Increasing Rate of a Positive Family History of Inflammatory Bowel Disease (IBD) in Pediatric IBD Patients

Abstract The role of a positive family history in pediatric inflammatory bowel disease (IBD) in the era of biologic therapy has not been elucidated. We retrospectively reviewed the medical records of children with IBD and retrieved demographic and clinical characteristics, including the presence of a positive family history of IBD, IBD phenotype, disease course, and therapy. Overall, 325 children (age range at diagnosis 11-15 years) were included, of whom 82 (25.2%) had a positive family history. Children diagnosed during 2016-2020 had a higher frequency of positive family history compared to those diagnosed during 2010-2015 (31.8% versus 20.7%, respectively, p = 0.024). Children with a positive family history had a higher risk for a stricturing phenotype than those with a negative family history (11.3% versus 2.8%, respectively, p = 0.052). They more often received nutritional therapy (53.7% versus 36.6%, p = 0.007) and less often received corticosteroids (36.6% versus 52.7%, p = 0.012). More children with a negative family history needed intensification of biologic therapy (p = 0.041). Conclusion: The rate of a positive family history of IBD in the pediatric IBD population is increasing. A positive family history may have some impact upon IBD phenotype but none on IBD outcome.

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P197 The effect of a positive family history on the phenotype and course of paediatric Inflammatory Bowel Disease

Journal of Crohn s and Colitis ◽

10.1093/ecco-jcc/jjab076.323 ◽

2021 ◽

Vol 15 (Supplement_1) ◽

pp. S262-S263

Author(s):

M Ruban ◽

S Cohen ◽

A Yerushalmy-Feler

Keyword(s):

Inflammatory Bowel Disease ◽

Family History ◽

Family Member ◽

Bowel Disease ◽

Positive Family History ◽

Nutritional Therapy ◽

Negative Family History ◽

History Of ◽

Inflammatory Bowel ◽

Aggressive Clinical Course

Abstract Background Previous studies have suggested that adult patients with inflammatory bowel disease (IBD) that have a positive family history of IBD may be at risk for a more aggressive clinical course, compared with sporadic cases. The role of a positive family history in paediatric IBD, however, has not been elucidated. Methods We retrospectively reviewed the medical records of children with IBD and retrieved demographic and clinical characteristics, including IBD phenotype, course and therapy. We have documented the presence of a positive family history of IBD, and compared the clinical data of children with a positive family history to that of children with a negative family history. Results Overall, 325 children with a median (IQR) age of 13.9 (11–15) years at diagnosis were included: 194 (59.7%) with Crohn’s disease (CD) and 131 (40.3%) with ulcerative colitis. A positive family history of IBD was observed in 82 children (25.2%). Of them, 36 (43.9%) had a first-degree family member with IBD, and 23 (28%) had more than one family member with IBD. Children with a positive family history had a higher risk for stricturing phenotype (11.3% versus 2.8%, P=0.05) and were treated more often by nutritional therapy (53.7% versus 36.6%, P=0.007) and less by corticosteroids (36.6% versus 52.7%, P=0.012). Children with a negative family history had a higher need to intensification of biologic therapy [Hazard ratio (HR)=1.792, 95% confidence interval (CI) 1.005–3.195, P=0.041 and HR=2.597, 95% CI 1.224–5.525, P=0.008 for CD) (Figure 1). No difference in the risk for IBD exacerbation, hospitalization and operation was found between the groups. Conclusion A positive family history of IBD may have a mild impact on the phenotype and course of paediatric IBD.

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Increasing rate of a positive family history of inflammatory bowel disease (IBD) in pediatric IBD patients

European Journal of Pediatrics ◽

10.1007/s00431-021-04269-8 ◽

2021 ◽

Author(s):

Maya Ruban ◽

Adam Slavick ◽

Achiya Amir ◽

Amir Ben-Tov ◽

Hadar Moran-Lev ◽

...

Keyword(s):

Inflammatory Bowel Disease ◽

Family History ◽

Bowel Disease ◽

Positive Family History ◽

History Of ◽

Inflammatory Bowel ◽

Pediatric Ibd

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Phenotypic Features and Longterm Outcomes of Pediatric Inflammatory Bowel Disease Patients with Arthritis and Arthralgia

The Journal of Rheumatology ◽

10.3899/jrheum.170168 ◽

2017 ◽

Vol 44 (11) ◽

pp. 1636-1643 ◽

Cited By ~ 2

Author(s):

Osnat Nir ◽

Firas Rinawi ◽

Gil Amarilyo ◽

Liora Harel ◽

Raanan Shamir ◽

...

Keyword(s):

Inflammatory Bowel Disease ◽

Bowel Disease ◽

Medical Records ◽

Biologic Therapy ◽

Joint Involvement ◽

Pediatric Inflammatory Bowel Disease ◽

History Of ◽

Inflammatory Bowel ◽

Phenotypic Features ◽

Pediatric Ibd

Objective.The natural history of pediatric inflammatory bowel disease (IBD) patients with joint involvement has not been clearly described. Thus, we aimed to investigate phenotypic features and clinical outcomes of this distinct association.Methods.The medical records of patients with pediatric IBD diagnosed from 2000 to 2016 were reviewed retrospectively. Main outcome measures included time to first flare, hospitalization, surgery, and biologic therapy.Results.Of 301 patients with Crohn disease (median age 14.2 yrs), 37 (12.3%) had arthritis while 44 (14.6%) had arthralgia at diagnosis. Arthritis and arthralgia were more common in women (p = 0.028). Patients with arthritis and arthralgia demonstrated lower rates of perianal disease (2.7% and 4.5% vs 16.9%, p = 0.013), whereas patients with arthritis were more likely to be treated with biologic therapy (HR 2.05, 95% CI 1.27–3.33, p = 0.009). Of 129 patients with ulcerative colitis (UC; median age 13.7 yrs), 3 (2.3%) had arthritis and 16 (12.4%) had arthralgia at diagnosis. Patients with arthralgia were treated more often with corticosteroids (p = 0.03) or immunomodulator therapies (p = 0.003) compared with those without joint involvement. The likelihood to undergo colectomy was significantly higher in patients with arthralgia (HR 2.9, 95% CI 1.1–7.4, p = 0.04). During followup (median 9.0 yrs), 13 patients developed arthritis (3.3%). Arthralgia at diagnosis was a significant predictor for the development of arthritis during followup (HR 9.0, 95% CI 2.86–28.5, p < 0.001).Conclusion.Pediatric IBD patients with arthritis have distinct phenotypic features. Arthralgia at diagnosis is a predictor for colectomy in UC and a risk factor for the development of arthritis during followup.

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POS1004 BOTH SPONDYLOARTHRITIS AND PSORIATIC ARTHRITIS PATIENTS HAVE STRONG FAMILY HISTORIES

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2021-eular.3330 ◽

2021 ◽

Vol 80 (Suppl 1) ◽

pp. 770.2-771

Author(s):

G. K. Yardimci ◽

B. Farisoğullari ◽

E. C. Bolek ◽

E. Bilgin ◽

E. Duran ◽

...

Keyword(s):

Inflammatory Bowel Disease ◽

Psoriatic Arthritis ◽

Family History ◽

Bowel Disease ◽

Positive Family History ◽

Laboratory Data ◽

Strong Family History ◽

Male Predominance ◽

History Of ◽

Inflammatory Bowel

Background:Family history is one of the hallmarks of spondyloarthritis (SpA) and psoriatic arthritis (PsA) [1, 2]. Some patients have a strong family history that more than 2 relatives have spondyloarthritis related diseases. The effects of strong family history on SpA features were not known very well.Objectives:The aim of this study is to evaluate the effects of family history in SpA and PsA patients.Methods:HUR-BIO (Hacettepe University Biologic Registry) is a prospective, single center database of biological treatments since 2005, and to date 3071 SpA and 526 PsA patients have been recorded. Demographic, clinical characteristics, disease activity parameters, a detailed family history of SpA and SpA features (presence of SpA including PsA, psoriasis, inflammatory bowel disease and uveitis) and laboratory data before anti-TNF treatments of the patients were noted.Results:2807 SpA (53.6% male) and 506 PsA (31.4% male) patients’ family history were available and analysed. A positive family history was noted in 27.6% of the SpA and 31.0% of the PsA patients (ns). 7.4% of the SpA patients and 8.9% of the PsA patients had family history in more than one relative (Table 1). In SpA patients with a family history, uveitis was more frequent than patients without (14.4% vs 10.6%, p=0.006). Except for a higher male predominance and uveitis (53% vs 32% p=0.006 and 9% vs 2% p=0.003 respectively) in patients with ≥2 relatives with SpA features, there were no differences in PsA patients regarding family history. The presence of family history and HLA-B27 (63.7% vs 37.6%, p<0.001) positivity were associated in SpA patients but not in PsA patients (31.2% vs 20.0, p=0.13).Conclusion:Family history was present in about one third of the patients of PsA and SpA. It is not uncommon for two or more family members to have a SpA feature. Presence of family history may be associated with some clinical conditions, such as uveitis.References:[1]Solmaz, D., et al., Impact of Having Family History of Psoriasis or Psoriatic Arthritis on Psoriatic Disease. Arthritis Care Res (Hoboken), 2020. 72(1): p. 63-68.[2]Zurita Prada, P.A., et al., Influence of smoking and obesity on treatment response in patients with axial spondyloarthritis: a systematic literature review. Clin Rheumatol, 2020.Table 1.Family history in PsA and SpA patientsPsA (n=506)SpA (n=2807)≥ 1 family history, n (%)157 (31.0)774 (27.6)≥1 first-degree relative, n (%)114 (22.5)489 (17.4)≥2 first-degree relatives, n (%)21 (4.2)77 (2.7)≥2 relatives (both first- and second-degree), n (%)45 (8.9)208 (7.4)Family history •Psoriasis, n (%)120 (23.7)155 (5.5) •Psoriatic arthritis, n (%)14 (2.8)9 (0.3) •Spondyloarthritis, n (%)38 (7.5)643 (22.9) •Inflammatory bowel disease, n (%)1 (0.2)10 (0.4) •Uveitis, n (%)02 (0.1)Disclosure of Interests:None declared.

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Influence of a Positive Family History on the Clinical Course of Inflammatory Bowel Disease

Journal of Crohn s and Colitis ◽

10.1093/ecco-jcc/jjw063 ◽

2016 ◽

Vol 10 (9) ◽

pp. 1024-1032 ◽

Cited By ~ 10

Author(s):

Sung Wook Hwang ◽

Min Seob Kwak ◽

Wan Soo Kim ◽

Jeong-Mi Lee ◽

Sang Hyoung Park ◽

...

Keyword(s):

Inflammatory Bowel Disease ◽

Family History ◽

Bowel Disease ◽

Clinical Course ◽

Positive Family History ◽

Inflammatory Bowel

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Mo1375 Prevalence of Family History of Inflammatory Bowel Disease Among Ulcerative Colitis Patients

Gastroenterology ◽

10.1016/s0016-5085(13)62406-3 ◽

2013 ◽

Vol 144 (5) ◽

pp. S-649-S-650

Author(s):

Ryan E. Childers ◽

Swathi Eluri ◽

Christine Vazquez ◽

Theodore M. Bayless ◽

Susan Hutfless

Keyword(s):

Ulcerative Colitis ◽

Inflammatory Bowel Disease ◽

Family History ◽

Bowel Disease ◽

History Of ◽

Inflammatory Bowel

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P127 INFLAMMATORY BOWEL DISEASE IN CHILDREN: THE ROLE OF A POSITIVE FAMILY HISTORY

Journal of Crohn s and Colitis Supplements ◽

10.1016/s1873-9954(08)70138-1 ◽

2008 ◽

Vol 2 (1) ◽

pp. 43

Author(s):

E. Roma ◽

I. Panagiotou ◽

J. Pahoula ◽

C. Constantinidou ◽

A. Polyzos ◽

...

Keyword(s):

Inflammatory Bowel Disease ◽

Family History ◽

Bowel Disease ◽

Positive Family History ◽

Inflammatory Bowel

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Crohn’s disease severity in familial and sporadic cases

Gut ◽

10.1136/gut.44.1.91 ◽

1999 ◽

Vol 44 (1) ◽

pp. 91-95 ◽

Cited By ~ 44

Author(s):

F Carbonnel ◽

G Macaigne ◽

L Beaugerie ◽

J P Gendre ◽

J Cosnes

Keyword(s):

Inflammatory Bowel Disease ◽

Crohn’S Disease ◽

Family History ◽

Crohn's Disease ◽

Disease Severity ◽

Bowel Disease ◽

History Of ◽

Sporadic Cases ◽

Inflammatory Bowel ◽

Excisional Surgery

BackgroundHaving a relative with inflammatory bowel disease increases the risk for Crohn’s disease but may also increase its severity in affected patients.AimsTo evaluate the influence of a family history on Crohn’s disease course and severity.Methods1316 patients followed in the same unit were studied retrospectively. Age at onset, duration of illness, site, and extent of disease were determined in patients with and without a family history. Additionally, disease severity was estimated by the need for medical therapy (steroid and immunosuppressive requirement) and the frequency and extent of excisional surgery.Results152 (12%) patients had a family history of inflammatory bowel disease. Duration of follow up was longer in patients with a family history and there were more operations for perforating complications in familial cases. However, the importance of medical therapy, and the incidence and extent of excisional surgery were similar in familial and and sporadic cases. Kaplan-Meier estimated time to prescription of immunosuppressive drugs and first intestinal resection were similar in familial and sporadic cases. When the 152 patients with familial Crohn’s disease were paired for sex, location of disease at onset, date of birth, and date of diagnosis with 152 patients with sporadic Crohn’s disease, the disease severity remained similar in the two groups of paired patients.ConclusionPatients with Crohn’s disease and a family history of inflammatory bowel disease do not have a more severe course.

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Granulomatous Inflammation Causing Severe Supraglottic Edema and Airway Obstruction in a Pediatric Patient

Ear Nose & Throat Journal ◽

10.1177/0145561320973772 ◽

2020 ◽

pp. 014556132097377

Author(s):

Sophia M. Colevas ◽

Bradley T. Gietman ◽

Shelly M. Cook ◽

Tony L. Kille

Keyword(s):

Inflammatory Bowel Disease ◽

Family History ◽

Crohn Disease ◽

Bowel Disease ◽

Sleep Disordered Breathing ◽

Granulomatous Inflammation ◽

Laser Resurfacing ◽

History Of ◽

Inflammatory Bowel ◽

Supraglottic Larynx

A 12-year-old male with a family history of inflammatory bowel disease presented with sleep-disordered breathing and was found to have chronic, granulomatous swelling of the supraglottic larynx. His airway was managed with tracheostomy, regular interval laryngeal steroid injections, supraglottoplasty, and “pepper pot” CO2 laser resurfacing leading to eventual decannulation. Due to the non-necrotic nature of the granulomatous inflammation, as well as the patient’s family history of inflammatory bowel disease, the leading diagnosis was Crohn disease, but isolated laryngeal sarcoidosis could not be ruled out. There are only 13 reported cases of laryngeal manifestations of Crohn disease in the literature, with only 2 cases occurring in pediatric patients. This case report adds to this body of literature and discusses strategies for managing granulomatous supraglottic edema when definitive diagnosis is not fully clear.

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