Churg–Strauss syndrome manifesting as cholestasis and diagnosed by liver biopsy

Harada, Masaru; Oe, Shinji; Shibata, Michihiko; Taguchi, Masashi; Matsuhashi, Toru; Hiura, Masaaki; Abe, Shintaro; Harada, Riko; Shimajiri, Shohei

doi:10.1111/j.1872-034x.2012.00993.x

Cited by 8 publications

(3 citation statements)

References 13 publications

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“…Severe complications, including bowel obstruction and stenosis, can occur in 22–45% of the patients [ 72 ]. Hepatobiliary complications are infrequent and include cholestasis and liver infarction [ 73 – 75 ]. The potential underlying causes of hepatic involvement are fibrinoid necrosis of arteries, eosinophilic infiltration, and granulomatous reaction in the portal area.…”

Section: Small-vessel Vasculitismentioning

confidence: 99%

Imaging of intestinal vasculitis focusing on MR and CT enterography: a two-way street between radiologic findings and clinical data

et al. 2022

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Diagnosis of intestinal vasculitis is often challenging due to the non-specific clinical and imaging findings. Vasculitides with gastrointestinal (GI) manifestations are rare, but their diagnosis holds immense significance as late or missed recognition can result in high mortality rates. Given the resemblance of radiologic findings with some other entities, GI vasculitis is often overlooked on small bowel studies done using computed tomography/magnetic resonance enterography (CTE/MRE). Hereon, we reviewed radiologic findings of vasculitis with gastrointestinal involvement on CTE and MRE. The variety of findings on MRE/CTE depend upon the size of the involved vessels. Signs of intestinal ischemia, e.g., mural thickening, submucosal edema, mural hyperenhancement, and restricted diffusion on diffusion-weighted imaging, are common in intestinal vasculitis. Involvement of the abdominal aorta and the major visceral arteries is presented as concentric mural thickening, transmural calcification, luminal stenosis, occlusion, aneurysmal changes, and collateral vessels. Such findings can be observed particularly in large- and medium-vessel vasculitis. The presence of extra-intestinal findings, including within the liver, kidneys, or spleen in the form of focal areas of infarction or heterogeneous enhancement due to microvascular involvement, can be another radiologic clue in diagnosis of vasculitis. The link between the clinical/laboratory findings and MRE/CTE abnormalities needs to be corresponded when it comes to the diagnosis of intestinal vasculitis.

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Section: Small-vessel Vasculitismentioning

confidence: 99%

Imaging of intestinal vasculitis focusing on MR and CT enterography: a two-way street between radiologic findings and clinical data

et al. 2022

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“…Furthermore, the direct pathologic confirmation of vasculitis in the liver is extremely rare. 2,3 Therefore, liver involvement of EGPA may be overlooked because of its rarity. To differentiate between EGPA involvement and other causes, such as drug-induced liver failure, a liver needle biopsy should be performed when the patient presents with hypereosinophilia, liver dysfunction, and multiple nodular low-density areas on a CT scan.…”

Section: Answer To: Image 4: Liver Involvement Of Eosinophilic Granulmentioning

confidence: 99%

Multiple Low-Density Areas in the Liver

2020

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Question: A 57-year-old man was transferred to our hospital because of hypereosinophilia, skin eruption on his lower leg, and liver dysfunction while hospitalized for status asthmaticus in another hospital. Furthermore, he complained of paresthesia and myalgia of the extremities during further investigation in our hospital. Laboratory analysis revealed an elevated white blood cell count of 23,200/mm 3 and hypereosinophilia (70.0%). Cholestatic liver dysfunction tests showed the following results: aspartate transaminase (51 U/L), alanine aminotransferase (106 U/L), lactate dehydrogenase (262 U/L), alkaline phosphatase (736 U/L), and gglutamyl transferase (117 U/ L). Hepatitis B surface antigen and hepatitis C virus RNA test results were negative. Antinuclear antibody, antimitochondrial antibody, myeloperoxydase anti-neutrophil cytoplasmic antigen, and proteinase-3 anti-neutrophil cytoplasmic antigen were normal. Transbronchial lung biopsy and skin biopsy showed no pathological significance. An enhanced abdominal computed tomography (CT) scan was performed (Figure A; enlarged view in Figure B). What is the diagnosis? See the Gastroenterology web site (www. gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI.

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“…The gastrointestinal tract, kidney and heart must be screened for vasculitis involvements, due to their poorer prognosis. Still, it should be taken into consideration that cases of EGPA with hepatic involvement have been rarely reported (6,7).…”

Section: Introductionmentioning

confidence: 99%

Hepatic Involvement in Eosinophilic Granulomatosis with Polyangiitis

Mahdavi¹,

Dolatshahi²,

Tabesh³

2022

IGJ

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We presented a case report of Eosinophilic Granulomatosis with Polyangiitis (EGPA) with hepatic involvement which presented itself as multiple nodules in the liver. This confirmed Hprevious literature results, which have reported hepatic involvement in EGPA. The results of the study showed a 45-year-old female with asthma, presented with polyarthralgia, hypostasis in both hands and skin lesions on the body. Lab tests revealed elevated Erythrocyte Sedimentation Rate (ESR) and C-Reactive Protein (CRP). The total Leukocyte count was 11900 (/ul) with 22% Eosinophil. Computed Tomography (CT) scan of both lungs and liver showed multiple irregular nodules. In the biopsy of the skin lesions, Eosinophilic Vasculitis was reported. Electromyography and Nerve Conduction Velocity (EMG-NCV) was compatible to C8-T11 radiculopathy and Axonal Sensory Motor Polyneuropathy. According to the tests and biopsies the patient was diagnosed with EGPA. Although EGPA is characterized by asthma, hypereosinophilia and vasculitis, it can be presented with atypical manifestations as well.

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Churg–Strauss syndrome manifesting as cholestasis and diagnosed by liver biopsy

Cited by 8 publications

References 13 publications

Imaging of intestinal vasculitis focusing on MR and CT enterography: a two-way street between radiologic findings and clinical data

Imaging of intestinal vasculitis focusing on MR and CT enterography: a two-way street between radiologic findings and clinical data

Multiple Low-Density Areas in the Liver

Hepatic Involvement in Eosinophilic Granulomatosis with Polyangiitis

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