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The main clinical variants of extraintestinal manifestations of inflammatory bowel diseases (Crohn's disease and ulcerative colitis)

DOI 10.18127/j20700997-201804-04


A.D. Nikogosova – Post-graduate Student, Department of Internal Diseases, Faculty of Fundamental Medicine, Lomonosov Moscow State University
V.G. Avdeev – Ph.D. (Med.), Associate Professor, Department of Internal Diseases, Faculty of Base Medicine Lomonosov Moscow State University
T.N. Krasnova – Ph.D. (Med.), Associate Professor, Department of Internal Diseases, Faculty of Fundamental Medicine, Lomonosov Moscow State University
T.P. Rozina – Ph.D. (Med.), Associate Professor, Head of office of gastroenterology, Medical Scientific and Educational Center of Lomonosov Moscow State University; Department of Internal Diseases, Faculty of Base Medicine, Lomonosov Moscow State University
E.P. Pavlikova – Dr.Sc. (Med.), Professor, Deputy Director and Head of the Department of Internal Diseases, Medical Scientific-Educational Center of Lomonosov Moscow State University
E.E. Yazev – Post-graduate Student, Department of Internal Diseases, Faculty of Fundamental Medicine, Lomonosov Mos-cow State University
N.A. Mukhin – Academician of RAS, Dr.Sc. (Med.), professor (1936–2018)

The aim of this study was to determine the importance of the extraintestinal manifestations of inflammatory bowel disease (IBD) for diagnostics and their relation to disease progression and gender.
Materials and methods: The analysis of the case histories of 74 patients with inflammatory bowel diseases (63.5% UC and 36.5% CD) was carried out.
Results: The autoimmune lesions of the liver were most frequently observed in patients with IBD: 28 patients with primary sclerosing cholangitis (PSC) and a variant form of autoimmune hepatitis in combination with primary sclerosing cholangitis (AIH / PSC) (14 patients). In patients with a combination of three diseases (IBD / AIH / PSC), the nature and course of the disease could be determined from initial clinical picture.
In 19 patients, peripheral or axial articular syndrome was identified, in 14 patients - various skin lesions, in 9 cases dam-age to the eyes and in 16 cases, other systemic manifestations were detected. Articular syndrome in 40% of cases was combined with other systemic manifestations and appeared 2.8 times more often in women than men.
Conclusion: Systemic manifestations can outpace the clinical manifestations of IBD or serve as their mask. The study of systemic manifestations will improve the diagnosis of inflammatory bowel diseases.
Key words: inflammatory bowel diseases, ulcerative colitis, Crohn's disease, systemic manifestations, systemic non-intestinal manifestations, primary sclerosing cholangitis, variant form of autoimmune hepatitis in combination with primary sclerosing cholangitis.
We examined 74 patients with inflammatory bowel disease (IBD): with ulcerative colitis (UC) - 46, with Crohn's disease (CD) - 28. Systemic manifestations were revealed in 66 (89.1%) patients. This slightly exceeds their frequency in other studies (17-40%) [1-4]. Systemic manifestations included various lesions of liver (autoimmune hepatitis - AIH, primary sclerosing cholangitis - PSC), joints (peripheral arthropathy, sacroiliitis, ankylosing spondylitis), eye (uveitis, scleritis), skin (dermatitis, erythema nodosum, gangrenous pyoderma), and thyroid. Some of them were symptoms of the disease itself, others (autoimmune liver disease, ankylosing spondylitis) were independent diseases accompanying IBD [10].
The articular syndrome was the most common variant as in the other studies (19 patients, 25.6%) [11,12]. The joint syn-drome was mainly a lesion of 2-3 large joints without changing their configuration. It was 2.8 times more common in women and accompanied with an acute exacerbation of IBD. In a number of cases, arthropathy was accompanied by a lesion of the skin and eyes and was associated with the presence of the HLA-B27 allele [13]. In 4 cases, IBD was accompanied by ankylosing spondylitis (5.5%), which was revealed as spinal cord injury, ankylosis and changes in the configuration of the joints. In this case, the damage to the joints in our patients occurred 20-27 years earlier than appearance of the intestinal symptoms.
Liver involvement in most patients was represented by autoimmune disease (46 patients, 62%): AIH in 4 patients (5.5%), PSC in 42 patients (56.7%). The frequency of combination of diseases of the intestine and liver in our study was higher than usually reported, due to the fact that the patients were examined in the hepatology department of the clinic [14].
As in previous studies, PSC was more often combined with UC (30 patients, 40%) than with CD (12 patients, 16%) [15]. The disease in 18 cases began with a clinical picture of UC, and in 24 cases with liver damage. At the same time, the UC is characterized by a greater extent of intestinal lesioning (in 17 patients total and subtotal, in 4 with proctosigmoiditis). However, in patients with UC without PSC, the total forms are much less frequent (proctosigmoiditis predominates) [16]. Intestinal disease in combination with PSC is characterized by slow progression.
In 14 patients with PSC (out of 46), an autoimmune cross between AIH / PSC was found (18.9%, 14 of 74) [17]. In this subgroup, the disease debuted with a clinical picture of AIH (6 patients), PSC (4 patients) or IBD (4 patients). The mean age at onset of the disease with AIH was 19 [15, 32] years, with PSC was 29.5 [19, 38] and with IBD was 25 [15, 33]. Some of the patients whose onset of the disease was with AIH was characterized by a younger age, predominantly female sex (5 women, 1 male), while PSC usually started at middle age and equally in men and women [18,19]. According to the results of our study, in patients with a combination of these three diseases (IBD / AIH / PSC), the nature and course of the disease depends on the initial clinical picture.
Systemic manifestations were sometimes first seen in the clinical picture of IBD. They often outstrip the intestinal symptoms, which can complicate timely diagnosis. Moreover, while the intestinal disease itself can occur with low activity and do not affect life expectancy, a number of systemic manifestations have a significant, determinative effect on the prognosis, significantly worsening it, such as PSC.

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