Juvenile sarcoidosis presenting as Crohn’s Disease

Brunner, Jürgen; Sergi, Consolato; Müller, Thomas; Gaßner, Ingmar; Prüfer, Friederike; Zimmerhackl, Lothar Bernd

doi:10.1007/s00431-005-0059-y

Cited by 21 publications

(9 citation statements)

References 16 publications

(20 reference statements)

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“…It is known that GI sarcoidosis can appear clinically and pathologically like a Crohn’s disease, a Whipple’s disease, or an infection by tuberculosis, syphilis or fungi. Especially colon and terminal ileum sarcoidosis could easily mime a Crohn’s disease, also in the histopathological findings [26–28]. Therefore, it is necessary to find the presence of non-caseating granulomatous inflammation to differentiate GI sarcoidosis from Crohn’s disease.…”

Section: Discussionmentioning

confidence: 99%

A case of sarcoidosis with isolated hepatosplenic onset and development of inflammatory bowel disease during recovery stage

Sangineto

Luglio

Suppressa

et al. 2017

Autoimmun Highlights

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Sarcoidosis is a systemic disease characterized by an immune-mediated disorder, which leads to the development of non-caseating granulomas in the involved organs. More than 90% of patients with sarcoidosis present lungs and lymphatic system involvement at onset, while less than 10% has an isolated extrapulmonary localization. Here, we describe the case of an elderly patient with isolated hepato-splenic onset (multiple splenic lesions at imaging and cholestasis), and subsequent pulmonary involvement. The liver biopsy showed the presence of non-caseating granulomas, suggesting sarcoidosis. Despite the complete recovery was obtained with steroid therapy, after dosage reduction the patient presented watery diarrhea. Endoscopic investigations with biopsies were performed, describing the presence of an important lympho-plasmacytic infiltrate of terminal ileum mucosa with typical aspects of inflammatory bowel disease. The symptomatology completely disappeared after steroid dosage increase. This case confirms that sarcoidosis could present in a very atypical way, involving several organs in a different manner at the same time and that every symptom should not be underestimated, despite the rare presentation.

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Section: Discussionmentioning

confidence: 99%

A case of sarcoidosis with isolated hepatosplenic onset and development of inflammatory bowel disease during recovery stage

Sangineto

Luglio

Suppressa

et al. 2017

Autoimmun Highlights

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“…Cases in which sarcoidosis involves the bowel may closely mimic Crohn's disease clinically, but although there is then usually some thickening of the affected segment, together with the microscopic change of intense granulomatous inflammation, this is rarely transmural, so that typically the gross pathology differs from that of Crohn's disease and does not give rise to the obstruction and fistulation characteristic of the latter 30 40. When the presenting symptoms are those of intestinal inflammation, the earliest investigations do not always allow for the possibility that sarcoidosis might be the cause; so that an incomplete history or delay in requesting a chest film showing bilateral hilar lymphadenopathy and/or pulmonary shadowing raises doubt as to whether this was the primary diagnosis or perhaps secondary to the increased permeability of an inflamed intestinal mucosa to some agent capable of triggering sarcoidosis 30 40.…”

Section: Discussionmentioning

confidence: 99%

“…When the presenting symptoms are those of intestinal inflammation, the earliest investigations do not always allow for the possibility that sarcoidosis might be the cause; so that an incomplete history or delay in requesting a chest film showing bilateral hilar lymphadenopathy and/or pulmonary shadowing raises doubt as to whether this was the primary diagnosis or perhaps secondary to the increased permeability of an inflamed intestinal mucosa to some agent capable of triggering sarcoidosis 30 40. In one case, it was not until autopsy was performed that sarcoidosis became the preferred diagnosis 41…”

Section: Discussionmentioning

confidence: 99%

Crohn's disease and sarcoidosis in siblings: follow-up of a published report with a new case and brief review of the literature

Willoughby

2016

Frontline Gastroenterol

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Reported in 1971 were the cases of three brothers, two of whom had developed sarcoidosis and the third Crohn's disease. That now presented concerns one brother who, 50 years after the diagnosis and successful treatment of his sarcoidosis, was found incidentally, at colonoscopy performed to exclude malignancy, to have Crohn's colitis in the absence of any symptoms attributable to this. The report concludes with a brief review of the literature.

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“…It has been hypothesized that NOD2 susceptibility loci in intestinal Paneth cells led to defective NF-κB activation, altered intestinal bacterial clearance and granulomatous inflammation [22,29,30]. Juvenile sarcoidosis presenting as Crohn's disease of the GIT has been reported [31].…”

Section: Blau Syndrome and Early Onset Sarcoidosis (Bs-eos)mentioning

confidence: 99%

Pediatric Sarcoidosis: A Review with Emphasis on Early Onset and High-Risk Sarcoidosis and Diagnostic Challenges

et al. 2019

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Sarcoidosis is a non-necrotizing granulomatous inflammatory syndrome with multisystemic manifestations. We performed a systematic review of sarcoidosis in the pediatric population with particular emphases on early onset sarcoidosis, high-risk sarcoidosis, and newly reported or unusual sarcoid-related diseases. Blau Syndrome and early onset sarcoidosis/ BS-EOS are seen in children younger than five years old presenting with extra-thoracic manifestations but usually without lymphadenopathy and/or pulmonary involvement. The prevalence of high-risk sarcoidosis is very low in children and is further limited by the difficulty of diagnosis in symptomatic children and underdiagnosis in subclinical or asymptomatic patients. Reports of sarcoidal syndromes in users of E-cigarette/marijuana/other flavorings and their induction in cancer immunotherapies are of interests and may be challenging to differentiate from metastatic malignancy. The diagnostic considerations in pediatric sarcoidosis are to support a compatible clinicoradiographic presentation and the pathologic findings of non-necrotizing granulomas by ruling out granulomas of infective etiology. There is no absolutely reliable diagnostic test for sarcoidosis at present. The use of endoscopic bronchial ultrasound (EBUS) and transbronchial fine needle aspiration (TBNA) sampling of intrathoracic lymph nodes and lung, and for superficially accessible lesions, with cytopathological assessment and pathological confirmations provide fair diagnostic yield and excellent patient safety profile in children.

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Juvenile sarcoidosis presenting as Crohn’s Disease

Cited by 21 publications

References 16 publications

A case of sarcoidosis with isolated hepatosplenic onset and development of inflammatory bowel disease during recovery stage

A case of sarcoidosis with isolated hepatosplenic onset and development of inflammatory bowel disease during recovery stage

Crohn's disease and sarcoidosis in siblings: follow-up of a published report with a new case and brief review of the literature

Pediatric Sarcoidosis: A Review with Emphasis on Early Onset and High-Risk Sarcoidosis and Diagnostic Challenges

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