Bilateral Hilar Lymphadenopathy Research Articles

Löfgren syndrome, characteristics of Japanese cases: a case and a review of the literature.

Löfgren syndrome (LS) is a sarcoidosis subtype characterized by an acute disease course, bilateral hilar lymphadenopathy, erythema nodosum, and ankle arthritis. LS in Caucasians appears to be self-limiting; however, our patients required glucocorticoid (GC) treatment. Here, we present a case of LS and review the literature to identify the characteristics of the Japanese patients with LS. Sixty-six-year-old woman was referred to our hospital; she initially presented with an acute onset of low-grade fever and ankle arthritis, followed by erythema nodosum. Skin biopsy revealed a non-caseating granuloma, and chest computed tomography scan displayed bilateral hilar lymphadenopathy; she was diagnosed with LS. Her arthralgia ameliorated spontaneously, but erythema persisted, necessitating GC treatment. Literature review revealed that the Japanese LS patients showed more fever, were more frequently treated with GC and more patients seemed to relapse, which may be explained by the absence of human leukocyte antigen-DR isotype 3, a good prognostic allele in Caucasians. Japanese LS may cause severe symptoms after development because of the differences in human leukocyte antigen from foreign countries. For early diagnosis, it is important to evaluate erythema nodosum and bilateral hilar lymphadenopathy in patients with polyarthritis involving ankle arthralgia.

The Use of Ibrutinib in Extranodal Marginal Zone Lymphoma of the Ocular Adnexa with Multifocal Disease that is Refractory to Rituximab

Tumors of the ocular adnexa been well documented. The most common ocular adnexal lymphoma is extranodalmarginal zone B-cell lymphoma (EMZL), with about a quarter of those occurring in the conjunctiva. We present acase with a patient suspected ocular EMZL, confirmed with biopsy. Positron emission tomography (PET) scan wasperformed to evaluate for distal lesions and the patient was found to have multiple hypermetabolic lesionsthroughout the body. Treatment with rituximab was initiated, and after four weekly treatments, subsequent PETscan showed that the original hypermetabolic lesions remained and new lesions appeared. Rituximab was stoppedand ibrutinib started. After eight months of ibrutinib treatment, PET scan showed that the original hypermetaboliclesions had disappeared. 19 months from initiation of treatment, a new hilar lesion was found. This progressedinto bilateral hilar and mediastinal lymphadenopathy, which was seen on PET scan performed 29 months intotreatment. These findings remained stable 35 months post-initiation of treatment. The use of ibrutinib in thispatient that initially presented with a lesion in the conjunctiva resulted in an initial resolution of the originalsystemic hypermetabolic regions on PET scan. While more studies are needed on ibrutinib in the treatment ofocular EMZL, physicians should be aware of the presentation of this disease and the range of treatment modalities.

Risk factors for uveitis in a Finnish sarcoid population.

To evaluate possible risk factors for uveitis among Finnish sarcoidosis patients. Patient charts of patients with sarcoidosis, with (n = 97) or without (n = 255) uveitis, and with a comprehensive eye examination from January 2014 to January 2021 at Tays Eye Centre, Tampere University Hospital, Finland were studied. Sarcoidosis patients with uveitis had higher rate of lymphocytopenia (43% vs. 29%, p = 0.041) and lower serum lysozyme levels (2.0 mg/L vs. 2.3 mg/L, p = 0.049; 95% CI, -0.692 to -0.002). Lysozyme level or lymphocytopenia did not have a statistically significant effect on the probability of uveitis in a binary logistic regression analysis. No other differences in the potential risk factors with p-values ≤0.05 were found, including bilateral hilar lymphadenopathy, serum angiotensin-converting enzyme (ACE) levels, sex, age and history of smoking. Lymphocytopenia and lower serum lysozyme levels present as possible risk factors for uveitis among patients with sarcoidosis. Systematic measurement of lymphocyte and lysozyme levels in sarcoidosis is needed to further understand their role as potential risk factors.

Ocular Signs and Testing Most Compatible with Sarcoidosis-Associated Uveitis: A Latent Class Analysis

PurposeThis study aims to explore the potential subgroups of sarcoidosis-associated uveitis (SAU) within a multicenter cohort of uveitis participants. DesignCross-sectional study. ParticipantsA cohort of 826 uveitis patients from a uveitis registry from 19 clinical centers in 12 countries between January 2011 and April 2015. MethodsWe employed a latent class analysis (LCA) incorporating recommended tests and clinical signs from the revised International Workshop on Ocular Sarcoidosis (IWOS) to identify potential SAU subgroups within the multicenter uveitis cohort. Additionally, we assessed the performance of the individual tests and clinical signs in classifying the potential subclasses. Main Outcome MeasuresLatent subtypes of SAU. ResultsAmong 826 participants included in this analysis, the two-class LCA model provided a best fit with the lowest BIC of 7218.7 and an entropy of 0.715. One class, consisting of 548 participants, represented the non-SAU while the second class, comprised of 278 participants, was most representative of SAU. Snowballs/string of pearls vitreous opacities had the best test performance for classification, followed by bilaterality and bilateral hilar lymphadenopathy (BHL). The combination of 4 tests with the highest classification importance, including snowballs/string of pearls vitreous opacities, periphlebitis and/or macroaneurysm, bilaterality, and BHL, demonstrated a sensitivity of 84.8% and a specificity of 95.4% in classifying the SAU subtypes. In the exploratory analysis of the 3-class LCA model, which had comparable fit indices as the 2-class model, we identified a candidate non-SAU subtype, candidate SAU subtype with pulmonary involvement, and a candidate SAU with less pulmonary involvement. ConclusionsLatent class modelling, incorporating tests and clinical signs from the revised IWOS criteria, effectively identified a subset of participants with clinical features indicative of SAU. Though the sensitivity of individual ocular signs or tests was not perfect, using a combination of tests provided a satisfactory performance in classifying the SAU subclasses identified by the 2-class LCA model. Notably, the classes identified by the 3-class LCA model, including a non-SAU subtype, an SAU subtype with pulmonary involvement, and an SAU subtype with less pulmonary involvement, may have potential implication for clinical practice and hence should be validated in further research.

Sarcoidosis‐related uveitis: A case series study

Purpose: The aim of this study was to characterize the epidemiological and clinical characteristics of ocular involvement in patients with systemic sarcoidosis.Methods: Retrospective case series study carried out at the Ophthalmology department of Habib Thameur University Hospital of Tunis between the 1st January 2022 and the 1st January 2023. Fifteen patients (30 eyes) were included. Based on the International Workshop on Ocular Sarcoidosis criteria, three patients had biopsy‐proven sarcoidosis; eight had presumed sarcoidosis and four had probable sarcoidosis. All patients had a complete ocular examination and systemic work‐up.Results: The median age at presentation was 45 years (range: 29–66). The sex ratio was 1:13. Ocular involvement was the presenting symptom in 11 patients. Bilateral granulomatous uveitis was the presenting form in all patients. Panuveitis was the most common anatomic pattern (9), followed by anterior (3), anterior and intermediate (2) and intermediate (1) uveitis. Posterior segment involvement included multifocal choroiditis (3) retinal vasculitis (5) macular oedema (4) and optic nerve head granuloma (1). Systemic manifestations included bilateral hilar lymphadenopathy (8) eyelid granuloma (2) neurosarcoidosis (3) Peripheral facial nerve palsy (2).Conclusions: Uveitis was the initial presentation of sarcoidosis in the majority of our subjects. Panuveitis was the most commonly encountered anatomic form. Our findings support the prominent role of the ocular examination in the early detection of sarcoidosis.

Sarcoidosis Presented with Bilateral Trigeminal Neuropathy – A Case Report

Sarcoidosis is a multisystemic granulomatous disease of unknown origin, characterized by the presence of noncaseating granuloma in affected organs, and most frequently presented with bilateral hilar lymphadenopathy, pulmonary infiltration, skin and eye lesion. However, neurological involvement including peripheral neuropathy is relatively rare. Although the diagnosis of neurosarcoidosis can be challenging, electrophysiologic and laboratory evaluations suggestive of sarcoidosis lead to diagnosis of sarcoidosis. We report a patient who presented bilateral trigeminal neuropathies initially without other systemic symptoms or organ involvements.

Sarcoidosis Presented with Bilateral Trigeminal Neuropathy – A Case Report

Sarcoidosis is a multisystemic granulomatous disease of unknown origin, characterized by the presence of noncaseating granuloma in affected organs, and most frequently presented with bilateral hilar lymphadenopathy, pulmonary infiltration, skin and eye lesion. However, neurological involvement including peripheral neuropathy is relatively rare. Although the diagnosis of neurosarcoidosis can be challenging, electrophysiologic and laboratory evaluations suggestive of sarcoidosis lead to diagnosis of sarcoidosis. We report a patient who presented bilateral trigeminal neuropathies initially without other systemic symptoms or organ involvements.

Sarcoidosis: A Case Report of an Often-Misdiagnosed Clinical Entity from Bangladesh

A chronic granulomatous disease of unknown etiology without any definite diagnostic criteria with an incidence of 1-1,00,000 among Asians affects multiple systems of our body and is often misdiagnosed owing to its elusive nature and resemblance with other granulomatous disease , especially tuberculosis (TB) in our subcontinent .A 38 years old diabetic woman came with the complaints of low grade fever, night sweats, cough, weight loss, migratory joint pain involving multiple small joints of hands without any morning stiffness and painful skin lesions which resolved spontaneously. Investigation showed Hb 11.5 g/dl with normocytic normochromic anaemia, increased sedimentation, increased CRP, insignificant MT, RA and ANA serology, Sputum for AFB didn't reveal any Mycobacteria. CXR revealed Bilateral Hilar Lymphadenopathy (BHL) and HRCT lungs showed bilateral hilar, mediastinal lymphadenopathy and pulmonary inflammatory lesions. Angiotensin Converting Enzyme (ACE)was 163 U/L which was higher than the normal. She was diagnosed as a case of sarcoidosis and was treated in line with oral prednisolone 40mg/day which was gradually tapered over a period of 5 months. Methotrexate 15mg/day once a week was supplemented with folic acid every consecutive day.Vaccination against H.influenzae and S.pneumoniae were advised and on subsequent follow ups she has been improving .The mortality rate is around 5% however, early suspicion, diagnosis and treatment can be life saving for the patient.

Outcomes of Sarcoidosis on Patients with Sickle Cell Disease - a Review of the National Inpatient Database 2016-2020

Introduction: Sickle Cell Disease (SCD) is an inherited hemolytic disorder due to an autosomal recessive mutation affecting mainly African Americans. SCD can lead to various complications, including Sickle Cell Crisis (SCC), Acute Chest Syndrome (ACS), strokes, and different osseous complications. Notably, these complications can be provoked using steroids. Sarcoidosis is a multisystem inflammatory disorder that is characterized by the presence of bilateral hilar lymphadenopathy that is commonly treated with steroids. Unfortunately, it is also preferentially affecting African Americans. The complication rates in patients with both diseases have rarely been described. We aim to describe the effect of sarcoidosis on the mortality and complication rates of admitted patients with SCC. Methods: Using the National Inpatient Sample (NIS) 2016-2020, we analyzed adult SCC and ACS hospitalizations with and without sarcoidosis using International Classification of Diseases - 10 Clinical Modification (ICD-10-CM) codes. The primary outcome was inpatient mortality. Secondary outcomes were inpatient morbidities, mean length of stay (LOS), and mean total hospital charge (THC). A multivariate logistic regression and linear regression analyses were used to adjust for potential confounders. Results: Out of 359655 patients hospitalized with SCC, only 4.6% had concomitant sarcoidosis. Of these, 70% were females, with a mean age of 38.4 compared to an average age of 32 in patients without sarcoidosis (p-value <0.05). The mean LOS for patients with sarcoidosis and SCC was 6.4 days, while it was 5.1 days for patients without sarcoidosis (p-value 0.001). While adjusting for common comorbidities and patients' characteristics, patients with SCC and sarcoidosis did not have a statistical mortality difference compared to patients without sarcoidosis [adjusted odds ratio (aOR): 2.3, CI: 0.4-14). On stratified analysis, patients with ACS and sarcoidosis had increased odds of mortality with an aOR of 6.68, CI: 1.1-39.7, and a p-value of 0.037. Moreover, amongst patients who had SCC, patients with concomitant sarcoidosis had a higher risk of acute coronary syndrome (aOR: 5.1, CI 1.24-21.0) and avascular necrosis (aOR: 1.57, CI: 1.12-2.20). There were no statistically significant differences in the odds of ischemic strokes, transient ischemic attacks, acute and chronic kidney disease, risk of intubation, pulmonary edema, osteoporosis, venous thrombosis, and THC. Figure 1 shows the Forrest plot for multivariate analysis of in-hospital morbidities when adjusted for patient demographics, comorbidities, and hospital characteristics. Conclusion: Patients admitted with ACS and sarcoidosis had higher odds of mortality. Patients with SCC and sarcoidosis also had higher odds of acute coronary syndrome, avascular necrosis, and mean LOS. Nevertheless, this has not impacted other inpatient comorbidity.

A Rare Case of Pulmonary Veno-Occlusive Disease, Pulmonary Capillary Hemangiomatosis

A 40-year-old male with past medical history of chronic pain on methadone for a few years, hypertension, and obesity presented with worsening dyspnea and was found to have significant hypoxemia. A CT-angiogram of the chest showed extensive diffuse bilateral pulmonary infiltrates with bilateral hilar and mediastinal lymphadenopathy.

SAT531 Thyroid Metastasis From Non-small Cell Lung Cancer: An Unusual Case Presentation

Abstract Disclosure: E.Y. Ibrahim: None. I. Guillen Alvarez: None. I. Hulinsky: None. D. Regelmann: None. C. Janowiecki: None. Background: Cases of thyroid tumors metastasizing from the lungs are rare, and their diagnosis is often challenging and can be mistaken for primary thyroid tumors or subacute thyroiditis. Thyroid function tests are typically normal and not helpful in establishing the diagnosis. Ultrasonography commonly shows focal or diffuse hypoechoic lesions. Correspondingly, heterogeneous hypodense areas with mild contrast enhancement are seen on CT scans. Clinical Case: Our patient is a 61-year-old male with a history of diabetes mellitus and 40 years tobacco use, who presented with a 1-week history of right-sided weakness and 3-weeks of dry cough. The weakness of his leg progressed to the point he could not ambulate. Initially, he was evaluated for a stroke and underwent CT imaging showing extensive lesions of the bilateral frontal, temporal, and parietal lobes. These findings were suspicious, and confirmed by MRI, to reflect multifocal metastatic lesions with vasogenic edema. Initial physical examination revealed a palpable thyroid nodule in the right lobe with bilateral cervical lymphadenopathy. CT of the neck demonstrated lymphadenopathy as well as multiple bilateral hypoechoic thyroid nodules, the largest of which measured 0.8 cm in the right thyroid lobe. TSH was 0.34 (0.48-4.17mU/L) with Free T4 at 1.39 (0.8-1.90ng/dL). The patient had denied symptoms of heat intolerance, tremor, or palpitations. A CT chest with contrast found a 3.5 cm cavitary lesion in the right upper lobe concerning for a primary lung neoplasm, as well as multiple bilateral lung nodules, mediastinal and hilar lymphadenopathy, and bilateral adrenal masses. Subsequent ultrasound-guided biopsy of the thyroid tumor revealed histologic features of a poorly differentiated non-small cell carcinoma, with features of a primary lung origin. These features were also present on a concurrent fine needle biopsy of an enlarged cervical lymph node. Immunohistochemistry staining showed the tumor was highly expressive for PDL1, focally positive for p40, pancytokeratin AE1/AE3, and negative for TTF-1 and thyroglobulinDue to the vasogenic cerebral edema, he was given dexamethasone with good response, and his weakness improved to the point that patient was able to ambulate. Treatment also included radiation therapy with subsequent pembrolizumab upon completion of radiotherapy. Conclusion: Several thyroid lesions representing metastases may go unnoticed because the physician’s attention toward lesions at other, more common sites, and detailed thyroid examination can be commonly overlooked. Patients with newly diagnosed malignancies should also be screened for thyroid involvement, including detailed physical, radiographic, and histologic examination of pathologic findings. Presentation Date: Saturday, June 17, 2023

P15 JAKi of all trades

P15 JAKi of all trades

OA12 Grimey granulomas of sarcoidosis

Abstract Introduction Sarcoidosis affects approximately 1% of the population. There are two situations where it came become life threatening: seizures secondary to neuro-sarcoidosis and arrhythmias due to cardiac infiltration. Currently there are limited licenced therapies for the treatment of sarcoidosis. We describe a case of cutaneous and neuro-sarcoidosis who developed subsequent multi-system sarcoidosis. Case description A previously fit and well 50-year-old presented to his local hospital with tonic clonic seizures. He worked as a mortgage broker and never smoked. There was no pro-drome and on examination it was noted he had erythema nodosum on his shins. He was investigated with a CT head and subsequent MRI demonstrating enhancement of his leptomeninges, mid brain parenchyma, hypothalamus and pituitary gland. A lumbar puncture was performed with an elevated protein level of 934g with a normal glucose and lymphocyte level. The CSF was negative for TB. A CT of his chest was performed demonstrating bilateral hilar lymphadenopathy and subsequent histology of these lymph nodes demonstrated non-necrotising, non-caseating epithelioid granulomas. A diagnosis of sarcoidosis was made and he was commenced on mycophenolate. His disease remained quiescent for 5 years before the eruption of further skin lesions. He developed multiple subdermal nodules on his fingers, wrists, elbows which were biopsied demonstrating cutaneous sarcoidosis. Neurological examination demonstrated brisk lower limb reflexes with upgoing plantars. He was commenced on a tapering course of prednisolone and an evaluation for extra-cutaneous disease was made. An IL2Ra was 4719ng/L (423-1843) with a normal ACE. Pituitary axis testing revealed a slightly raised prolactin (326 mIU/L). A CT-PET was performed demonstrating extensive metabolic activity in his skin, central nervous system, liver, spleen, salivary glands, skeletal uptake in both humeral heads alongside myocardial uptake. An urgent cardiac MRI was performed demonstrating active myocardial disease. An ECG did not demonstrate any conduction abnormalities. A diagnosis of multi-system sarcoidosis was made warranting an urgent MDT discussion given the progression of his disease. Discussion In this particular case, it had been assumed that his sarcoidosis was inactive until the emergence of the skin lesions. It was the skin lesions that lead to an assessment for multi-system sarcoidosis. It is common for the serum ACE level to be normal, as seen in up to 50% of cases and it was the Il2Ra elevation that prompted the CT-PET request. Given the extent and progression of disease despite treatment with mycophenolate, this gentlemen was given a tapering regime of prednisolone (starting at 20mg) and his mycophenolate was switched to methotrexate which has shown to have superior efficacy to mycophenolate for sarcoidosis. Furthermore, infliximab at a dose of 5mg/kg was commenced shortly after to control the increasing CNS lesions. Infliximab is a well recognised treatment for sarcoidosis; however, due to limited clinical trial data, it remains unlicenced for use in sarcoidosis with the exception of neuro-sarcoidosis (following the failure of DMARD therapy). To date, the patient remains well with no further worsening in his disease. Key learning points Sarcoidosis is a clinical entity with no one specific test. The diagnosis should be constructed using multiple parameters including radiographic findings, biochemical and histological proof. We advocate that suspected cases of sarcoidosis should have a thorough assessment and discussion in the MDT. When isolated skin disease is suspected or confirmed, baseline testing for extra-cutaneous involvement should be sent including NT-proBNP, troponin I and T, creatinine kinase, a full pituitary profile, ACE and cytokine assessment for Il2Ra, amongst other routine blood tests. An ECG is of paramount importance as arrhythmias are common cardiac features of sarcoidosis. Should there be ongoing suspicion of multiple organ involvement, a CT-PET should be performed at baseline. Isolated lymph node disease does not commonly warrant treatment; however, this cohort of patients should be closely monitored. The management of sarcoidosis in the acute setting is commonly prednisolone, but steroid sparing therapies should be considered in patients needing recurrent, prolonged courses of corticosteroids or those with evidence of critical organ involvement such as CNS, cardiac or lung disease. Life threatening disease or disease refractory to DMARD therapy should be considered for anti-TNF therapy with infliximab or adalimumab. More recently, tofacitinib has been promising as a treatment for sarcoidosis with clinical trials in Yale and Portland. Newer therapies in development include the GM-CSF inhibitor namilumab, and efzofitimod, a neuropilin 2 inhibitor.

Central Bronchial Deformity in Pulmonary Sarcoidosis: A Finding Suggestive of an Upper Lobe Fibrotic Phenotype on Chest Images

Bronchial and lung parenchymal structural remodeling may occur due to disease progression in patients with pulmonary sarcoidosis; however, its mechanisms remain unclear. Central bronchial deformity (CBD) associated with shrinkage in the upper lobe (SUL) is often observed in such patients. This study aimed to examine the association between CBD and structural remodeling to identify features indicating disease severity on chest images. This retrospective cohort study included 72 patients with pulmonary sarcoidosis, excluding patients with only bilateral hilar lymphadenopathy. The participants were divided into with and without CBD groups to examine the association between CBD and other structural remodeling, including SUL, cyst and/or low attenuation area-like emphysema (Cyst/LAA), pleural/sub-pleural thickening (PT), and traction bronchiectasis (TrBE), in the upper lobe on chest images. The association of CBD phenotype with respiratory dysfunction was also examined. CBD was highly associated with SUL (81.4% vs. 8.9%), Cyst/LAA (44.4% vs. 6.7%), and PT (59.2% vs. 3.7%). The respective odds ratios in the univariable and multivariable analyses were as follows: SUL, 45.1 and 39.9; Cyst/LAA, 11.2 and 14.2; and PT, 64.0 and 68.7. TrBE was frequently associated with CBD (22.25% vs. 4.4%); the odds ratio was 6.14 in the univariable analysis. Furthermore, participants with CBD exhibited lower %FVC and %DLCO. CBD is significantly associated with lung remodeling (SUL, Cyst/LAA, TrBE, and PT) and respiratory dysfunction. CBD may be a crucial clinical phenotype to identify upper lobe fibrotic changes.

E033 The evolution of neurosarcoidosis on infliximab: a case report

Abstract Background/Aims We report a case of neurosarcoidosis (NS) 16 years after initial diagnosis of systemic sarcoidosis in a patient treated with methotrexate, infliximab and corticosteroids. Methods In 2015 a 47-year-old male presented with florid synovitis in MCPs, PIPs, in a pattern reminiscent of rheumatoid arthritis (rheumatoid factor, anti-CCP antibodies were negative; CRP 46g/l). Previously in 2006 a diagnosis of sarcoidosis had been made due to systemic symptoms, arthralgia, bilateral hilar lymphadenopathy (BHL) and confirmed on biopsy of bone and mediastinal nodes, for which he was currently prescribed prednisolone 10mg. So in 2015, considering this to be an inflammatory arthritis (IA), in the presence of sarcoid, methotrexate 15mg subcutaneously was added with good response initially. Though his BHL had regressed and he has was systemically well his IA subsequently flared, and after liaising with his respiratory consultant, adalimumab was added in 2017. Subsequently, as he was well, prednisolone was weaned to 3mg daily. In May 2020 his IA flared again, this time with DIP involvement and dactylitis (his father has psoriasis). Adalimumab was switched to baricitinib, and prednisolone increased to 15mg daily. Baricitinib was inefficacious and linked to recurrent sinusitis so this was switched to infliximab in January 2022 and methotrexate increased to 25mg weekly with resolution of his IA. The patient presented in September 2022 with confusion, slurred speech and unsteady gait with a background of recurrent headaches for six months since starting infliximab. Results Routine blood tests were normal: full blood count, biochemistry, serum ACE, calcium and CRP 2mg/L. Chest X-ray was normal. Neuroimaging: MRI (non-contrast) and CT head were normal. CSF fluid: protein 1.75(0.15-0.4)g/l; glucose 1.8(2.2-3.9)mmol/L; Lymphocytes 98cmm; bacterial and viral culture negative. A diagnosis of neurosarcoidosis was made. Prednisolone was increased to 30mg daily with resolution of neurological symptoms and headache. Conclusion In the UK the incidence of NS is 1/100,000. NS is reported in 5-10% of patients with sarcoidosis and typically presents within 2 years of diagnosis. Presentation of NS includes: cranial neuropathies (50-75%), aseptic meningitis (10-20%), parenchymal disease (<50%), hydrocephalus (10%), sellar disease (2-8%), myopathy (10%), myelopathy (5-26%), neuropsychiatric illness (depression 60%, psychosis 20%) and peripheral neuropathy (2-86%). Typical CSF examinations in NS are: a mild to moderate pleocytosis (usually <100cells/μL), with lymphocyte predominance and elevated protein. Though Infliximab is reported as a treatment option for NS, we reflect that anti-TNF alpha therapies, though they reduce systemic inflammation, do not cross the blood brain barrier. We speculate that introduction of infliximab unmasked NS in this patient. This case also highlights the importance of probing and investigating neurological symptoms in patients with sarcoidosis. Disclosure H.S. Jayasekera: None. S.A. Tabassum: None. K.M.J. Douglas: None.

Heerfordtův syndrom – vzácná manifestace sarkoidózy z pohledu ORL lékaře

Summary Sarcoidosis is a systemic granulomatous disease that mainly affects the pulmonary and lymphatic systems. In its rare, acute forms, it can manifest as Lofgren’s syndrome (combination of erythema nodosum, bilateral pulmonary hilar lymphadenopathy, joint pain, fever and general symptoms) and even more rarely as Heerfordt’s syndrome (combination of fever, anterior uveitis, bilateral edema of the parotid area and uni or bilateral facial nerve paresis). Although this dia­gnosis is rare, it belongs to the differential dia­gnosis of sudden paresis of the facial nerve and swelling of the parotic area. In our case report, we describe Heerfordt’s syndrome in a 50-year-old woman. The pathophysiology, symptomatology, dia­gnosis, and treatment of this rare syndrome are discussed. Key words sarcoidosis – uveoparotid fever – facial nerve paresis

Atypical and Typical Distant Sarcoid-like Reactions during Treatment of Metastatic Melanoma.

HomeRadiology: Imaging CancerVol. 5, No. 2 PreviousNext Images in CancerFree AccessAtypical and Typical Distant Sarcoid-like Reactions during Treatment of Metastatic MelanomaLacey J. McIntosh , Evan C. Ruppell, Maria BarileLacey J. McIntosh , Evan C. Ruppell, Maria BarileAuthor AffiliationsFrom the Division of Oncologic and Molecular Imaging (L.J.M., E.C.R.) and Division of Cardiothoracic Imaging (M.B.), UMass Chan Medical School/Memorial Health Care, 55 Lake Avenue North, Worcester, MA 01655.Address correspondence to L.J.M. (email: [email protected]).Lacey J. McIntosh Evan C. RuppellMaria BarilePublished Online:Mar 24 2023https://doi.org/10.1148/rycan.220177MoreSectionsPDF ToolsImage ViewerAdd to favoritesCiteTrack CitationsPermissionsReprints ShareShare onFacebookTwitterLinked In Sarcoid-like reactions are reported in 4%–14% of patients with cancer and are likely driven by T-cell–mediated hypersensitivity reactions forming granulomas (1). They can occur with infection, treatment, or the presence of foreign bodies (2) and have been increasingly reported in the era of precision targeted and immune checkpoint inhibitor therapy (3,4), including the use of BRAF and MEK inhibitors (Figure) (5). The classic pattern mimics sarcoidosis and involves mediastinal and hilar lymph nodes, which may be enlarged and demonstrate increased fluorodeoxyglucose (FDG) uptake at FDG PET/CT; pulmonary nodules and infiltrates may or may not be present (1,2). The incidence of extrathoracic sarcoid-like reactions is unknown but rare (6), with case reports documenting reactions including the liver, spleen, skin, central nervous system, pancreas, bone, and eyes (6,7). Absence of findings at baseline and emergence after treatment initiation can be helpful to identify a sarcoid-like reaction, although it may be indistinguishable from disease and may require tissue sampling. Radiologists should recognize these patterns to avoid confusion with disease progression. If necessary, steroids are the mainstay of treatment; immunotherapy discontinuation may be required in severe cases.Sarcoid-like reactions in a 29-year-old woman with +BRAF V600 mutation metastatic melanoma. (A, B) Baseline fluorodeoxyglucose (FDG) PET/CT maximum intensity projection (MIP) and axial fusion images show FDG-avid left axillary mass (black and white arrows), found to be metastatic melanoma (no primary cutaneous lesion identified). (C, D) Follow-up FDG PET/CT MIP and axial fusion images 8 months after left axillary nodal dissection, while receiving adjuvant dabrafenib and trametinib, show that the left axillary mass has been excised; however, there is a new FDG-avid right axillary lymph node (dashed black and white arrows). Subsequent biopsy showed lymphoid tissue with noncaseating granulomatous inflammation, consistent with atypical presentation of a sarcoid-like reaction. (E, F) Follow-up FDG PET/CT MIP and axial fusion images 2 months later show FDG-avid mediastinal and bilateral hilar lymphadenopathy (short black and white arrows), in a classic pattern of sarcoid-like reaction, which was confirmed with endobronchial US-guided fine needle aspiration cytology.Download as PowerPointOpen in Image Viewer Disclosures of conflicts of interest: L.J.M. Consultant providing clinical trial reads (Bioclinica/Clario, WorldCare Clinical, Imaging Endpoints) and content consultation (Medality) but without conflicts of interest for this article. E.C.R. No relevant relationships. M.B. No relevant relationships.Keywords: Molecular Imaging–Cancer, PET/CTAuthors declared no funding for this work.

Coexistance of Probable Neurosarcoidosis and Generalized Myasthenia Gravis: Case Report

Objective NA. Background Sarcoidosis is an immune mediated disorder characterized by the formation of non-necrotizing granulomas in multiple organs. The broad range of manifestations of sarcoidosis in the nervous system includes cranial neuropathy, meningitis or myelopathy. Generalized Myasthenia Gravis is a neuromuscular disorder characterized by the formation of antibodies against the acetylcholine receptor, and typically presents with muscle weakness and fatigue. Association of Myasthenia Gravis and Neurosarcoidosis is infrequent. Design/Methods NA. Results Case report: A thirty-five year old woman, natural from Senegal was admitted to the hospital with a two month history of left peripheral facial nerve palsy, progressive dysphonia and dysphagia, associated with fever, headache and asthenia. Examination revealed, left facial numbness and weakness (V and VII nerve) and right vocal cord paresis (X nerve). Chest ray showed bilateral hilar lymphadenopathy and cranial MRI revealed enhancement of both Meckel cavum, bilateral trigeminal nerves and canalicular segment of the left facial nerve. CSF examination detected elevated protein and lymphocytic pleocytosis. For further study a PET/TC was done which revealed supradiaphragmatic and infradiaphragmatic hypermetabolic adenopathies. Biopsy of an adenopathy was performed objecting non-caseating granulomas consistent with the diagnosis of probable neurosarcoidosis. Moreover, the patient explained weakness and muscle fatigue since she was 18 years old, which was observed at proximal limb muscles. Acetylcholine receptor antibodies were detected and electromyographic study showed a decremental response to repeated nerve stimulation. Conclusions It is well known that autoimmune disorders may coexist in some patients. Neurosarcoidosis and Myasthenia Gravis are two rare diseases with different pathogenesis. Although their coexistance could be coincidental it may also suggest immunologic mechanisms triggering the occurrence of these diagnoses together.

IMMUNE RECONSTITUTION INFLAMMATORY SYNDROME CAUSED BY TWO SPECIES OF NONTUBERCULOUS MYCOBACTERIA PRESENTING AS MEDIASTINAL LYMPHADENOPATHY

IMMUNE RECONSTITUTION INFLAMMATORY SYNDROME CAUSED BY TWO SPECIES OF NONTUBERCULOUS MYCOBACTERIA PRESENTING AS MEDIASTINAL LYMPHADENOPATHY

UNCOMMON CAUSES OF RECURRENT UNILATERAL PLEURAL EFFUSIONS

UNCOMMON CAUSES OF RECURRENT UNILATERAL PLEURAL EFFUSIONS