Hepatic sarcoidosis in patients presenting with liver dysfunction: imaging appearance, pathological correlation and disease evolution

Fetzer, David T.; Rees, Mitchell A.; Dasyam, Anil K.; Tublin, Mitchell

doi:10.1007/s00330-015-4169-2

Cited by 24 publications

(27 citation statements)

References 33 publications

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“…2,6 Symptoms of sarcoidosis are associated with the involved organs and include shortness of breath and cough with pulmonary involvement and portal hypertension manifestation with liver sarcoidosis. [10][11][12] Our patient presented with chronic abdominal pain and distention, and her chronic constipation was concerning for possible functional bowel disease or chronic mechanical obstruction.…”

Section: Discussionmentioning

confidence: 99%

“…10 CT scan is considered a more sensitive test in the setting of symptoms associated with involved organs such as the portal hypertension complication with liver sarcoidosis. [7][8][9][10][11] The clinical presentation of abdominal sarcoidosis is not specific and generally mimics other abdominal diagnoses such as small bowel obstruction or other obstructive pathology. Having such extensive disease of the gastrointestinal tract and surrounding organs without involvement of mediastinal structures is extremely rare.…”

Section: Discussionmentioning

confidence: 99%

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Extensive Abdominal Sarcoidosis Without Pulmonary Manifestation

Alatmi

Joury

Mubarak

2018

TOJ

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Background: Sarcoidosis is a rare multisystem disease of idiopathic etiology that affects different organs and is characterized histopathologically by the presence of noncaseating granulomas. The most common location of sarcoidosis is the lungs, accounting for approximately 95% of sarcoidosis cases. Coincident involvement of pulmonary and extrapulmonary organs is present in approximately one-third of patients. However, the sole involvement of intraabdominal organs is extremely rare. Intraabdominal sarcoidosis has a different presentation depending on the involved organs. To the best of our knowledge, a limited number of case reports have been published on intraabdominal sarcoidosis without pulmonary involvement. Case Report: A 69-year-old female patient presented with short onset of abdominal pain and change in bowel habits, without a significant medical history of abdominal problems. Physical examination only revealed generalized abdominal tenderness on deep palpation. Computed tomography of the abdomen and pelvis showed mild to moderate thickening of the omentum and other abdominal organs. After excluding various etiologies such as fungi and acid-fast bacilli, the omentum, ovaries, and surrounding lymph nodes were removed via a laparoscopic procedure. The histopathology report confirmed the diagnosis of intraabdominal sarcoidosis with the presence of noncaseating granulomas. Conclusion: Extrapulmonary sarcoidosis is a rare disease, and intraabdominal sarcoidosis might mimic other malignant etiologies. We treated our case with laparoscopic removal of involved organs and oral steroids with significant recovery during a short period.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Extensive Abdominal Sarcoidosis Without Pulmonary Manifestation

Alatmi

Joury

Mubarak

2018

TOJ

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“…Mild liver disease with hepatic granulomas frequently occurs in sarcoidosis, 54,55 although cirrhosis occurs in less than 1% of sarcoidosis patients. 56 Hypothetically, cirrhosis and portal hypertension might lead to PH in this subgroup of patients, 56,57 although evidence is scarce.…”

Section: Portal Pulmonary Hypertensionmentioning

confidence: 99%

Sarcoidosis-Associated Pulmonary Hypertension

Huitema

Mathijssen

Mager

et al. 2020

Semin Respir Crit Care Med

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Pulmonary hypertension (PH) is a well-known complication of sarcoidosis, defined by a mean pulmonary artery pressure of ≥25 mm Hg. Since both PH and sarcoidosis are rare diseases, data on sarcoidosis-associated PH (SAPH) is retrieved mostly from small retrospective studies. Estimated prevalence of SAPH ranges from 3% in patients referred to a tertiary center up to 79% in patients awaiting lung transplant. Most patients with SAPH show advanced parenchymal disease as the underlying mechanism. However, some patients have disproportional elevated pulmonary artery pressure, and PH can occur in sarcoidosis patients without parenchymal disease. Other mechanisms such as vascular disease, pulmonary embolisms, postcapillary PH, extrinsic compression, and other sarcoidosis-related comorbidities might contribute to SAPH. The diagnosis of PH in sarcoidosis is challenging since symptoms and signs overlap. Suspicion can be raised based on symptoms or tests, such as pulmonary function tests, laboratory findings, electrocardiography, or chest CT. PH screening mainly relies on transthoracic echocardiography. Right heart catheterization should be considered on a case-by-case basis in patients with clinical suspicion of PH, taking into account clinical consequences. Treatment options are considered on patient level in a PH expert center, and might include oxygen therapy, immunosuppressive, or PH-specific therapy. However, qualitative evidence is scarce. Furthermore, in a subset of patients, interventional therapy or eventually lung transplant can be considered. SAPH is associated with high morbidity. Mortality is higher in sarcoidosis patients with PH compared with those without PH, and increases in patients with more advanced stages of sarcoidosis and/or PH.

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“…[33][34][35] Intraabdominal lymphadenopathy may be present up to 40 to 60% of cases, mainly located in the periportal and gastrohepatic areas. 36,37 Focal hepatic lesions may be found in 5 to 38% of patients, consisting of nodules of 0.5 to 1.5 cm, hypodense on CT or hypointense on MRI, with or without calcifications. 33,37 These nodules are more infrequently found with US and may involve the spleen in 15 to 45% patients with hepatic sarcoidosis.…”

Section: Radiological Findingsmentioning

confidence: 99%

Hepatic Sarcoidosis: Current Concepts and Treatments

Rossi

Ziol

Roulot

et al. 2020

Semin Respir Crit Care Med

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Hepatic sarcoidosis is a relatively common manifestation of extrapulmonary sarcoidosis. It occurs in 20 to 30% of cases and is rarely severe. However, a cluster of patients may develop severe complications such as cirrhosis and portal hypertension. In this review, we describe the current knowledge of clinical, biological, pathological, and radiological features of liver involvement in sarcoidosis and discuss essential clues for management and treatment.

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Hepatic sarcoidosis in patients presenting with liver dysfunction: imaging appearance, pathological correlation and disease evolution

Cited by 24 publications

References 33 publications

Extensive Abdominal Sarcoidosis Without Pulmonary Manifestation

Extensive Abdominal Sarcoidosis Without Pulmonary Manifestation

Sarcoidosis-Associated Pulmonary Hypertension

Hepatic Sarcoidosis: Current Concepts and Treatments

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