scholarly journals Histology of Cardiac Sarcoidosis with Novel Considerations Arranged upon a Pathologic Basis

2022 ◽  
Vol 11 (1) ◽  
pp. 251
Author(s):  
Shu Kato ◽  
Yasuhiro Sakai ◽  
Asako Okabe ◽  
Yoshiaki Kawashima ◽  
Kazuhiko Kuwahara ◽  
...  

Sarcoidosis is a rare disease of isolated or diffuse granulomatous inflammation. Although any organs can be affected by sarcoidosis, cardiac sarcoidosis is a fatal disorder, and it is crucial to accurately diagnose it to prevent sudden death due to dysrhythmia. Although endomyocardial biopsy is invasive and has limited sensitivity for identifying granulomas, it is the only modality that yields a definitive diagnosis of cardiac sarcoidosis. It is imperative to develop novel pathological approaches for the precise diagnosis of cardiac sarcoidosis. Here, we aimed to discuss commonly used diagnostic criteria for cardiac sarcoidosis and to summarize useful and novel histopathologic criteria of cardiac sarcoidosis. While classical histologic observations including noncaseating granulomas and multinucleated giant cells (typically Langhans type) are the most important findings, others such as microgranulomas, CD68+ CD163− pro-inflammatory (M1) macrophage accumulation, CD4/CD8 T-cell ratio, Cutibacterium acnes components, lymphangiogenesis, confluent fibrosis, and fatty infiltration may help to improve the sensitivity of endomyocardial biopsy for detecting cardiac sarcoidosis. These novel histologic findings are based on the pathology of cardiac sarcoidosis. We also discussed the principal histologic differential diagnoses of cardiac sarcoidosis, such as tuberculosis myocarditis, fungal myocarditis, giant cell myocarditis, and dilated cardiomyopathy.

2019 ◽  
Vol 152 (Supplement_1) ◽  
pp. S63-S63 ◽  
Author(s):  
Virian Serei ◽  
Billie Fyfe-Kirschner

Abstract Introduction Cardiac involvement by sarcoidosis, a disease characterized by nonnecrotizing granulomas of unknown etiology, is rare and affects up to 7% of sarcoidosis patients clinically, but with an autopsy prevalence of 15% to 90%. The clinical presentation, as well as macroscopic and microscopic findings in cardiac sarcoidosis patients, varies. Methods Retrospective clinical chart review and gross and microscopic examination of surgical specimen from two adult cardiac sarcoidosis patients with literature review were performed. Results Case 1: A 23-year-old woman with no past medical history presented with 2 months of heart failure symptoms following a viral gastrointestinal infection. Endomyocardial biopsy showed a dense chronic inflammatory infiltrate, without giant cells. Anterior mediastinal lymph node biopsy and left apical core showed effacement by nonnecrotizing granulomas with giant cells and multifocal calcification, supporting cardiac sarcoidosis. Twelve months later, she underwent cardiac transplantation. The explanted heart showed concentric ventricular scarring, which microscopically corresponded to myocardial fibrosis with scattered lymphocytic inflammation with no residual granulomas. Case 2: A 52-year-old man with a medical history significant for ischemic cardiomyopathy and cutaneous sarcoidosis with lung involvement presented with congestive heart failure. Endomyocardial biopsy and left apical core showed myocyte hypertrophy and scattered interstitial inflammation. Mediastinal lymph node showed nonnecrotizing granulomas, consistent with sarcoidosis. Eighteen months later, he underwent cardiac transplantation. The explanted heart showed biventricular dilatation with interstitial fibrosis. There was also mild diffuse inflammation and occasional granulomas with rare focal necrosis. Conclusion Though the gold standard for diagnosing cardiac sarcoidosis is demonstration of myocardial nonnecrotizing granulomas, the sensitivity of endomyocardial biopsy is low. This is due to sampling error and stage of disease. Chronic end-stage disease can lead to fibrous encasement of granulomas and end-stage heart failure, necessitating cardiac transplantation. Current guidelines employ a combination of clinical, radiologic, and histologic criteria for diagnosing cardiac sarcoidosis.


2017 ◽  
Vol 2017 ◽  
pp. 1-4
Author(s):  
Ali Zakaria ◽  
Bayan Al Share ◽  
Issam Turk ◽  
Samira Ahsan ◽  
Waseem Farra

Sarcoidosis is a systemic granulomatous disease of unknown etiology, characterized by the formation of noncaseating granulomas. Gastrointestinal (GI) system involvement that is clinically recognizable occurs in less than 0.9% of patients with sarcoidosis, with data revealing small intestine involvement in 0.03% of the cases. A high index of suspension is required in patients presenting with small-bowel obstruction and previous history of sarcoidosis. Establishing a definitive diagnosis of GI sarcoidosis depends on biopsy evidence of noncaseating granulomas, exclusion of other causes of granulomatous disease, and evidence of sarcoidosis in at least one other organ system. Treatment of GI sarcoidosis depends on symptomatology and disease activity. Herein, we are presenting a case of 67-year-old female patient who had acute small-bowel obstruction at the level of jejunum with postoperative histopathologic evidence of noncaseating granulomatous inflammation with multinucleated giant cells, consistent with sarcoidosis.


2021 ◽  
Vol 7 (1) ◽  
Author(s):  
Tomoko Mizota ◽  
Masato Suzuoki ◽  
Saya Kaku ◽  
Kenichi Mizunuma ◽  
Kazuto Ohtaka ◽  
...  

Abstract Background Sarcoid-like reaction (SLR) is a histological pattern of granulomatous inflammation that is clinically differentiated from sarcoidosis. Since SLR is known to occur in several neoplasias and occasionally causes lymphadenopathy and mimics metastatic malignancy, it needs to be considered whether lymphadenopathy is due to metastasis or SLR for the choice of cancer treatment. Few cases of hepatocellular carcinoma (HCC) with SLR have been reported. Here, a case of HCC with lymphadenopathy diagnosed as SLR without metastasis is presented. Case presentation A 69-year-old woman was admitted to our hospital because of upper abdominal pain. She tested positive for hepatitis C virus ribonucleic acid. Imaging modalities showed an 81 × 65-mm-sized tumor with multiple nodules in segment 3 and a 17 × 12-mm-sized tumor in segment 5 with a common HCC enhancement pattern. In addition, a lymph node in the hepatoduodenal ligament was enlarged at 13 mm in size, suggesting the metastasis of HCC. Hepatectomy of the lateral segment and segment 5 and lymph node dissection in the hepatoduodenal ligament were performed. Both tumors in segments 3 and 5 were pathologically diagnosed as HCC without vessel invasion. The tumors contained necrotic cells and epithelioid cell granulomas with multinucleated giant cells, which is typically observed in sarcoidosis. The dissected lymph nodes also contained epithelioid cell granulomas, as well as giant cells with asteroid bodies. There was no malignancy in the lymph nodes. The pathological findings suggested the coexistence of malignancy and sarcoidosis. However, since the patient did not show any typical findings of pulmonary or cardiac sarcoidosis, the case was diagnosed as HCC with SLR in the primary lesion and regional lymph nodes. Conclusions SLR needs to be considered in the differential diagnosis when a cancer patient develops lymphadenopathy. However, lymphadenopathy due to SLR is indistinguishable from that due to metastasis even when using multiple imaging modalities. Pathological examinations may be helpful for the diagnosis.


Author(s):  
Danielle M. Haanschoten ◽  
Ahmet Adiyaman ◽  
Nils A. ‘t Hart ◽  
Piet L. Jager ◽  
Arif Elvan

2003 ◽  
Vol 127 (3) ◽  
pp. 326-330 ◽  
Author(s):  
Do Youn Park ◽  
Jee Yeon Kim ◽  
Kyung Un Choi ◽  
Jin Sook Lee ◽  
Chang Hun Lee ◽  
...  

Abstract Objective.—To investigate the relationship between various histopathologic features and the results of the tuberculosis (TB)–polymerase chain reaction (PCR) method in routinely submitted histologic specimens for the histopathologic diagnosis of TB. Design.—We used 95 formalin-fixed, paraffin-embedded tissue blocks from 81 patients who were clinically suspected of having TB. We assessed the presence of histopathologic features including well-formed granuloma, poorly formed granuloma, caseous necrosis, and Langhans-type giant cells. We performed nested PCR for IS6110 and Ziehl-Neelsen staining for acid-fast bacilli (AFB). Results.—Of the 81 patients studied, 53 patients had chronic granulomatous inflammation, whereas 28 patients had only chronic inflammation without definite granulomatous inflammation. Of the 53 cases with chronic granulomatous inflammation, 17 (32%) were AFB positive and 36 (68%) were TB-PCR positive. Among cases with chronic granulomatous inflammation, the percentage that were positive and negative by TB-PCR differed significantly with the presence of various histopathologic features. All of the 13 cases with well-formed granuloma, caseous necrosis, and Langhans-type giant cells were TB-PCR positive; however, 10 (36%) of the 28 cases with chronic inflammation without granulomatous lesions were also TB-PCR positive. Conclusions.—TB-PCR is a rapid, sensitive method for the diagnosis of TB in routinely processed formalin-fixed, paraffin-embedded histologic specimens and is readily available in histopathology laboratories. We recommend use of TB-PCR when TB is suspected clinically, especially in cases of chronic inflammation without definite evidence of granulomatous inflammation.


Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Seiji Takashio ◽  
Megumi Yamamuro ◽  
Toshihisa Anzai ◽  
Hisao Ogawa

Background: Cardiac amyloidosis (CA) is an important differential diagnosis in patients with myocardial hypertrophy. The precise diagnosis of CA requires endomyocardial biopsy to demonstrate amyloid deposition, but this procedure is relatively invasive and cannot be performed routinely. Therefore, it is important to increase pretest probability of CA in patients with myocardial hypertrophy by noninvasive modalities. Because it is well known that cardiac troponin level is elevated in patients with CA, we hypothesized that increased level of cardiac troponin T contributes to diagnosis of CA in patients with myocardial hypertrophy using high sensitive assay (hs-TnT). Methods and Results: Among nonischemic patients with myocardial hypertrophy (interventricular septal thickness≧ 12 mm), hs-TnT level was measured in 28 CA patients (senile systemic amyloidosis: 13, AL amyloidosis: 14, familial amyloidosis: 1) proven amyloidosis pathologically and 29 non-CA patients proven by endomyocardial biopsy. It was significantly higher in CA patients than non-CA patients (0.075 [0.047-0.116] ng/ml vs. 0.013 [0.009-0.019] ng/ml; p<0.001: Figure). Receiver operating characteristic analysis selected 0.030 ng/ml as the best cutoff value of diagnosis for CA, with a sensitivity and specificity of 96% and 93%, respectively and area under the curve of 0.98 (95% confidence interval 0.94 to 1.00, p<0.001). Conclusions: Increased level of hs-TnT (>0.030 ng/ml) is highly suggestive of CA in patients with myocardial hypertrophy. These patients need additional diagnostic approach for CA.


2018 ◽  
Vol 131 (7-8) ◽  
pp. 186-187
Author(s):  
Petr Kuchynka ◽  
Tomas Palecek ◽  
Lukas Lambert ◽  
Antonin Fikrle ◽  
Ivana Vitkova ◽  
...  

2020 ◽  
Author(s):  
Robert P Baughman ◽  
Mary Beth Scholand

Sarcoidosis is a systemic disease characterized by the presence of noncaseating granulomas, which accumulate in affected organs. The incidence, organ involvement, and disease severity depend on environmental exposures and host factors. The cause of sarcoidosis remains unknown. Any organ can be affected; however, involvement of the lung, heart, and nervous system contributes most to morbidity and mortality. This review discusses the epidemiology, etiology, genetics, pathogenesis, diagnosis (including clinical manifestations), differential diagnosis, management, complications, and prognosis of sarcoidosis. Figures depict the pathogenesis of sarcoidosis, radiographic stages of sarcoidosis, contrast-enhanced magnetic resonance imaging of a patient with neurosarcoidosis, noncaseating granuloma from a tissue biopsy of a patient with sarcoidosis, various manifestations of sarcoidosis, approach to the use of anti-inflammatory therapy for sarcoidosis, and an algorithm for symptomatic disease.  This review contains 7 highly rendered figures, 7 tables, and 127 references Keywords: cardiac sarcoidosis, hilar lymphadenopathy, Löfgren syndrome, neurosarcoidosis, noncaseating granuloma, sarcoidosis, uveitis 


2015 ◽  
Vol 7 (1) ◽  
pp. 101-104 ◽  
Author(s):  
Ryuji Yajima ◽  
Yasuko Toyoshima ◽  
Yoko Wada ◽  
Tetsuya Takahashi ◽  
Hiroyuki Arakawa ◽  
...  

Central nervous system (CNS) involvement, such as pachymeningitis and/or cerebrovascular events, is rare in patients with granulomatosis with polyangiitis (GPA). Furthermore, the details of pathological examinations of cases have rarely been described. We describe a case of GPA that manifested as an isolated paranasal sinus disease that invaded the subarachnoid space and caused a hemorrhagic venous infarction. We also describe the pathological characteristics of the biopsied brain material from the successful decompressive craniectomy. In particular, granulomatous inflammation with geographic necrosis and multinucleated giant cells were observed in the perivascular area of the thickened dura mater and leptomeninges. Small vessels in the meninges were involved in the granulomatous lesions, and the lumens of the veins were often occluded. In the cerebral cortices and white matter in these areas, hemorrhagic infarction was widely observed. We suggest that our findings represent a novel mechanism of CNS involvement in GPA. Moreover, we believe that the emergency decompressive craniectomy and partial lobectomy for the cerebral infarction in this patient with GPA likely contributed to his survival.


2018 ◽  
pp. bcr-2018-225436
Author(s):  
Sarah Pratt ◽  
Arthur Henderson ◽  
Stuart Gillett

An 80-year-old woman presented with dysphagia, odynophagia and progressive weight loss. Initial investigations suggested a large base of tongue squamous cell carcinoma. At panendoscopy, biopsies were taken which revealed granulomatous inflammation with multinucleated giant cells and areas of caseous necrosis. Acid-fast bacilli were identified on Ziehl-Neelsen stain and the appearances were consistent with Mycobacterium bovis. Closer inspection of the patient’s medical history revealed that she had previously undergone right hemicolectomy for an ascending colon stricture. The histology had shown granulomatous inflammation consistent with intestinal tuberculosis but no systemic treatment had been instigated at the time. This case highlights the importance of taking a detailed clinical history as well as the need to be cognisant of tuberculosis masquerading as cancer in the head and neck.


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