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The current review discusses the diagnosis and management of nonorgan-related symptoms that commonly arise in the setting of systemic sarcoidosis. Fatigue, small fiber neuropathy (SFN) and neuropsychological symptoms are highlighted.The debilitating effects of chronic nonorgan-based symptoms in sarcoidosis have led to recent studies focusing on incidence rates, contributing factors and potential therapeutic strategies. In a web-based survey of over 1000 sarcoidosis patients, the most common symptom was fatigue, which was reported by over 90% of participants, whereas memory loss and concentration problems were reported in 50%. SFN was also common, and may be diagnosed with tools such as skin biopsy measurement of intraepidermal nerve fibers and corneal confocal microscopy. In a recent cohort study of SFN patients, serologic evaluation demonstrated other contributing causes such as diabetes and vitamin B12 deficiency, which warrant-specific treatment. Finally, physical inactivity in patients with sarcoidosis correlated with lower quality-of-life (QOL) scores and possibly fatigue. Multidisciplinary programs that include physical therapy, patient education and psychological support were found to improve fatigue and mood disorders.Recognition of nonorgan-related symptoms and their impact on patient QOL is essential to optimal treatment of the sarcoidosis patient.

Objective. To identify clinical and morphofunctional changes in the lacrimal gland in sarcoidosis.Material and methods. Ultrasonography was conducted in 53 patients (106 eyes) with a verified diagnosis of systemic sarcoidosis (7 men and 46 women) whose age was 31 to 72 years.Results. Different types of lacrimal gland structural changes in sarcoidosis were detected. Comparing the acoustic and functional findings revealed a high correlation (r > 0.7) between these parameters as vascularization index, a reflex component of tear secretion, and the presence of tubular structures. Types of changes in the acoustic structure of the lacrimal glands were identified, which were designated as edematous and polymorphic changes, as well as atrophic changes with a necrotic component.Conclusion. The findings can be used to diagnose lacrimal gland changes in patients suffering from sarcoidosis.

An unusual case is presented of a 29 year-old Caucasian woman with a granuloma in an old scar in the right periocular region as a first clinical sign of a systemic sarcoidosis. An excisional biopsy was performed, for which the histological diagnosis was a chronic non-necrotising granulomatous inflammation, suggestive of scar sarcoidosis. The lesion re-appeared one year after initial treatment, and was treated with intralesional depot steroids, showing adequate progression. This disease occurs more frequently in wound areas where there are foreign bodies and could be the first sign of systemic sarcoidosis.

Hepatic granulomas are a common finding in systemic sarcoidosis, but most patients remain asymptomatic. Elevated alkaline phosphatase is the most common sign of hepatic sarcoidosis (HS). Lacking a specific diagnostic test, the diagnosis of HS is one of exclusion. Therapy may be indicated in a minority of patients to control symptoms, but the effects of therapy in the natural history of HS are unknown.

Importance The Neurosarcoidosis Consortium Consensus Group, an expert panel of physicians experienced in the management of patients with sarcoidosis and neurosarcoidosis, engaged in an iterative process to define neurosarcoidosis and develop a practical diagnostic approach to patients with suspected neurosarcoidosis. This panel aimed to develop a consensus clinical definition of neurosarcoidosis to enhance the clinical care of patients with suspected neurosarcoidosis and to encourage standardization of research initiatives that address this disease. Observations The work of this collaboration included a review of the manifestations of neurosarcoidosis and the establishment of an approach to the diagnosis of this disorder. The proposed consensus diagnostic criteria, which reflect current knowledge, provide definitions for possible, probable, and definite central and peripheral nervous system sarcoidosis. The definitions emphasize the need to evaluate patients with findings suggestive of neurosarcoidosis for alternate causal factors, including infection and malignant neoplasm. Also emphasized is the need for biopsy, whenever feasible and advisable according to clinical context and affected anatomy, of nonneural tissue to document the presence of systemic sarcoidosis and support a diagnosis of probable neurosarcoidosis or of neural tissue to support a diagnosis of definite neurosarcoidosis. Conclusions and Relevance Diverse disease presentations and lack of specificity of relevant diagnostic tests contribute to diagnostic uncertainty. This uncertainty is compounded by the absence of a pathognomonic histologic tissue examination. The diagnostic criteria we propose are designed to focus investigations on NS as accurately as possible, recognizing that multiple pathophysiologic pathways may lead to the clinical manifestations we currently term NS. Research recognizing the clinical heterogeneity of this diagnosis may open the door to identifying meaningful biologic factors that may ultimately contribute to better treatments.

Purpose: This work reports a case of systemic sarcoidosis presenting with ischemic ocular manifestations without uveitis. Methods: This case report describes a 28-year-old White male who presented with pain, redness, and blurry vision of his left eye for 1 month who was found to have proliferative retinopathy and neovascular glaucoma. Results: Normal fasting blood glucose and glycated hemoglobin A1c levels warranted further investigation. Abnormal findings from chest x-ray of hilar lymphadenopathy as well as an elevated angiotensin-converting enzyme level were suggestive of sarcoidosis, and they were further confirmed by histopathology derived through bronchoscopy. Conclusions: Systemic sarcoidosis may present with rapidly progressing ischemic retinopathy without signs of inflammation, which may obscure the diagnosis. Such presentation may be misdiagnosed, which may result in delayed treatment and poor outcome.

E.A.Burylova, L.A.Mamaeva, A.S.Berdnikova, O.K.Fedorova Ural Scientific Research Institute of Phtysiopulmonology — Branch of the National Medical Research Center of Phtysiopulmonology, Yekaterinburg, Russian Federation Abstract Ocular manifestations of systemic sarcoidosis may emerge at any disease stage. Ocular sarcoidosis is typically characterized by uveitic presentations that predominantly involve the choroid of anterior eye segment. However, orbital pseudotumor, dacryoadenitis, and other rare presentations (optic neuritis, dacryocystitis etc.) also occur in sarcoidosis. Threatened vision loss and poor quality of life are the reasons to prescribe systemic steroids in generalized sarcoidosis. Chest CT is required to identify respiratory diseases when sarcoidosis manifests as uveitis or granulomatous orbital inflammation. Multidisciplinary diagnostic and treatment approach to suspected sarcoidosis provides timely verified diagnosis and management strategy. This paper addresses case report of systemic sarcoidosis in a 34-year-old man. The initial manifestation was vision loss and left orbital pseudotumor. The enlarged subclavian lymph node and its biopsy were suspicious of granulomatous inflammation. Chest CT identified disseminated pulmonary lesions and intrathoracic lymphadenopathy that are typical of sarcoidosis. The results of multidisciplinary examina tions verified systemic sarcoidosis. Systemic steroids resulted in clinical and radiological improvement and medicinal regression of the disease. Keywords: sarcoidosis, orbital pseudotumor, dacryoadenitis, adenopathy, computed tomography, multidisciplinary approach. For citation: Burylova E.A., Mamaeva L.A., Berdnikova A.S., Fedorova O.K. Orbital lesion (pseudotumor and dacryoadenitis) is a manifestation of systemic sarcoidosis. Russian Journal of Clinical Ophthalmology. 2021;21(1):40–44. DOI: 10.32364/2311-7729-2021-21-1-40-44.

International audience; Recently, concerns have been raised about an increased risk of cardiac sarcoidosis in patients with sarcoid uveitis. While cardiac sarcoidosis has a high mortality burden, there is still a lack of precise data on this association. The objective of this study is to describe the frequency and type of cardiac complications associated with sarcoidosis of a large cohort of patients with sarcoid uveitis. We analyzed the cardiac outcomes of a monocentric retrospective cohort of consecutive adults with a diagnosis of sarcoid uveitis between January 2004 and March 2020 in a tertiary French university hospital. A total of 294 patients with a final diagnosis of sarcoid uveitis were included. At final follow-up, seven (2.4%) patients of the cohort had cardiac sarcoidosis. Cardiac sarcoidosis was more frequent among patients with previously reported systemic sarcoidosis (p = 0.008). The prevalence of cardiac sarcoidosis among patients with sarcoid uveitis is low, but patients with previously diagnosed sarcoidosis or those who develop systemic sarcoidosis during follow-up appear to be at increased risk.

Introduction: We present a case of a patient with systemic sarcoidosis with cardiac involvement who presented with palpitations and was noted to be in non-sustained ventricular tachycardia (NSVT) after being tapered down the prednisone dose and being started on methotrexate. Clinical Course: 49-year-old female presented with symptoms of palpitations for 10 day prior to presentation with associated lightheadedness. Her past medical history included cardiac sarcoidosis, atrial tachycardia and complete heart block with a pacemaker placement. Upon checking her remote pacemaker interrogation during the current admission she was found to have recurrent episodes of non-sustained ventricular tachycardia. Patient was diagnosed with sarcoidosis 4 months prior to presentation and was started on 50 mg of prednisone which was tapered down to 30 mg 2 months prior to patient’s presentation due to weight gain along with initiation of methotrexate therapy .Patient was started on amiodarone 200 mg daily with a loading dose of 400 TID while admitted. Her prednisone was increased temporarily and weekly methotrexate was continued. The patient was found to have resolution of symptoms and did not have recurrence of arrhythmia on telemetry. She was upgraded to ICD placement during the same admission and was discharged with follow up. Conclusions: Ventricular tachycardia (VT) resulting from myocardial inflammation is the most common arrhythmia in cardiac sarcoidosis (CS), found in up to 23% of the patients. Antiarrhythmic drugs such as amiodarone are standard treatment for VT in patients with CS. However, corticosteroid therapy has shown to be beneficial for ventricular arrhythmias (VA) as demonstrated in some studies while even worsening ventricular arrhythmias in minority of the patients. In our patient, the tapering of the prednisone dose despite introduction of methotrexate resulted in the recurrence of ventricular tachycardia which eventually resolved with the re-administration of the original dose of prednisone. This indicates that more definitive immunosuppression therapy guidelines are required for patients with CS.

Background Sarcoidosis is a multisystem granulomatous disease of unknown etiology. The incidence is higher in women than in men, according to some studies. Studies regarding prevalence and characteristics of cutaneous sarcoidosis in our region are scarce. Objective This study aimed to describe the characteristics of patients with cutaneous sarcoidosis and to estimate its prevalence. Methods A cross-sectional study was conducted of patients with cutaneous sarcoidosis between January 1, 2004 and April 30, 2019 at the Hospital Italiano de Buenos Aires in Argentina. We included all patients age >17 years with biopsy-proven cutaneous sarcoidosis. Isolated cutaneous sarcoidosis was defined as the presence of epithelioid noncaseating granulomas on a skin biopsy without further evidence of systemic involvement. To estimate period prevalence, we only considered the subgroup of patients affiliated with our private health system. Results A total of 38 patients with cutaneous sarcoidosis were included. The median age at the time of diagnosis was 55.5 years. There was a striking female predominance in our series (73.7%). Overall, 15 patients (39.5%) had isolated cutaneous sarcoidosis and 23 (60.5%) had systemic sarcoidosis with cutaneous involvement. The median follow-up of the study population from histological diagnosis was 50 months (interquartile range, 24–10 months). Regarding skin involvement, 28 patients (73.7%) presented with only sarcoidosis-specific lesions, 6 (15.8%) presented with erythema nodosum, and 4 (10.5%) presented with both sarcoidosis-specific lesions and erythema nodosum. Treatment was given to 29 patients (73.6%), with systemic and topical corticosteroids being the most frequent. The crude prevalence of cutaneous sarcoidosis was 16.9 (95% confidence interval, 10.6–25.5) per 100,000 persons. Conclusion One of the major findings of our study was that 40% of patients had isolated cutaneous sarcoidosis.

Background 2-deoxy-2-[18F]fluoro-d-glucose’s (FDG) biodistribution limits the evaluation of cardiac sarcoidosis (CS) and neurosarcoidosis (NS). While protocols for cardiac suppression exist, they can be inconvenient for patients and lead to incomplete cardiac suppression in many cases. Furthermore, FDG PET is limited in the detection of neurosarcoidosis due to an inability to suppress high level of physiological uptake within the brain. 3′-deoxy-3′-[18F]fluorothymidine (FLT) has been shown to accumulate in sarcoidosis lesions and this tracer lacks significant physiological myocardial and brain uptake, suggesting that this tracer may be useful for the assessment of sarcoidosis, including CS and NS, without the need for patient preparation. This prospective pilot study examined the performance of FLT vs FDG PET for systemic sarcoidosis, including cardiac and neural involvement. Materials and methods Fourteen subjects with sarcoidosis were prospectively recruited and imaged with FDG- and FLT-PET. Two blinded, experienced readers independently reviewed the FLT-PET and FDG-PET images. Lesion distribution was compared between FLT and FDG. Agreement between FLT- and FDG-PET was determined using Cohen’s kappa and the intra-class correlation coefficient. Inter-observer variability of FLT and FDG-PET was assessed. Results Twelve subjects had CS as per Heart Rhythm Society criteria and 1 had NS. FLT-PET was positive in 12 (86%), and FDG-PET in 11 (79%), with cardiac uptake present in 6 (50%) and 7 (58%) of subjects with CS, respectively. The subject with NS demonstrated uptake on both FLT and FDG-PET, with more lesions on FLT. There were no significant differences in the anatomical distribution of lesions between FLT and FDG. SUVs were significantly (p p = 0.90) after adjusting for blood pool activity (2.8 ± 1.4 vs 2.8 ± 1.1, respectively). Agreement between FLT- and FDG-PET was good to excellent for the diagnosis of sarcoidosis, lung involvement, CS, and NS (κ = 0.76, 0.69, 0.86, and 1.0, respectively). Inter-observer agreement for FLT was excellent for diagnosing sarcoidosis, CS and NS (κ = 0.81, 0.85, and 1.0, respectively) and comparable to that of FDG. Conclusions FLT-PET may be useful for the assessment of systemic sarcoidosis, as well as cardiac and neural involvement.

A 40‐year‐old man with bilateral hilar lymphadenopathy in chest X‐ray and the λ‐sign in gallium‐67 scintigraphy was subsequently diagnosed with systemic sarcoidosis according to the findings of bronchoalveolar lavage and histopathological examinations of transbronchial lung biopsies. Identifying the λ‐sign could be a valuable clue to the early diagnosis of sarcoidosis., The λ‐sign strongly suggests the diagnosis of sarcoidosis because all reported patients with the λ‐sign were subsequently diagnosed as having sarcoidosis. Identifying the λ‐sign with gallium‐67 scintigraphy could be a valuable clue to the early diagnosis of sarcoidosis.

Cardiac sarcoidosis is a rare immunologically driven process seen in 2% to 5% of patients with systemic sarcoidosis. We present a 31-year-old woman who presented after a ventricular fibrillation cardiac arrest. A comprehensive diagnostic workup was unrevealing. Despite negative cardiac magnetic resonance imaging, positron emission tomography facilitated the diagnosis. This case illustrates both the limitations of the diagnostic workup of sarcoidosis and the usefulness of positron emission tomography in the early diagnosis of cardiac sarcoidosis.

To investigate if T1 and T2 mapping is able to differentiate between diseased and healthy myocardium in patients with systemic sarcoidosis, and to compare the standard mapping measurement (measurement within the whole myocardium of the midventricular short axis slice, SAX) to a more standardized method measuring relaxation times within the midventricular septum (ConSept). 24 patients with biopsy-proven extracardiac sarcoidosis and 17 healthy control subjects were prospectively enrolled in this study and underwent CMR imaging at 1.5 T including native T1 and T2 mapping. Patients were divided into patients with (LGE+) and without (LGE-) cardiac sarcoidosis. T1 and T2 relaxation times were compared between patients and controls. Furthermore, the SAX and the ConSept approach were compared regarding differentiation between healthy and diseased myocardium. T1 and T2 relaxation times were significantly longer in all patients compared with controls using both the SAX and the ConSept approach (p 0.05). However, LGE+ and LGE- patients showed no significant differences in T1 and T2 relaxation times regardless of the measurement approach used (ConSept/SAX) (p 0.05). Direct comparison of ConSept and SAX T1 mapping showed high conformity in the discrimination between healthy and diseased myocardium (Kappa = 0.844). T1 and T2 mapping may not only enable noninvasive recognition of cardiac involvement in patients with systemic sarcoidosis but may also serve as a marker for early cardiac involvement of the disease allowing for timely treatment. ConSept T1 mapping represents an equivalent method for tissue characterization in this population compared to the SAX approach. Further studies including follow-up examinations are necessary to confirm these preliminary results. · Mapping may enable noninvasive recognition of cardiac involvement in patients with systemic sarcoidosis. · Mapping may serve as a marker for early cardiac involvement in patients with systemic sarcoidosis. · The ConSept approach can be used as an alternative measuring method in sarcoidosis patients.· Dabir D, Luetkens J, Kuetting D et al. Myocardial Mapping in Systemic Sarcoidosis: A Comparison of Two Measurement Approaches. Fortschr Röntgenstr 2021; 193: 68 - 76.ZIEL: Untersuchung, ob T1- und T2-Mapping bei Patienten mit systemischer Sarkoidose zwischen krankem und gesundem Myokard unterscheidet. Vergleich der herkömmlichen Messmethode (Messung im gesamten mittventrikulären Myokard der kurzen Herzachse, SAX) mit einem standardisierteren Messansatz, bei dem die Relaxationszeiten ausschließlich im mittventrikulären Septum gemessen werden (ConSept). 24 Patienten mit bioptisch gesicherter extrakardialer Sarkoidose und 17 gesunde Probanden wurden prospektiv in diese Studie eingeschlossen und an einem 1,5-Tesla-Magneten unter anderem mittels nativem T1- und T2-Mapping untersucht. Patienten wurden im Rahmen der Auswertung unterteilt in Patienten mit (LGE+) und Patienten ohne späte myokardiale Kontrastmittelaufnahme (LGE–). Des Weiteren wurden die SAX- und die ConSept-Messmethode auf ihre Differenzierbarkeit zwischen gesundem und krankem Herzmuskelgewebe untersucht. Alle Patienten wiesen unabhängig von der gewählten Messmethode (ConSept bzw. SAX) signifikant längere T1- und T2-Relaxationszeiten im Vergleich zu den gesunden Probanden auf (p 0,05). Jedoch zeigten sich keine signifikant unterschiedlichen Relaxationszeiten zwischen Patienten mit (LGE+) und ohne späte myokardiale Kontrastmittelaufnahme (LGE–), unabhängig von der gewählten Messmethode (ConSept/Sax) (p 0,05). Der direkte Vergleich zwischen den Messmethoden ConSept und SAX zeigte beim T1-Mapping eine hohe Übereinstimmung in Bezug auf die Differenzierung zwischen krankem und gesundem Myokard (Kappa = 0,844). Mittels T1- und T2-Mapping ist es möglich, bei Patienten mit systemischer Sarkoidose zwischen krankem und gesundem Myokard zu unterscheiden. Darüber hinaus stellt das myokardiale Mapping einen möglichen Marker für die Früherkennung einer kardialen Beteiligung dar, was eine zeitgerechte Behandlung begünstigt. ConSept-T1-Mapping stellt einen gleichwertigen Messansatz zur SAX-Methode bei Sarkoidose-Patienten dar. Weitere Studien, inklusive Follow-up-Studien, sind notwendig, um diese vorläufigen Ergebnisse zu bestätigen. · Mapping kann zwischen krankem und gesundem Myokard bei Patienten mit systemischer Sarkoidose unterscheiden. · Mapping könnte zur Früherkennung einer kardialen Sarkoidose beitragen. · ConSept-T1-Mapping stellt einen alternativen Messansatz zur SAX-Methode bei Sarkoidose-Patienten dar.

is missing (Short communication)

We report a case of sudden death due to systemic sarcoidosis in a fifty-four year old male who was reportedly healthy. A computerized tomography (CT) scan was performed before the autopsy. It showed cardiomegaly with hilar and abdominal lymphadenopathy. The autopsy showed pale yellow plaque deposition on the heart surface which was infiltrating the myocardium. Histological examination of the heart, lungs, liver, and spleen showed extensive sarcoid granulomata which helped in establishing the cause of death.

Background Lesions of the skull make up a small but important part of neurosurgical practice. Several systemic disorders may involve the cranial vault including neoplastic and non-neoplastic conditions. Sarcoidosis of the skull is a little-known cause of calvarial involvement that has been rarely reported in the literature. The available information about skull sarcoidosis (SS) is sparse and is not well described; for this reason, we consider that a detailed description of this uncommon condition is necessary. Methods An illustrative case of SS is presented; in addition, a PubMed and Scopus search adhering to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines was performed to include studies reporting patients with SS. Different information was analyzed in these cases to describe the characteristics of this condition. Also, different sources of literature were analyzed to complete the description of this clinical entity. Results The search yielded 22 cases of patients with SS showing a variety of clinical manifestations. All studies were case reports. Most patients diagnosed with SS had no previous history of systemic sarcoidosis. Different characteristics of SS are analyzed and described in this paper. Conclusions The information collected from this review shows that SS is a rare condition that frequently is observed in patients without previous diagnosis of sarcoidosis. SS may manifest in different ways, and even may be found incidentally in some patients. The diagnosis of SS should be considered when multiple lytic skull lesions are observed, especially in cases of patients without a previous history of malignancy.

Permanent make-up tattooing as a cosmetic procedure is gaining popularity especially among women. Although it is considered a relatively safe intervention, the ink used can rarely be a trigger of sarcoidosis. Uveitis can occur as part of this inflammatory process. In this study, we describe two ladies presented with tattoo-associated uveitis as the first manifestations of systemic sarcoidosis. They developed intermediate uveitis shortly after skin inflammation several months after permanent make-up tattooing of eyebrows. Lung involvement, high ACE levels, and negative PPD were present. Skin granuloma formation was diffuse over the area of tattoo in one patient and localized in the other one. This is the first report of uveitis following make-up tattoo.

In this paper, we presented a patient, who applied to our clinic because of infertility. After an intensive investigation, we diagnosed systemic sarcoidosis with testicular involvement. Urogenital sarcoidosis is a rare and insidious condition, however, it can lead to infertility. Therefore, following the diagnosis, we applied systemic steroid therapy to the patient. Within one year, the patient had a child without assisted reproductive techniques. According to our experience, in this case, we concluded that infertility without an etiology should be investigated elaborately. Furthermore, urogenital sarcoidosis should keep in mind as a rare etiology. Keywords: Infertility, Sarcoidosis, Secondary infertility, Testis, Urogenital sarcoidosis

Key Clinical Message Progressive multifocal leukoencephalopathy (PML) is a severe infectious brain disease with lethal outcome mainly seen in immunocompromised subjects. Herein, we describe a new form of PML with different outcome which was observed in patients suffering from systemic sarcoidosis. Thus, we raise the question whether preexisting sarcoidosis might predispose for or even trigger PML.

Current guidelines for the diagnosis of cardiac sarcoidosis (CS) include late gadolinium enhancement (LGE) cardiac magnetic resonance (CMR), which provides the excellent negative predictive value for ruling out future cardiac events ([1][1],[2][2]). However, early diagnosis of CS remains clinically

Sarcoidosis is a systemic disease of unknown aetiology characterized by the presence of non-caseating epithelioid cell granulomas in multiple organs, mainly the lungs and the lymphatic system. It is also one of the leading cause of inflammatory eye diseases. For the past 70 years, sarcoidal granulomas on tattoos and permanent make-up have been documented. They can be the first and sometimes unique cutaneous manifestation of systemic sarcoidosis. A few cases of sarcoidosis with uveitis and granulomatous reactions within tattoos have been described. However, since the end 60s, a singular entity has been reported associating isolated uveitis with granulomatous cutaneous reaction restricted to tattoos in the notable absence of systemic sarcoidosis. It remains unclear whether this entity must be distinguished from sarcoidosis. This review summarizes the currently available data on this topic and compares cases of sarcoidosis with granulomatous tattoo reaction and uveitis to the cases without notable sarcoidosis. We propose the acronym TAGU (TAttoo Granulomas with Uveitis) as an exclusion diagnosis that emcompasses the patients for whom we fail to find any sarcoidosis or other causes after extensive investigation.

International audience; This joint position paper illustrates the role and the correct use of echocardiography, radionuclide imaging with 18F-fluorodeoxyglucose positron emission tomography, radionuclide myocardial perfusion imaging and cardiovascular magnetic resonance imaging for the evaluation and management of patients with known or suspected cardiac sarcoidosis. This position paper will aid in standardizing imaging for cardiac sarcoidosis and may facilitate clinical trials and pooling of multi-centre data on cardiac sarcoidosis. Proposed flow charts for the work up and management of cardiac sarcoidosis are included.

Background:Neurosarcoidosis is a serious and infrequent complication of sarcoidosis. Data on Biologic Therapy (BT) efficacy is scarce.Objectives:To assess in neurosarcoidosis a) its prevalence and associations of clinical clusters and b) efficacy and safety of BT.Methods:Study of neurosarcoidosis from a large cohort (n=384) of all consecutive patients diagnosed with sarcoidosis from January 1, 1999 to December 31, 2019 in a single University hospital.Results:30 (19 women/ 11 men) out of 384 (7.8%) patients had neurosarcoidosis. Mean age at diagnosis was 41.8±15.8 years. Clusters of clinical associations were lungs (n=23, 76.7%) and joints (n=15, 50.0%) (Figure 1). The underlying neurological manifestations were chronic headache (n=13, 43.4%), peripheral neuropathy (n=6, 20.0%), cranial neuropathy (n=5, 16.7%), spinal cord abnormalities (n=3, 10.0%) and aseptic meningitis (n=3, 10.0%). 26 (86.7%) patients received oral corticosteroids (mean maximum dose 50±19.2mg) and 7 (23.3%) iv corticosteroids. In addition, 18 (60.0%) patients received conventional immunosuppressants and 12 (40.0%) BT. 4 (13.3%) did not have any treatment. Table 1 shows the main clinical features and treatment.Figure 1.Clusters of clinical associations in neurosarcoidosis12 patients received treatment with 14 BT. Monoclonal anti-TNFα (n=10, 83.3%) was the most used BT. 10 (83.3%) patients treated with BT achieved clinical remission. No serious adverse effects were observed.Conclusion:Neurosarcoidosis was observed in 7.8% of systemic sarcoidosis. Most patients treated with BT, especially monoclonal anti-TNFα, achieved clinical remission.Table 1.Main clinical features and treatment of 30 patients with neurosarcoidosis.n (%)Conventional immunosuppressant, n (%)monoclonal anti-TNFα, n (%)Etarnecept, n (%)Tocilizu-mab, n (%)Secukinu-mab, n (%)Rituxi-mab, n (%)Chronic headache13 (43.4)5 (38.5)2 (15.4)0 (0)01 (7.7)1 (7.7)Peripheral neuropathy6 (20.0)4 (66.7)3 (50.0)1 (16.7)000Cranial neuropathy5 (16.7)4 (80.0)3 (60.0)0000Spinal cord abnormalities3 (10.0)1 (33.3)1 (33.3)0000Aseptic meningitis3 (10.0)2 (66.7)2 (66.7)01 (33.3)00Disclosure of Interests:Alba Herrero-Morant: None declared, Lara Sanchez-Bilbao: None declared, Iñigo González-Mazón: None declared, David Martínez-López: None declared, José Luis Martín-Varillas Grant/research support from: AbbVie, Pfizer, Lilly, Janssen, and Celgene, Raúl Fernández-Ramón: None declared, Miguel Á. González-Gay Speakers bureau: AbbVie, Pfizer, Roche, Sanofi, Lilly, Celgene and MSD, Grant/research support from: AbbVie, MSD, Jansen and Roche, Ricardo Blanco Speakers bureau: AbbVie, Pfizer, Roche, Bristol-Myers, Janssen, Sanofi, Lilly and MSD, Grant/research support from: AbbVie, MSD, and Roche

Orbital involvement in systemic sarcoidosis is a rare condition. We report a case of orbital sarcoidosis with bilaterally huge lacrimal gland involvement as the initial manifestation of systemic sarcoidosis. A 20-year-old woman admitted the ophthalmology department with progressive bilateral upper eyelid swelling for 6 months. The only pathologic finding was the presence of bilateral, symmetrical, solid, lobular masses at the lateral upper eyelids at the location of lacrimal glands. On systemic examination, bilateral parotid and submandibular glands appeared swollen. Magnetic resonance imaging of the orbit revealed bilateral symmetrical diffuse enlargement of the lacrimal glands with maximum and minimum thickness of 11 mm and 7 mm, respectively. The biopsy findings were compatible with sarcoidosis. Although lacrimal gland involvement has been reported in different studies, we for the first time report an unusual case with bilateral diffuse huge lacrimal gland involvement. Normal lacrimal gland thickness is approximately 4-5 mm in magnetic resonance imaging, while our case had bilateral diffuse enlargement of lacrimal glands, which showed maximum and minimum thickness of 11 mm and 7 mm, respectively. Although orbital involvement is uncommon in sarcoidosis, it should be remembered in the differential diagnosis of orbital masses.

A 46-year-old woman presented with massive hematemesis, caused by the rupture of esophageal varices. The laboratory investigations showed pancytopenia, and imaging tests revealed hepatosplenomegaly and ascites. A diagnosis of systemic sarcoidosis was made based on biopsies of the liver, stomach, lungs, heart, and skin. Although fat deposition was predominant, non-caseating granuloma and cirrhotic changes were found in the liver. Non-caseating granuloma was also identified in a biopsy specimen from minute depressions of the gastric folds. This case illustrates the rare involvement of the digestive system in a case of systemic sarcoidosis.

Background:Sarcoidosis is an inflammatory disease which can affect multiple organs. The most frequent affected organs are lungs, skin and eyes (1-5). Ocular involvement is a severe complication.Objectives:To assess the association of ocular sarcoidosis with other clinical domains.Methods:Study of a large cohort of systemic sarcoidosis from a single tertiary university hospital. All consecutive patients were diagnosed with systemic sarcoidosis from January 1, 1999 to January 1, 2019 according the ATS/ERS/WASOG criteria (6).Results:41 (22 women/19 men) of 383 (10.7%) patients had ocular involvement, mean age 44.8±16 years. Lung was the most common affected organ associated with ocular sarcoidosis (n=36; 87.8%) followed by skin (n=14; 34.1%), joints (n=12; 29.3%) and neurological affection (n=8; 19.5%).Ocular sarcoidosis presents a higher percentage of renal and neurological affection compared to organs affected in general sarcoidosis of our larger cohort (12% vs 6% and 19.5 vs 7%; respectively) (FIGURE).Conclusion:The proportion of clinical domains affected in ocular sarcoidosis is mostly similar to global sarcoidosis, except the neurological (which almost is threefold) and renal (which doubles) affection. Hence, the importance of being aware of neurological and renal complications when ocular affection is present.References:[1]Riancho-Zarrabeitia L, et al. Semin Arthritis Rheum. 2015; 45:361-8.[2]Calvo-Río V, et al. Clin Exp Rheumatol. 2014; 32:864-8.[3]Riancho-Zarrabeitia L, et al. Clin Exp Rheumatol. 2014; 32:275-84.[4]Vegas-Revenga N, et al. Am J Ophthalmol. 2019; 200:85-94.[5]Calvo-Río V, et al. Clin Exp Rheumatol. 2014;32 (4 Suppl 84): S54-7.[6]Costabel U, Hunninghake GW. Eur Respir J 1999; 14: 735-737FIGURE.Comparison between distribution of organs affected in ocular sarcoidosis (left) and distribution of organs affected in general sarcoidosis (right)Disclosure of Interests:Carmen Alvarez Reguera: None declared, Jorge Javier Gaitán-Valdizán: None declared, Raúl Fernández-Ramón: None declared, Rosalía Demetrio-Pablo: None declared, Ricardo Blanco Grant/research support from: Abbvie, MSD and Roche, Consultant of: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen and MSD, Speakers bureau: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen, Lilly and MSD

There are two important differentials for non-caseating granulomatous inflammatory tissue—leprosy and sarcoidosis—which presents a diagnostic challenge due to their histological similarities and specific geographical distribution. This article describes a rare presentation of systemic sarcoidosis called Heerfordt's syndrome with the triad of parotitis, uveitis, fever, and optional paralysis of facial nerve. This case was initially diagnosed as leprosy.

Background: Sarcoidosis is only revealed in 3% of the cases among Caucasians by ophthalmic damage and, when it does, it presupposes that the visceral impairment has remained silent so far. In this article, the exceptional case of a patient with systemic sarcoidosis revealed by unilateral exophthalmia is reported. Case presentation: The patient is a female with no history of substantial pathology. She had a unilateral right exophthalmia and ptosis evolving over 3 years. A dyspnea and dry cough were also reported with a duration of 1 year. The chest X-ray and CT scan revealed bilateral hilar opacities and mediastinal lymphadenopathy that lead to the suspicion of sarcoidosis. The cerebro-orbital CT scan led to the classification of the patient’s exophthalmia as Grade I and eliminated the possibility of other aetiologies. The mediastinoscopy indicated a granulomatous adenitis with no caseous necrosis, which allowed the diagnosis of a mediastinopulmonary sarcoidosis. Discussion and conclusion: The diagnostic approach to exophthalmia should involve a systematic search for sarcoidosis, although this aetiology remains exceptional.

Uveoparotid fever, also known as Heerfordt-Waldenstrom syndrome, is an uncommon acute presentation of systemic sarcoidosis. Patients may have features of complete/classic or incomplete disease. Early diagnosis and multidisciplinary care should be initiated to prevent sequelae. Herein, the authors report a rare case of retrospectively diagnosed incomplete uveoparotid fever in a patient with anterior uveitis, parotid gland enlargement, and fever who presented to our dermatology clinic with cutaneous sarcoidosis.

Introduction: There has been an increasing number of tattoo-related uveitis without evidence of systemic sarcoidosis. This finding may indicate a possible association of tattoos with uveitis. Case description: This report is a 10-year follow-up of a single case of tattoo-related uveitis with no proven systemic sarcoidosis. Conclusion: Literature review suggests possible pathogenesis and the patient’s management is discussed. This case highlights the importance of a good systemic history and physical examination, especially with regard to tattoos.

To analyze the condition of the lacrimal gland in patients confirmed to have sarcoidosis.The study included 46 patients (92 orbits) with verified diagnosis of systemic sarcoidosis that were examined during the period of 2009 to 2014. The age of patients ranged from 23 to 65 years and the average was 45.1 years. Among the examined patients, 66% were female and 34% male. In all patients, the diagnosis was verified according to modern criteria.Acoustic structure of 46 lacrimal glands of patients with systemic sarcoidosis (92 orbit), and lacrimal glands of 30 healthy individuals (60 orbits) were examined. In-depth in vivo examination of morphological changes was done using 'advanced' analysis of three-dimensional images.The analysis of a large number of studies devoted to changes in the lacrimal glands caused by sarcoidosis revealed a difficult problem: are there any differences between isolated involvement of the lacrimal gland tissue and the defeat of various organs and tissues? For now, the question remains unanswered.Цель - анализ состояния структуры слезной железы у пациентов с верифицированным диагнозом саркоидоза. Материал и методы. Обследовано 46 пациентов (92 орбиты) с верифицированным диагнозом 'системный саркоидоз' в период с 2009 по 2014 г. Возраст пациентов варьировал от 23 до 65 лет и в среднем составил 45,1 года. Среди обследованных больных преобладали женщины - 66%, мужчины составляли 34%. У всех пациентов диагноз системного саркоидоза был верифицирован с учетом современных критериев постановки диагноза. Результаты. Изучена акустическая структура слезных желез 46 пациентов (92 орбиты) с системным саркоидозом, а также слезных желез 30 условно здоровых людей (60 орбит). Для углубленного исследования прижизненных морфологических изменений был использован 'продвинутый' анализ объемного изображения. Заключение. В результате анализа данных большого ряда исследований, посвященных изучению изменения слезных желез при саркоидозе, был поставлен очень сложный вопрос: существуют ли различия между изолированным изменением слезной железы и поражением различных органов и тканей? Окончательно ответа на него до настоящего времени не получено.