Abdominal Sarcoidosis Research Articles

S3310 Abdominal Sarcoidosis Including Hepatic and Portal Lymphadenopathy as a Primary Manifestation in a Case of Systemic Sarcoidosis

S3310 Abdominal Sarcoidosis Including Hepatic and Portal Lymphadenopathy as a Primary Manifestation in a Case of Systemic Sarcoidosis

CT Findings in Pulmonary and Abdominal Sarcoidosis. Implications for Diagnosis and Classification.

Sarcoidosis is a granulomatous disorder of unknown etiology characterized by noncaseating granulomas virtually in every organ and tissue. This finding represents the most important diagnostic clue to reach a correct definition of sarcoidosis, although the biopsy is invasive and has several risk procedures. Several efforts are made to suspect the diagnosis of sarcoidosis by combining noninvasive elements, in particular from imaging, though these findings are often nonspecific and reflect the wide multifactorial pathogenesis. Every effort should be made to obtain a detailed radiological picture that, if associated with a suggestive clinical picture, could avoid the need of biopsy in some specific cases. In this narrative review, we aim to describe main computed tomography (CT) features of pulmonary and abdominal sarcoidosis, by reporting strengths and limits of this technique, in particular for the identification of extrapulmonary, isolated disease.

MON-340 Isolated Extrapulmonary Sarcoidosis Presenting as Symptomatic Hypercalcemia

Introduction: Sarcoidosis is a granulomatous disease of unknown etiology often involving multiple organ systems. Sarcoidosis most frequently affects the lungs, but in upto 30% of cases, can present with extrapulmonary manifestations. Less than 10% of patients with sarcoidosis present with disease at extrapulmonary sites. 10%-20% patients with sarcoidosis present with hypercalcemia. Hypercalcemia in sarcoidosis is secondary to increased intestinal calcium absorption due to increased levels of 1,25-dihydroxy Vitamin D. Clinical Case: 71-year-old Caucasian male presenting to his primary care physician with 3-week duration of fatigue, anorexia, mild confusion and unintentional weight loss was found to have moderate hypercalcemia and acute kidney injury. He was admitted the hospital for evaluation and management. Labs at admission revealed albumin-corrected calcium of 13.5 mg/dL (normal 8.5-11.0 mg/dL), creatinine of 1.78 mg/dL (normal 0.7- 1.3mg/dL) and alkaline phosphatase of 173 U/L (normal 45-117 U/L). Workup noted low PTH (3,normal 14-72 pg/mL), normal 25-hydroxy Vitamin D (50.7,normal 30-100 ng/dL), normal PTHrP and normal serum electrophoresis and immunofixation indicating non-PTH dependent hypercalcemia. 1,25-dihydroxy Vitamin D (125, normal 18-64 pg/mL) and ACE levels (159 U/L, normal 16-85 U/L) were elevated. Patient denied being on any Vitamin D supplements. A CT chest, abdomen and pelvis was done to look for occult malignancy and hepatosplenomegaly was noted with only mild compressive atelectasis of lungs. Abdominal ultrasound confirmed hepatomegaly with lobulated outer contour consistent with cirrhosis. Acute hepatitis, infectious and autoimmune work up was negative. Patient was discharged with mild improvement in calcium and mental status with hydration. Due to high suspicion for granulomatous disease, a liver biopsy was done. Liver biopsy confirmed granulomatous hepatitis with stage 2 of 4 fibrosis with numerous foci of non-caseating granulomatous inflammation. With negative acid-fast staining, no fungal organisms, absence of foreign material, normal eosinophil counts and low clinical suspicion for Crohn’s disease, a diagnosis of abdominal sarcoidosis was made. The patient was started on 10 mg prednisone daily and within one week, his albumin- corrected calcium levels improved to 10.4 mg/dL with significant improvement in appetite and mental status. Conclusion: While isolated extrapulmonary sarcoidosis is rare, it is an important cause of hypercalcemia due to elevated 1,25-dihydroxyvitamin D levels. Management of hypercalcemia secondary to sarcoidosis often consists of initiating glucocorticoids which act mainly by inhibition of 1,25-dihydroxy vitamin D synthesis in addition to inhibiting calcium absorption and osteoclast activity.

Ultrasound imaging of abdominal sarcoidosis: State of the art.

Since it has been recognized that sarcoidosis (SA) is not an exclusive disorder of the lungs but can also affect other organs such as the liver and spleen, efforts have been made to define specific imaging criteria for the diagnosis of the single organ involvement, and the concept has been reinforced that the exclusion of alternative causes is important to achieve the correct diagnosis. Ultrasound (US) is a useful tool to evaluate patients with suspected abdominal SA, such as of the liver, spleen, kidney, pancreas and other organs, showing findings such as organomegaly, focal lesions and lymphadenopathy. While the diagnosis of abdominal SA is more predictable in the case of involvement of other organs (e.g., lungs), the problem is more complex in the case of isolated abdominal SA. The recent use of contrast-enhanced ultrasound and endoscopic ultrasound elastography has provided additional information about the enhancement patterns and tissue rigidity in abdominal SA. Here we critically review the role of US in abdominal SA, reporting typical findings and limitations of current evidence and by discussing future perspectives of study.

Hybrid Imaging in Evaluation of Abdominal Sarcoidosis.

To determine the prevalence of abdominal involvement, distribution pattern and evaluate role of hybrid molecular imaging in patients with abdominal sarcoidosis. Between January 2010 and December 2011, 98 patients with chronic sarcoidosis and presence of prolonged symptoms or other findings suggestive of active disease were referred to FDG PET/CT examination. Active disease was found in 82 patients, and they all were screened for the presence of abdominal sarcoidosis on FDG PET/CT. All patients also underwent MDCT and assessment of serum ACE level. Follow up FDG PET/CT examination was done 12.3±5.4 months after the baseline. Abdominal sarcoidosis was present in 31/82 patients with active sarcoidosis. FDG uptake was present in: retroperitoneal lymph nodes (77%), liver (26%), spleen (23%), adrenal gland (3%). Majority of patients had more than two locations of disease. Usually thoracic disease was spread into the extrathoracic localizations, while isolated abdominal sarcoidosis was present in 10% of patients. After first FDG PET/CT examination therapy was changed in all patients. Eleven patients came to the follow up examination where SUVmax significantly decreased in the majority of them. Three patients had total remission, three had absence of abdominal disease but discrete findings in thorax and others had less spread disease. ACE levels did not correlate with SUVmax level. FDG PET/CT can be a useful tool for detection of abdominal sarcoidosis and in the evaluation of therapy response in these patients. Awareness of the presence of intra-abdominal sarcoidosis is important in order to prevent long-standing unrecognized disease.

Abdominal Sarcoidosis Mimicking Peritoneal Carcinomatosis.

We present a patient diagnosed with skin sarcoidosis, breast cancer, pulmonary tuberculosis, and peritoneal sarcoidosis with a past history of colorectal cancer. During stage work up for breast cancer, suspicious lesions on peritoneum were observed in imaging studies. Considering our patient’s history and imaging findings, we initially suspected peritoneal carcinomatosis. However, the peritoneal lesion was diagnosed as sarcoidosis in laparoscopic biopsy. This case demonstrates that abdominal sarcoidosis might be considered as a differential diagnosis when there is a lesion suspected of being peritoneal carcinomatosis with nontypical clinical presentations.

Ultrasonography and Computed Tomographic Manifestations of Abdominal Sarcoidosis in Children.

Sarcoidosis is a multisystem, granulomatous inflammatory disease affecting both pediatric and adult patients. So far in children, very few radiological descriptions of abdominal sarcoidosis manifestations have been reported. The present study describes the frequency and the appearance of abdominal radiologic manifestations in pediatric patients with histologically proven sarcoidosis. We reviewed retrospectively all of the radiological examinations of 22 patients ages 1 to 15 years at diagnosis with proven sarcoidosis evaluated in a university pediatric hospital between 1994 and 2014. The locations of biopsies and the angiotensin-converting enzyme level were reported. The size, shape, and parenchymal homogeneity of the liver and spleen, the presence of abdominal lymph nodes, and abnormalities of the gastrointestinal tract were tabulated. The study included 22 children (mean age: 9.9 ± 2.8 years). The liver was the most frequent location of biopsy (12/22), even without radiological involvement. Abdominal manifestations were present in 11 of 22 children with sarcoidosis. Hepatomegaly was the most frequent abnormality, reported in 8 of 11 cases either homogeneous (n = 7) or nodular (n = 1). Homogeneous lymph node enlargement was noted in 6 of 11 cases and splenomegaly in 4 of 11 cases. No calcification was observed. All patients with initial abdominal sarcoidosis had simultaneous thoracic involvement. Abdominal manifestations in children sarcoidosis are frequent but often nonspecific. Nodular hepatosplenomegaly is rare. All of our patients with abdominal abnormalities had a more specific associated thoracic involvement. Awareness of this association could assist the clinicians in assessing the initial diagnosis of abdominal sarcoidosis in children.

Abdominal sarcoidosis: cross-sectional imaging findings.

Sarcoidosis is a multisystem inflammatory disease of unknown etiology. The lungs and the lymphoid system are the most commonly involved organs. Extrapulmonary involvement is reported in 30% of patients, and the abdomen is the most common extrapulmonary site with a frequency of 50%-70%. Although intra-abdominal sarcoidosis is usually asymptomatic, its presence may affect the prognosis and treatment options. The lesions are less characteristic and may mimick neoplastic or infectious diseases such as lymphoma, diffuse metastasis, and granulomatous inflammation. The liver and spleen are the most common abdominal sites of involvement. Sarcoidosis of the gastrointestinal system, pancreas, and kidneys are extremely rare. Adenopathy which is most commonly found in the porta hepatis, exudative ascites, and multiple granulomatous nodules studding the peritoneum are the reported manifestations of abdominal sarcoidosis. Since abdominal sarcoidosis is less common and long-standing, unrecognized disease can result in significant morbidity and mortality. Imaging contributes to diagnosis and management of intra-abdominal sarcoidosis. In this report we reviewed the cross-sectional imaging findings of hepatobiliary, gastrointestinal, and genitourinary sarcoidosis.

Isolated abdominal sarcoidosis presenting with hypercalcemic crisis: A rare case

Sarcoidosis is a granulomatous disease of unknown origin, with pulmonary findings in more than 90% of patients. Extrapulmonary involvement is common and all organs can be involved (especially lymph nodes, eyes, joints, and central nervous system) but it is rare to find an isolated extrapulmonary disease (

Ultrasound presentation of abdominal tuberculosis in a German tertiary care center

Objective. Abdominal tuberculosis (TB) is a relatively rare disease in most of Europe and the typical clinical and sonographic findings in this setting have not been studied. We aimed to define sonographic findings that should alert an examiner to the possibility of abdominal TB in a low endemic region. Methods. Case records of 17 patients with proven (n = 11) or highly likely (n = 6) abdominal TB detected in the gastrointestinal ultrasound unit at a German tertiary care center in 2003–2013 were analyzed retrospectively. Findings were compared with reported series from high-prevalence regions. Results. While 76% of patients had an immigrant background, only 35% had a condition associated with immunosuppression. Lymphadenopathy was present in all cases of abdominal TB, while it was absent in 28% of patients from a control group with proven abdominal sarcoidosis. Moreover, retroperitoneal lymphadenopathy was significantly more common in TB. Other findings in patients with abdominal TB in descending order of frequency were ascites, altered hepatic texture, splenomegaly, splenic lesions, peritoneal thickening, intestinal wall lesions, hepatic lesions and hepatomegaly. 76% of abdominal TB patients had 2 or more pathological findings. Conclusions. Multiple pathological intra-abdominal findings including lymphadenopathy should alert the examiner to the possibility of abdominal TB.

Erratum to “A Case of Abdominal Sarcoidosis in a Patient with Acute Myeloid Leukemia”

Erratum to “A Case of Abdominal Sarcoidosis in a Patient with Acute Myeloid Leukemia”

A Case of Abdominal Sarcoidosis in a Patient with Acute Myeloid Leukemia

The allogeneic bone marrow transplantation usually preceded by induction chemotherapy, in fit patients, represents the gold standard in the acute myeloid leukaemia. In the last years, many trials have been set up with the view of improving the number of remissions during the induction by adding new drugs. Several early or late side effects have been described in the literature. We herein present a patient with acute myeloid leukaemia patient who, after chemotherapy, developed ascites that turned out to be abdominal sarcoidosis.

Imaging manifestations of abdominal sarcoidosis.

Imaging Manifestations of Abdominal SarcoidosisDavid M. Warshauer1 and Joseph K. T. LeeAudio Available | Share

Hepatic, Splenic, and Gastrointestinal Involvement with Sarcoidosis

Sarcoidosis is a multisystem granulomatous disease of unknown cause that primarily affects the lung and lymphatic systems of the body. However, any organ may be affected, including the abdominal organs and gastrointestinal tract. As with other forms of sarcoidosis, abdominal sarcoidosis presents diagnostic, prognostic, and therapeutic dilemmas. This article reviews the various forms of abdominal sarcoidosis, with particular attention given to methods of diagnosis and treatment options.

Nodular hepatic and splenic sarcoidosis in a patient with normal chest radiograph

Almost all the patients with sarcoidosis have an abnormal chest radiograph, while nodular lesions of both the liver and the spleen is an unusual manifestation of abdominal sarcoidosis. We report a case of a patient with numerous hypodense nodular hepato-splenic lesions on abdominal CT and a normal chest X-ray. Biopsy of an hepatic lesion revealed sarcoidosis.

CT patterns of nodular hepatic and splenic sarcoidosis: a review of the literature.

The purpose of our study was to investigate the less commonly demonstrated CT patterns nodular hepatosplenic sarcoidosis. We studied five women patients, utilizing contrast enhanced incremental or spiral CT. We also performed a literature search of hepatosplenic sarcoid for the years 1966-1995. We found five nodular hepatic and/or splenic patterns of sarcoidosis. We conclude that increased awareness and recognition of these patterns of nodular hepatic/splenic sarcoidosis on CT will greatly improve detection and diagnosis of abdominal sarcoidosis.

Abdominal manifestations of sarcoidosis CT appearances

Sarcoidosis frequently involves the abdomen, although imaging studies often fail to demonstrate disease. The most common computed tomography (CT) findings in abdominal sarcoidosis are hepatosplenomegaly and retroperitoneal adenopathy, followed by focal low-attenuation lesions of the liver and spleen. Other abdominal viscera are involved infrequently. We present four cases of abdominal sarcoidosis demonstrating a range of CT findings.

Hepatic and splenic sarcoidosis: Ultrasound and MR imaging

Abdominal imaging studies may be performed for various indications in patients known to have sarcoidosis. To assess magnetic resonance imaging (MRI) and sonographic ability to detect abnormalities in sarcoidosis patients with abdominal involvement, a prospective study on 18 selected patients was performed. Besides organomegaly, when present, ultrasound demonstrated normal or increased hepatic parenchymal echogenicity, coarsening of the liver parenchyma with or without discrete nodules, focal calcifications, as well as contour irregularity. Splenic discrete nodules were seen on ultrasound in a single patient. Besides organomegaly, MRI abnormalities include abnormal hepatic signal intensity, discrete nodules, contour irregularity, speculation of small hepatic vascular branches, and a high periportal signal intensity. MRI splenic abnormalities include contour irregularity, nodularity, and abnormal signal intensity. The data presented in this study reveals the spectrum of ultrasound and MRI findings in sarcoidosis patients with abdominal organ involvement, potentially enabling the evaluation of the severity of the disease. MRI appears more sensitive than ultrasound for study of abdominal sarcoidosis.

Bloody Ascites in Sarcoidosis

We present the findings in two patients in whom sarcoidosis was manifested by bloody ascites. Granulomas were present on peritoneal surfaces in one case and on the serosal surface of the spleen and liver in the other. Five patients with abdominal sarcoidosis have previously been reported with nonbloody ascites. Although bloody ascites due to sarcoidosis is rare, it is presumably akin to bloody pleural and pericardial effusions which have been previously reported. The long-term prognosis of patients with peritoneal sarcoidosis and ascites appears good.

Abdominal Sarcoidosis with Ascites

A patient with sarcoidosis limited to the abdomen and productive of massive ascites is reported. The patient presented in her last month of pregnancy with a markedly distended abdomen. Laparotomy was performed when abdominal swelling did not subside after delivery of a normal infant. Pathologic findings indicated active sarcoidosis. This is the third such case reported in the literature and the only one further substantiated with a positive Kveim test A patient with sarcoidosis limited to the abdomen and productive of massive ascites is reported. The patient presented in her last month of pregnancy with a markedly distended abdomen. Laparotomy was performed when abdominal swelling did not subside after delivery of a normal infant. Pathologic findings indicated active sarcoidosis. This is the third such case reported in the literature and the only one further substantiated with a positive Kveim test