Sarcoidosis vs. Sarcoid-like reactions: The Two Sides of the same Coin?

Tchernev, Georgi; Tana, Claudio; Schiavone, Cosima; Cardoso, JC; Ananiev, Julian; Wollina, Uwe

doi:10.1007/s10354-014-0269-x

Cited by 53 publications

(65 citation statements)

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“…In this regard, there is increasing evidence of a specific genetic predisposition to a selective sarcoid involvement of the nervous system in some patients (26)(27)(28). Caucasians present more often with manifestations deriving from peripheral nervous system involvement than do African-Americans, and patients with small fiber neuropathy have a high frequency of HLA-DQB1*0602 antigen and non-HLA polymorphic gene occurrence (26).…”

Section: Etiology Of Sarcoidosis and Selective Involvement Of The Nermentioning

confidence: 99%

“…However, these criteria have not given specific indications for each organ involvement and can sometimes result in classification bias or misdiagnosed cases in clinical practice (27,106,107). In the specific form of neurosarcoidosis, clinical findings can manifest without systemic or respiratory symptoms and signs and can be wrongly attributed to other conditions.…”

Section: Diagnostic Approachmentioning

confidence: 99%

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Challenges in the diagnosis and treatment of neurosarcoidosis

et al. 2015

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The diagnosis and treatment of neurosarcoidosis can be very challenging for several reasons. It affects clinically 5%-10% of sarcoidosis patients, but can be found in up to 25% of autopsies. These data reveal that a high percentage of asymptomatic or misdiagnosed cases can be missed at an initial diagnostic approach. Clinical and imaging findings are often non-specific since they can be found in a large number of neurological disorders. Histopathology can also be confounding if not performed by an expert pathologist and not placed in an appropriate clinical context. In this review, we discuss clinical features, laboratory findings, imaging, and histology of neurosarcoidosis, and we report current evidence regarding drug therapy. We conclude that a correct diagnostic approach should include a multidisciplinary evaluation involving clinicians, radiologists, and pathologists and that future studies should evaluate the genetic signature of neurosarcoidosis as they could be helpful in the assessment of this uncommon disease. With head-to-head comparisons of medical treatment for neurosarcoidosis still lacking due to the rarity of the disease and an increasing number of immunomodulating therapies at hand, novel therapeutic approaches are to be expected within the next few years. Key messages Neurosarcoidosis is a rare disorder that affects clinically 5%-10% of sarcoidosis patients, but can be found in up to 25% of autopsies, revealing that a high percentage of asymptomatic or misdiagnosed cases can be missed at an initial diagnostic approach. A multidisciplinary evaluation is useful to achieve a correct diagnosis because clinical and imaging findings are often non-specific. Corticosteroids are the first-line treatment for neurosarcoidosis, followed by steroid-sparing immune-modulating agents if prednisone therapy is insufficient. Dear Author, Please check these proofs carefully. It is the responsibility of the corresponding author to check against the original manuscript and approve or amend these proofs. A second proof is not normally provided. Informa Healthcare cannot be held responsible for uncorrected errors, even if introduced during the composition process. The journal reserves the right to charge for excessive author alterations, or for changes requested after the proofing stage has concluded.The following queries have arisen during the editing of your manuscript and are marked in the margins of the proofs. Unless advised otherwise, submit all corrections using the CATS online correction form. Once you have added all your corrections, please ensure you press the ''Submit All Corrections'' button.Please review the table of contributors below and confirm that the first and last names are structured correctly and that the authors are listed in the correct order of contribution. The diagnosis and treatment of neurosarcoidosis can be very challenging for several reasons. It affects clinically 5%-10% of sarcoidosis patients, but can be found in up to 25% of autopsies. These data reveal that a high percenta...

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Section: Etiology Of Sarcoidosis and Selective Involvement Of The Nermentioning

confidence: 99%

Section: Diagnostic Approachmentioning

confidence: 99%

Challenges in the diagnosis and treatment of neurosarcoidosis

et al. 2015

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“…CS is a granulomatous disorder that can develop either as a part of a multisystemic disease, most often in association with pulmonary sarcoidosis, or can be restricted to the skin (2). Although there are some specific data about the pathogenesis of CS, to a large extent much of what is known about the cutaneous form of the disease is extrapolated from studies on the pathogenesis of sarcoidosis in other organs, namely the lung (2).…”

Section: Pathogenesis Ofcutaneous Sarcoidosis (Es)mentioning

confidence: 99%

“…It is exactly the presence of certain bacterial or viral antigens in the lesions with epitheloid cell granulomas which raises the question of whether the case at hand is a true case of sarcoidosis as an autonomous disease or rather a certain type of sarcoid reaction (2).…”

Section: Pathogenesis Ofcutaneous Sarcoidosis (Es)mentioning

confidence: 99%

“…Although there are some specific data about the pathogenesis of CS, to a large extent much of what is known about the cutaneous form of the disease is extrapolated from studies on the pathogenesis of sarcoidosis in other organs, namely the lung (2). The difficulty in understanding the pathogenesis ofthis disease can be attributed to the elusive causative agent, the lack of a universally accepted animal model, and the protean manifestations of the disease with large variations in different populations and ethnic groups (2,3).…”

Section: Pathogenesis Ofcutaneous Sarcoidosis (Es)mentioning

confidence: 99%

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The “Mystery” of Cutaneous Sarcoidosis: Facts and Controversies

Tchernev

Cardoso

Chokoeva

et al. 2014

Int J Immunopathol Pharmacol

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The reason why the cutaneous form of sarcoidosis is well known in the literature is because of its spectrum of manifestations granting it the fame of a Great Imitator. The mystery shrouding the pathogenesis of this rare cutaneous disease is still there (in spite of the fundamental progress of the various diagnostic methods in current day medicine). The production of the morphological substrate-the epithelioid cell granuloma-which is considered to be characteristic of skin sarcoidosis, could, however, also be the end result of a reaction to i) various specific infectious agents such as Leishmaniasis cutis, coccidioidomycosis, etc., ii) certain residual bacterial or other mycobacterial antigens which, at the moment of setting the diagnosis are-by definition-non-infectious but still immunogenic, as well as iii) different tumor antigens in lesional tissue or other location. Often, differentiating between sarcodiosis and a sarcoid-like reaction, based on the updated criteria for cutaneous sarcoidosis, is problematic to downright impossible. A future characterization of the genetic signature of the two conditions, as well as the implementation of additional mandatory panels for i) the identification of certain infectious or ii) non-infectious but immunogenic and iii) tumor antigens in the epithelioid cell granuloma (or in another location in the organism), could be a considerable contribution to the process of differentiating between the two above-mentioned conditions. This will create conditions for greater accuracy when setting the subsequent therapeutic approaches.

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