Combined Sarcoidosis and Idiopathic Pulmonary Fibrosis (CSIPF): A New Phenotype or a Fortuitous Overlap? Scoping Review and Case Series

Bergantini, Laura; Nardelli, Gabriele; d’Alessandro, Miriana; Montuori, Giusy; Piccioli, Caterina; Rosi, Elisabetta; Gangi, Sara; Cavallaro, Dalila; Cameli, Paolo; Bargagli, Elena

doi:10.3390/jcm11072065

Cited by 6 publications

(7 citation statements)

References 34 publications

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“…Sarcoidosis is well acknowledged as a systemic ailment characterized most aptly upon histological observation of non-caseating sarcoid granulomas [ 151 ]. Its prevalence ranges from 0.001% to 0.04% of individuals, more widely affecting females and those aged 20–40 [ 152 , 153 ]. To date, it carries an unexplainable etiology, shifting considerable diagnostic reliance on biopsy.…”

Section: Leptomeningeal Mimicsmentioning

confidence: 99%

Leptomeningeal Metastasis: A Review of the Pathophysiology, Diagnostic Methodology, and Therapeutic Landscape

Nguyen

Dada

et al. 2023

Current Oncology

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The present review aimed to establish an understanding of the pathophysiology of leptomeningeal disease as it relates to late-stage development among different cancer types. For our purposes, the focused metastatic malignancies include breast cancer, lung cancer, melanoma, primary central nervous system tumors, and hematologic cancers (lymphoma, leukemia, and multiple myeloma). Of note, our discussion was limited to cancer-specific leptomeningeal metastases secondary to the aforementioned primary cancers. LMD mechanisms secondary to non-cancerous pathologies, such as infection or inflammation of the leptomeningeal layer, were excluded from our scope of review. Furthermore, we intended to characterize general leptomeningeal disease, including the specific anatomical infiltration process/area, CSF dissemination, manifesting clinical symptoms in patients afflicted with the disease, detection mechanisms, imaging modalities, and treatment therapies (both preclinical and clinical). Of these parameters, leptomeningeal disease across different primary cancers shares several features. Pathophysiology regarding the development of CNS involvement within the mentioned cancer subtypes is similar in nature and progression of disease. Consequently, detection of leptomeningeal disease, regardless of cancer type, employs several of the same techniques. Cerebrospinal fluid analysis in combination with varied imaging (CT, MRI, and PET-CT) has been noted in the current literature as the gold standard in the diagnosis of leptomeningeal metastasis. Treatment options for the disease are both varied and currently in development, given the rarity of these cases. Our review details the differences in leptomeningeal disease as they pertain through the lens of several different cancer subtypes in an effort to highlight the current state of targeted therapy, the potential shortcomings in treatment, and the direction of preclinical and clinical treatments in the future. As there is a lack of comprehensive reviews that seek to characterize leptomeningeal metastasis from various solid and hematologic cancers altogether, the authors intended to highlight not only the overlapping mechanisms but also the distinct patterning of disease detection and progression as a means to uniquely treat each metastasis type. The scarcity of LMD cases poses a barrier to more robust evaluations of this pathology. However, as treatments for primary cancers have improved over time, so has the incidence of LMD. The increase in diagnosed cases only represents a small fraction of LMD-afflicted patients. More often than not, LMD is determined upon autopsy. The motivation behind this review stems from the increased capacity to study LMD in spite of scarcity or poor patient prognosis. In vitro analysis of leptomeningeal cancer cells has allowed researchers to approach this disease at the level of cancer subtypes and markers. We ultimately hope to facilitate the clinical translation of LMD research through our discourse.

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Section: Leptomeningeal Mimicsmentioning

confidence: 99%

Leptomeningeal Metastasis: A Review of the Pathophysiology, Diagnostic Methodology, and Therapeutic Landscape

Nguyen

Dada

et al. 2023

Current Oncology

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“…Only one patient fulfilling the criteria of progressing fibrotic pulmonary sarcoidosis was found among 167 patients with progressive fibrosing ILDs hospitalised in the years 2012 and 2021 in two hospitals in Osaka, Japan [73]. The usual interstitial pneumonia (UIP) pattern is very rare in fibrotic pulmonary sarcoidosis [28,55,68,74]. It is still unknown whether these rare cases of UIP pattern in sarcoidosis are a form of fibrotic sarcoidosis, constitute a separate condition called combined sarcoidosis and IPF (CSIPF), or are only a fortuitous overlap [74].…”

Section: Saph According To the Predominant Pathophysiological Mechani...mentioning

confidence: 99%

“…The usual interstitial pneumonia (UIP) pattern is very rare in fibrotic pulmonary sarcoidosis [28,55,68,74]. It is still unknown whether these rare cases of UIP pattern in sarcoidosis are a form of fibrotic sarcoidosis, constitute a separate condition called combined sarcoidosis and IPF (CSIPF), or are only a fortuitous overlap [74]. If fibroblastic foci and honeycombing are present in pulmonary sarcoidosis, they are less numerous [55,56].…”

Section: Saph According To the Predominant Pathophysiological Mechani...mentioning

confidence: 99%

Phenotypes of Sarcoidosis-Associated Pulmonary Hypertension—A Challenging Mystery

Kacprzak,

Tomkowski,

Szturmowicz

2023

Diagnostics

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Sarcoidosis has been a well-recognised risk factor for pulmonary hypertension (PH) for a long time, but still, the knowledge about this concatenation is incomplete. Sarcoidosis-associated PH (SAPH) is an uncommon but serious complication associated with increased morbidity and mortality among sarcoidosis patients. The real epidemiology of SAPH remains unknown, and its pathomechanisms are not fully explained. Sarcoidosis is a heterogeneous and dynamic condition, and SAPH pathogenesis is believed to be multifactorial. The main roles in SAPH development play: parenchymal lung disease with the destruction of pulmonary vessels, the extrinsic compression of pulmonary vessels by conglomerate masses, lymphadenopathy or fibrosing mediastinitis, pulmonary vasculopathy, LV dysfunction, and portal hypertension. Recently, it has been recommended to individually tailor SAPH management according to the predominant pathomechanism, i.e., SAPH phenotype. Unfortunately, SAPH phenotyping is not a straightforward process. First, there are gaps in our understanding of undergoing processes. Second, the assessment of such a pivotal element as pulmonary vasculature on a microscopic level is non-feasible in SAPH patients antemortem. Finally, SAPH is a dynamic condition, multiple phenotypes usually coexist, and patients can switch between phenotypes during the course of sarcoidosis. In this article, we summarise the basic knowledge of SAPH, describe SAPH phenotypes, and highlight some practical problems related to SAPH phenotyping.

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“…Another study reported a subset of patients with end-stage sarcoidosis with a UIP pattern who had rapid clinical progression and epithelioid granuloma on lymph node biopsy [ 58 ]. A phenotype of “combined sarcoidosis and IPF” at high risk of rapid progression has been described [ 59 ], but its existence remains open to debate.…”

Section: Hrct Features Of Sapfmentioning

confidence: 99%

Sarcoidosis-associated pulmonary fibrosis: joining the dots

Bandyopadhyay,

Mirsaeidi

2023

Eur Respir Rev

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Sarcoidosis is a multisystem granulomatous disorder of unknown aetiology. A minority of patients with sarcoidosis develop sarcoidosis-associated pulmonary fibrosis (SAPF), which may become progressive. Genetic profiles differ between patients with progressive and self-limiting disease. The mechanisms of fibrosis in SAPF are not fully understood, but SAPF is likely a distinct clinicopathological entity, rather than a continuum of acute inflammatory sarcoidosis. Risk factors for the development of SAPF have been identified; however, at present, it is not possible to make a robust prediction of risk for an individual patient. The bulk of fibrotic abnormalities in SAPF are located in the upper and middle zones of the lungs. A greater extent of SAPF on imaging is associated with a worse prognosis. Patients with SAPF are typically treated with corticosteroids, second-line agents such as methotrexate or azathioprine, or third-line agents such as tumour necrosis factor inhibitors. The antifibrotic drug nintedanib is an approved treatment for slowing the decline in lung function in patients with progressive fibrosing interstitial lung diseases, but more evidence is needed to assess its efficacy in SAPF. The management of patients with SAPF should include the identification and treatment of complications such as bronchiectasis and pulmonary hypertension. Further research is needed into the mechanisms underlying SAPF and biomarkers that predict its clinical course.

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Combined Sarcoidosis and Idiopathic Pulmonary Fibrosis (CSIPF): A New Phenotype or a Fortuitous Overlap? Scoping Review and Case Series

Cited by 6 publications

References 34 publications

Leptomeningeal Metastasis: A Review of the Pathophysiology, Diagnostic Methodology, and Therapeutic Landscape

Leptomeningeal Metastasis: A Review of the Pathophysiology, Diagnostic Methodology, and Therapeutic Landscape

Phenotypes of Sarcoidosis-Associated Pulmonary Hypertension—A Challenging Mystery

Sarcoidosis-associated pulmonary fibrosis: joining the dots

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