions caused by IBD are called extraintestinal manifestations (EIMs) of IBD, and the incidence rate is as high as 47%. 3 patients occurred before the diagnosis of IBD, mainly involving the skin, oral cavity, musculoskeletal system, eyes, liver and gallbladder, blood and other systems, which had a great impact on the health and quality of life of patients.

This article provides an overview of the seven most common extraintestinal manifestations of IBD.

noduleserythema nodosum (EN)

EN is the most common cutaneous manifestation of IBD, The incidence can reach 15% in CD and about 10% in UC. The typical skin manifestations of EN are symmetric, raised and soft, purple or red subcutaneous nodules, 1-10 cm in diameter, variable in number, which are more common on the extensor sides of the lower extremities, especially the anterior tibia. The acute phase may be accompanied by systemic symptoms such as fever, fatigue, and joint pain. EN is characterized by vasculitis-like manifestations on pathology, mainly neutrophilic infiltration in the early stage, and increased lymphocytes in the late stage, often accompanied by vascular endothelial cell hyperplasia, Lumen occlusion and thrombosis generally do not occur. Because EN is not only closely related to genetics, but also to disease activity, treatment is mainly for IBD, requiring systemic corticosteroids.

pyoderma gangrenosum (PG) strong>

PG is the most severe cutaneous manifestation of IBD, and its symptoms can even exceed the impact of IBD on patients. The initial stage of PG is mostly painful, single or multiple erythema, papules, vesicles, blood blisters or pustules, which can merge with each other to form purple-red plaques, and then rapidly progress to deep, erosive and suppurative skin lesions with basal Infiltrate with undermined ulcer borders. It can occur anywhere on the body, including the external genitalia, but is more common in the pretibial and perioral regions. The histopathology of PG is nonspecific, but can be used to rule out other specific skin disorders. Due to the progressive deterioration of PG, its therapeutic goal is to make it heal quickly, and the treatment method is mainly immunosuppressive.

Oral Injury

Oral injuries include periodontitis, aphthous stomatitis and proliferative suppurative stomatitis (PV), where PV is oral injury the most serious type. Periodontitis is mainly manifested as gingival erythema, bleeding, swelling, and in severe cases, tooth loss.

Aphthous stomatitis is the most common, with multiple round or oval painful Ulcers, with yellow base and red borders, mostly occur in the oral cavity, lips, and mucous membranes. PV is more common in patients with UC. It begins as multiple yellow-white brittle small pustules, and then develops into proliferative, erosive, and hemorrhagic ulcers. It looks like a “snail trail” and often involves the lips, buccal mucosa, gums, soft and hard palate and Tonsils etc. Controlling IBD is the key to its treatment, as it usually occurs during the active phase of IBD and resolves with the remission of IBD symptoms.

Bone and joint lesions strong>

including peripheral and central types, peripheral arthritis is more common, often involving a single large joint, such as knee, ankle, wrist , Elbow joint, usually less than 5 joints are involved, mostly acute arthritis, often manifested as local redness, swelling, heat, pain in the affected joint, generally does not cause joint deformity.

Central arthritis refers to ankylosing spondylitis and sacroiliitis. The natural history of spondylitis is not related to IBD. Appearing before or at the same time as colitis, the disease often involves the entire spine, resulting in spinal deformity, stiffness, limited or loss of motion, radiographic examination can find arthritis, ligamentous osteophytes and bone hyperplasia, and finally form a typical “arthritis” Bamboo Spine”.

hepatobiliary lesions

Common hepatobiliary diseases include primary sclerosing cholangitis (PSC), fatty liver, cholelithiasis, pericholangitis, and IgG4-associated cholangitis (IAC), etc. . Among them, PSC is the most common and serious manifestation. In fact, about 70-80% of PSC patients have IBD at the same time, and only 1.4-7.5% of IBD patients develop PSC.

PSC is commonly diagnosed by MRCP, which can show narrowing and dilation of the bile duct. If MRCP is negative, liver biopsy is more effective in diagnosing PSC than diagnostic ERCP. The incidence of non-alcoholic fatty liver disease in IBD accounts for about 8.2%, and fatty liver may be related to intestinal malabsorption, protein loss and liver damage caused by IBD-related drug treatment.

Eye Disorders p>

Scleritis, anterior uveitis and conjunctivitis are common, but keratopathy, retinal vasculitis, choroiditis, iritis, etc. Uveitis and conjunctivitis are the most common. Uveitis affects vision and, in severe cases, can lead to blindness.

Scleritis is often associated with the activity of IBD, with clinical manifestations of scleral and/or conjunctival erythema, photophobia, Burning sensation in the eyes. Glucocorticoids or immunosuppressants are usually effective, but they are not parallel to the activity of enteropathy, and iritis and uveitis can still recur after colectomy.

Cardiovascular System< /p>

Thrombotic disease and anemia are extraintestinal manifestations of the blood system in IBD patients. Thromboembolic disease is a more serious extraintestinal manifestation in IBD patients. It is common in the elderly and those with severe intestinal disease. In severe cases, it can be life-threatening.

The reason for the formation of thrombus is mainly due to vascular endothelial injury and blood hypercoagulability. The most common thromboembolisms are deep venous thrombose (DVT) and pulmonary emboli (PE), but rare sites of embolism, such as cerebrovascular and portal veins, have also been reported. Iron deficiency anemia is the most common anemia in IBD patients. Long-term nutritional deficiency and chronic intestinal blood loss in IBD patients can cause anemia. IBD pro-inflammatory factors can inhibit the production of erythropoietin (EPO) and the stimulation of EPO on erythroid precursor cells. It is also one of the important causes of anemia in IBD patients.

References:1.

KO J S, UBERTI G, NAPEKOSKI K, et al. Cutaneous manifestations in inflammatory bowel disease: A single institutional study of non-neoplastic biopsies over 13 years[J]. J Cutan Pathol, 2016, 43(11): 946-955.

2. MARZANO A V, FANONI D, ANTIGA E, et al. Expression of cytokines, chemokines and other effector molecules in two prototypic autoinflammatory skin diseases, pyoderma gangrenosum and Sweet’s syndrome[J]. Clin Exp Immunol, 2014, 178(1): 48-56.

3. Yuan Yuting, Chen Yu, Li Ying, et al. Overview of traditional Chinese and western medicine treatment of pyoderma gangrenosum[J]. Chinese Journal of Chinese Medicine, 2017, 32( 8): 3614-3617.

4.LI J, YANG D Q, ZHAI Z F, et al. Analysis of 28 cases of pyoderma gangrenosum[J].J Clin Dermatol,2017,46(4): 230-233.

5. MARZANO A V, BORGHI A, STADNICKI A, et al. Cutaneous manifestations in patients with inflammatory bowel diseases: pathophysiology, clinical features, and therapy[J]. Inflamm Bowel Dis, 2014, 20(1): 213-327.

6. Lv Hong, Qian Jiaming, Wang Lihua. Extraintestinal manifestations of ulcerative colitis. Chinese Journal of Internal Medicine 2002; 41: 675-677.< /p>

7. Ge Wensong. Diagnosis and treatment strategies for extraintestinal manifestations of inflammatory bowel disease[J /CD]. Chinese Journal of Digestive Diseases and Imaging: Electronic Edition, 2017, 7( 3): 97-100.

8. Li Meng, Ding Kang, Yang Xu, et al. Classification and summary of skin manifestations of inflammatory bowel disease, Journal of Gastroenterology and Hepatology, 2019 (28) 7:821-825.

9. Zheng Lianpeng, Lv Zongshun, Zhang Jie, et al. A summary analysis of extraintestinal manifestations of inflammatory bowel disease in mainland China, World Chinese Journal of Digestion, 2019 , 17(21): 2217-2220.