Case Of Sarcoidosis Research Articles

Abstract 4119267: Diagnosing An Uncommon Presentation of Cardiac Sarcoidosis with Isolated Bi-Atrial Involvement: A Case Report

Background: Sarcoidosis, a systemic granulomatous inflammatory disorder can involve various organs, including the heart but isolated bi-atrial cardiac involvement is rare. Advanced cardiac imaging especially in atypical presentations, can aid in early diagnosis. Case: A 59 year-old man with history of biopsy-proven pulmonary sarcoidosis presented with non exertional chest pain for 2 months. EKG, cardiac enzymes, and Initial echocardiogram(TTE) was unremarkable. Stress echocardiogram ruled out myocardial ischemia. CT scan noted mediastinal lymphadenopathy consistent with known sarcidosis. In absence of other explainable etiolgies for chest pain, Cardiac MRI was done and showed preserved biventricular function, subepicardial enhancement in the basal inferior, inferolateral, and anterolateral walls. The enhancement raised suspicion for CS. However, symptoms resolved spontaneously, cardiac workup paused and he was monitored conservatively with serial Echocardiogram(TTE) until onset of dyspnea 3 years later. Repeat TTE and EKG then noted newly enlarged left atrium and atrial tachycardia. Further testing with Cardiac positron emission tomographic imaging with F-18 fluorodeoxyglucose (FDG-PET CT) showed abnormal myocardial uptake in both atrial posterior walls but no ventricular involvement, indicative of isolated bi-atrial inflammation in CS. He was treated with prednisone and methotrexate. Successful symptom and inflammation resolution on FDG-PET CT occurred in 3 months Discussion: CS is rare and seen clinically in about 5% and diagnosed postmortem in 25% of sarcoidosis cases. Symptoms vary widely, with potential for severe heart complications. Left ventricle and interventricular septum are commonly involved, but isolated bi-atrial involvement is rare. Early diagnosis aided by Cardiac MRI and FDG-PET CT is crucial. Prednisone is mainstay of treatment, often combined with methotrexate. Cases of concurrent atrial CS involvement and bi-atrial fibrosis with Left ventricular hyperenhancement are documented, but this is the first known report of isolated bi-atrial hyperenhancement on FDG-PET CT for CS. This case highlights the need for symptom correlation with clinical and imaging follow-up, especially in atypical presentations.

Atypical sarcoidosis: About a case

Chronic lung consolidation is often associated with various respiratory pathologies, ranging from chronic infections to interstitial diseases such as sarcoidosis, which is a benign systemic granulomatosis of unknown etiology. Interstitial parenchymal involvement is usual. The pseudo-alveolar form is atypical, often presenting acutely, difficult to diagnose, but tends to evolve rapidly favorably under corticosteroid therapy. We report here a case of pseudo-alveolar sarcoidosis in a 58-year-old patient whose radiological and clinical presentation is unique and perplexing. In this study, we emphasize the rarity of this pseudo-alveolar sarcoidosis.

Sarcoidosis: oral manifestations (case report)

Relevance. Sarcoidosis is a multisystem granulomatous disease of unknown origin, commonly presenting with bilateral hilar lymphadenopathy, pulmonary infiltrates, and involvement of the eyes, skin, gums, and oral mucosa. This clinical case illustrates the oral manifestations of this rare systemic disease. Gingival hypertrophy and soft tissue lesions on the lip mucosa were the initial signs of the condition. Following an extended diagnostic process, the patient underwent treatment by both a dentist and a rheumatologist, leading to a stable remission of the disease.Case description. This article presents a clinical case of sarcoidosis manifesting in the mouth as gingival hypertrophy and a soft tissue lesion on the upper lip. In most cases, a diagnosis of sarcoidosis is made before any oral symptoms appear. Diagnosing a systemic disease based on primary oral symptoms poses a unique challenge, as dental professionals may not always be familiar with the oral manifestations of systemic conditions. In cases of suspected sarcoidosis, the oral mucosa should be thoroughly examined, and systemic sarcoidosis should be considered in the differential diagnosis of granulomatous lesions in the mouth.Conclusion. Early initiation of treatment can significantly improve the patient's quality of life and prognosis.

Diagnosis of pulmonary sarcoidosis comorbid with non-specific interstitial pneumonia: a case report

BackgroundAlthough the imaging manifestations of pulmonary sarcoidosis have been described in detail in previous studies, a consensus has not been reached on the imaging presentation of non-specific interstitial pneumonia (NSIP) lookalike pattern as a distinct pattern in the diagnosis of pulmonary sarcoidosis in high-resolution computed tomography (HRCT). No cases of pulmonary sarcoidosis comorbid with NSIP have been reported.Case presentationA 53-year-old male presented to the hospital with a five-year history of recurrent coughing up sputum and a four-year history of shortness of breath. In addition to the typical features of pulmonary sarcoidosis, the patient’s HRCT also showed unexpected interstitial changes in the lower lobes of both lungs, suggesting an NSIP pattern. Histopathology of the lung tissue in this region confirmed well-formed noncaseating epithelioid granulomas and pathological modifications of NSIP. After a rigorous exclusion diagnosis combining the patient’s clinical features, radiological and pathological findings, we diagnosed this patient with pulmonary sarcoidosis comorbid with NSIP.ConclusionsThis suggests that NSIP may act as a rare comorbidity of pulmonary sarcoidosis thereby resulting in the patient’s HRCT presenting differently from routine sarcoidosis imaging.

Clinicopathologic features, demographics, disease burden, and therapeutics in alopecic sarcoidosis: a case series and systematic review.

Alopecic sarcoidosis is an uncommon cutaneous manifestation of sarcoidosis. Scarring and nonscarring alopecic sarcoidosis have been reported; however, information on the epidemiology, systemic disease associations, and treatment efficacy is limited. To address these gaps, we conducted a retrospective chart review and systematic literature review of alopecic sarcoidosis cases. Full-text English publications from PubMed, Scopus, and Google Scholar from inception to August 2023 were analyzed. Treatment evidence quality was assessed using the modified Oxford Centre for Evidence-Based Medicine rating scale. Three patients with biopsy-proven alopecic sarcoidosis were included as a case series, all demonstrating systemic sarcoidosis and 2 requiring multiple therapies. Among 1778 search results, 60 articles representing 77 cases of alopecic and scalp sarcoidosis were included. Patients were categorized into 4 distinct alopecic subgroups. Black patients constituted the majority of all subgroups. Extracutaneous sarcoidosis burden was high across all alopecic subgroups, with ocular disease appearing overrepresented. Topical and oral corticosteroids were the main treatments. Though scarring alopecia patients had poor outcomes despite receiving immunomodulators/cx, limited data suggest potential efficacy of tumor necrosis factor-alpha inhibitors. This study has a small sample size. Our findings underscore the importance of evidence-based strategies for improving alopecic sarcoidosis management. Prompt diagnosis and systemic evaluation, especially for scarring alopecia, are essential for timely intervention to optimize patient outcomes.

Ocular Sarcoidosis as Initial Presentation of the Disease: Clinical Characteristics and Risk Factors Associated with Disease Recurrence

ABSTRACT Purpose To review the clinical presentation and diagnostic workup of patients presenting with ocular sarcoidosis as initial manifestation of the disease and identify risk factors for disease recurrence. Methods All cases of sarcoidosis followed in our departments of Ophthalmology and Internal Medicine were retrospectively reviewed. Among 177 patients, 50 had ocular sarcoidosis as initial presentation of the disease. Clinical, biological, radiological, and treatment data were collected. Logistic regression analysis was conducted to identify risk factors for recurrence. Results Mean age of patients was 51 years, with an equal M/F ratio. Bilateral ocular involvement was present in 86% of cases, with granulomatous panuveitis being the most common manifestation (48%). Two-thirds of patients had extraocular symptoms related to the disease, mostly fatigue. The most frequently involved extraocular site was the supradiaphragmatic lymph nodes, identified in 84% of cases. CRP was normal in 68% of patients, while ACE and lysozyme were elevated in 19% and 64% of cases, respectively. Histopathological confirmation was obtained in 68% of patients. Female sex (odds ratio [OR] 6.66, 95% confidence interval [CI] 1.50–40.40, p = 0.021) and granulomatous panuveitis (OR 7.23, 95% CI 1.70–40.99, p = 0.013) were identified as significant risk factors for disease recurrence. Conclusion Ocular sarcoidosis is a common initial presentation of systemic sarcoidosis. Diagnosis should be suspected in the presence of bilateral granulomatous panuveitis. Chest CT scan or 18F-FDG PET/CT is mandatory for systemic workup and biopsy is necessary for definite diagnosis. Female sex and a panuveitis presentation are significantly associated with disease recurrence.

Discordant myocardial perfusion defects and coronary artery disease - a multimodality imaging journey in the natural history of cardiac sarcoidosis.

Cardiac sarcoidosis is a rare form of systemic sarcoidosis characterized by formation of non-caseating granulomas in the myocardium, leading to heterogeneous manifestations, including conduction disturbances, arrhythmias, and heart failure. Besides myocardial fibrosis and structural myocardial alterations, cardiac sarcoidosis can impact the epicardial and microvascular circulation, causing ischemia and regional microvascular dysfunction. We present a case of cardiac sarcoidosis with atypical initial presentation but with evidence of disease in myocardial perfusion imaging, which was overlooked since it did not correspond to bystander coronary artery disease. Multimodality imaging facilitated diagnosis and offered insights into the regional microcirculation impairment caused by sarcoidosis-induced inflammation.

Sarcoidosis in a young adult: A rare sequelae of COVID‐19 infection

Key Clinical MessageThis case illustrates sarcoidosis as a potential complication of COVID‐19, highlighting the need for a comprehensive diagnostic approach, including histopathology and prolonged monitoring, to distinguish it from post‐COVID fibrosis. Further research is crucial to elucidate these associations and understand their underlying mechanisms.AbstractSevere Acute Respiratory Syndrome Coronavirus‐ 2 (SARS‐CoV‐2), a positive‐sense single‐stranded RNA virus, causes COVID‐19 and has been linked to autoimmune disorders. Sarcoidosis is a multi‐system disease that is frequently triggered by infections. It is characterized by non‐necrotizing granulomas in multiple organs. We present a case of sarcoidosis as rare sequelae of COVID‐19. A 26‐year‐old man presented with mild COVID‐19 symptoms, followed by prolonged fever and cough despite initial therapy, prompting a provisional diagnosis of post‐COVID fibrosis. A subsequent assessment at a tertiary hospital revealed dyspnea, weight loss, and abnormal chest imaging, all of which were consistent with pulmonary sarcoidosis with pulmonary tuberculosis as a differential diagnosis. A biopsy taken during bronchoscopy confirmed pulmonary sarcoidosis and treatment with inhalation steroids resulted in symptom relief, which was followed by remission with oral steroid therapy. Sarcoidosis is a systemic disease of unknown etiology, characterized by non‐necrotizing granulomas in multiple organs. It may be triggered by infections and involves an abnormal immune response. COVID‐19 can potentially initiate sarcoidosis, with both sharing common immune mechanisms. Diagnosis involves imaging and biopsy, and treatment typically includes glucocorticoids and regular monitoring. This case report emphasizes the potential link between COVID‐19 and autoimmune conditions like sarcoidosis, highlighting the need for a comprehensive diagnostic approach and long‐term observation to distinguish between sarcoidosis and post‐COVID fibrosis.

National retrospective registry survey on the epidemiology of sarcoidosis in Finland 2002−2022

BackgroundThe prevalence of sarcoidosis is known to be high in the Nordic countries. There are no recent research data on the incidence or prevalence of sarcoidosis in Finland. Our aim...

A rare case of hepatic sarcoidosis, masquerading as lymphoma

A rare case of hepatic sarcoidosis, masquerading as lymphoma

Analysis of 14 cases of sarcoidosis of head and neck

Objective:To explore the clinical characteristics of sarcoidosis of head and neck symptoms, and to summarize the diagnosis and treatment experience. Methods:A retrospective study was conducted on patients with nodular disease with main symptoms in the head and neck who visited Henan Provincial People's Hospital from January 2020 to August 2023. The clinical data including symptom characteristics, pathological characteristics, treatment methods, and prognosis were analyzed. Results:A total of 14 patients were included, with 4 males（28.6%） and 10 females（71.4%）, age ranged from 11 to 71 years, with an average age of（52.0±15.8） years. The lesions were located in the parotid gland in 2 cases and the neck in 12 cases. Twelve cases underwent neck mass resection surgery, and 2 cases underwent ultrasound-guided core biopsy of parotid gland tumor and postoperative pathological diagnosis was confirmed in all cases. Four cases received steroid treatment postoperatively, and showed good prognosis with reduced lesion size after 3 months. Three cases did not take medication and the lesions continued to persist, causing discomfort. Seven cases did not take medication postoperatively, and the lesions expanded with multi-organ progression. Conclusion:Patients with head and neck sarcoidosis are rare in clinical practice, and it is prone to misdiagnosis and missed diagnosis. Steroid therapy can achieve good therapeutic effects.

An Unusual Case of Cutaneous Sarcoidosis Following Treatment with Ocrelizumab: Case Report and Literature Review.

An Unusual Case of Cutaneous Sarcoidosis Following Treatment with Ocrelizumab: Case Report and Literature Review.

Ocular Sarcoidosis Masquerading as Acute Retinal Necrosis: Two Case Reports.

To report two cases of ocular sarcoidosis (OS) initially presenting as unilateral acute retinitis, which mimicked acute retinal necrosis. Retrospective descriptive case reports. Two middle-aged healthy Asian women experienced progressive vision loss in their left eyes over a one-month period. Anterior uveitis, vitritis, and diffuse peripheral retinal infiltration were observed. Comprehensive diagnostic evaluations were conducted, including blood work-up, viral polymerase chain reaction of anterior chamber paracentesis samples, and chest X-ray, all yielding negative results. Despite prompt initiation of antiviral therapy, retinal infiltrations remained unchanged within the first 10 days. Chest CT imaging revealed multiple lymphadenopathies consistent with sarcoidosis. Case 1 was presumed OS, and case 2 was definite OS based on lung and lymph node biopsy results in accordance with the 2017 revised international workshop on OS (IWOS) criteria. In both cases, the retinal lesions gradually resolved after several weeks of systemic corticosteroids, and the best corrected vision of the affected eye improved to 20/25 at the 12 and 6-month follow-ups, respectively. Acute unilateral retinal infiltration in the peripheral region, exhibiting rapid progression resembling acute retinal necrosis, can be a rare manifestation of OS. Chest CT imaging can provide valuable assistance in the diagnostic process, especially when systemic examinations yield no significant findings.

A Rare Case of Concurrent Lupus Mastitis and Sarcoidosis in a 62-Year-Old Female.

Systemic lupus erythematosus (SLE) and sarcoidosis are two of the most well-recognized, chronically diagnosed conditions in the United States, with a plethora of known multisystem manifestations. With regard to breast pathology, lupus mastitis is a relatively uncommon manifestation of SLE, commonly involving both the mammary gland and subcutaneous soft tissues of the breast. Sarcoidosis in the breast is a similarly, exceedingly rare manifestation of this multi-system disorder, classically presenting with non-caseating granulomas. Both present with non-specific mammographic and sonographic features. We present a 62-year-old female with known diagnosis of discoid lupus and Graves' disease who presented initially with an abnormal screening mammogram, ultimately undergoing mammographic work-up and subsequent biopsy demonstrating lupus mastitis, including vasculitis, panniculitis, and fibrosis with chronic inflammation. The patient was also found to have small non-caseating granulomas, some in a perivascular distribution, classically seen in sarcoidosis. Given the rarity of both manifestations, our case explores the coexistence of these autoimmune processes and this atypical presentation.

Comparison between diffuse and partial involvement of thoracic lymph nodes on the outcome of sarcoidosis patients.

Sarcoidosis is a systemic disease of unknown etiology with diverse clinical manifestations. Disease may resolve spontaneously or require immunosuppression to control progression. Currently, there is no predictive model to direct treatment, and management is guided by symptoms and functional impairment. This study examines the association between biopsy features and prognosis. This is a retrospective population-based cohort study. New cases of biopsy-proven sarcoidosis were divided into two groups: those with diffuse thoracic lymph nodes (TLN) involvement, versus partial TLN involvement (Defined as Non-necrotizing granuloma (NNG) found in some but not all sampled TLN). We compared outcomes one year after diagnosis. We assessed the need for immunosuppression, the number of hospitalizations, and lung function deterioration. 77 cases were included in the final analysis. 48.1% demonstrated extensive TLN involvement, and 51.9% demonstrated partial or non-involvement of sampled TLN. The partial positive group had a more aggressive disease, reflected by a significantly higher need for steroid therapy in the first year after diagnosis (45.0% vs. 18.9% p=0.015). The number of hospitalizations and lung functions were not significantly different between groups. Our findings demonstrate a significantly increased need for steroidal therapy among sarcoidosis patients with a partial positivity of TLN. These findings suggest that the degree of TLN involvement can help predict worse outcome and guide therapeutic decisions.

A case of renal sarcoidosis complicated by parotid gland and uterine lesions

BackgroundSarcoidosis is a systemic disease that can affect multiple organs. While pulmonary sarcoidosis is most commonly observed, renal sarcoidosis occurs less frequently. We herein report a case of sarcoidosis with an exceptionally rare distribution including renal lesions.Case presentationA 51-year-old Japanese female was referred because of bilateral parotid swelling and renal dysfunction. Computed tomography scan showed the swelling of bilateral kidneys, parotid glands, and uterus. Ga scintigraphy also showed remarkable accumulation in these organs. Renal biopsy and cytological evaluations of parotid gland and uterus were performed and she was diagnosed as sarcoidosis of these organs. Treatment was initiated with prednisolone 40 mg/day and then renal dysfunction subsequently improved. In addition, the swelling of parotid glands and uterus improved and Ga accumulation in each organ had disappeared.ConclusionThis is a first case of renal sarcoidosis complicated by parotid glands and uterus lesions. Pathological findings and the reactivity observed in Ga scintigraphy indicated the presence of lesions in these organs.

A Case of Ocular Sarcoidosis with an Atypical Presentation of Subretinal Mass

Purpose: To describe a case of ocular sarcoidosis manifesting as an atypical subretinal mass.Case summary: A 59-year-old woman reported reduced visual acuity in her left eye lasting for 1 month. Her best corrected visual acuity in the left eye had diminished to 0.3. A fundus examination revealed a whitish elevated lesion, approximately one disc diameter in size, in the perimacular area of the left eye. Optical coherence tomography identified a hyporeflective oval-shaped mass in the choriocapillaris region accompanied by subretinal fluid. Initial assessment suggested choroidal osteoma with choroidal neovascularization. Consequently, the patient underwent two intravitreal bevacizumab injections, which did not lead to improvement. To rule out systemic diseases, including syphilis, tuberculosis, and sarcoidosis, further radiologic and laboratory tests were conducted. A chest computed tomography highlighted mediastinal lymph node enlargement and pervasive nodules in both lung parenchymata, and serum angiotensin-converting enzyme levels were elevated to 77 U/L. Based on these findings, a diagnosis of sarcoidosis was confirmed, and the patient commenced treatment with oral prednisolone. Eight weeks after treatment, the subretinal lesion and fluid had resolved, and the best corrected visual acuity in the patient’s left eye had improved to 0.9.Conclusions: Ocular sarcoidosis can manifest as a subretinal mass. This presentation should be differentiated from other subretinal lesions.

Understanding the Added Value of High-Resolution CT Beyond Chest X-Ray in Determining Extent of Physiologic Impairment

BackgroundSarcoidosis staging has primarily relied on the Scadding chest radiographic system, although chest CT is finding increased clinical use. Research QuestionWhether standardized CT assessment provides additional understanding of lung function beyond Scadding stage and demographics is unknown and the focus of this study. Study Design and MethodsWe used the NHLBI study Genomics Research in Alpha-1 Anti-Trypsin Deficiency and Sarcoidosis (GRADS) sarcoidosis cases(N=351) with Scadding stage and Chest CT scans obtained in a standardized manner. One chest radiologist scored all CT scans with a visual scoring system, with a subset read by another chest radiologist. We compared demographic features, Scadding stage and CT findings, and correlation between these measures. Associations between spirometry and DLCO and CT and Scadding stage were determined using regression analysis (N=318). Agreement between readers was evaluated using Cohen’s Kappa. ResultsCT features were inconsistent with Scadding stage in about ∼40% of cases. Most CT features assessed on visual scoring were negatively associated with lung function. Associations persisted for FEV1 and DLCO when adjusting for Scadding stage, although some CT feature associations with FVC became insignificant. Scadding stage was primarily associated with FEV1 and inclusion of CT features reduced significance in association between Scadding and lung function. Multivariable regression modeling to identify radiologic measures explaining lung function included Scadding stage for FEV1 and FEV1/FVC (P<0.05) and marginally for DLCO (P<0.15). Combinations of CT measures accounted for Scadding stage for FVC. Correlations among Scadding and CT features were noted. Agreement between readers was poor to moderate for presence/absence of CT features and poor for degree/location of abnormality. InterpretationCT features explained additional variability in lung function beyond Scadding stage, with some CT features obviating the associations between lung function and Scadding. Whether CT features/phenotypes/endotypes could be useful for managing patients with sarcoidosis needs more study.

Cirrhosis due to hepatic sarcoidosis: A rare presentation

Key Clinical MessageSarcoidosis, although predominantly affecting the lungs, can present with cirrhosis, posing diagnostic challenges. Elevated ACE levels and atypical liver enzyme patterns should prompt consideration of sarcoidosis in cryptogenic cirrhosis cases, necessitating comprehensive evaluation including liver biopsy and imaging for accurate diagnosis and timely management.AbstractSarcoidosis is a systemic disease that can affect various organs, leading to a diverse range of clinical manifestations that make diagnosis challenging. Here, we present a case of sarcoidosis in a middle‐aged male who presented with cirrhosis. The cause of cirrhosis remained unknown for 4 years until the development of lymphadenopathy and ground‐glass opacities on lung imaging. A liver biopsy was performed, which revealed noncaseating granulomatous inflammation, thereby identifying sarcoidosis as the cause of cirrhosis. The patient was treated with oral steroids, which slightly improved his liver function over a short period. Given the diverse presentations of sarcoidosis, it should be considered as a possible differential diagnosis in cases of cryptogenic cirrhosis.

A Multichannel CT and Radiomics-Guided CNN-ViT (RadCT-CNNViT) Ensemble Network for Diagnosis of Pulmonary Sarcoidosis.

Pulmonary sarcoidosis is a multisystem granulomatous interstitial lung disease (ILD) with a variable presentation and prognosis. The early accurate detection of pulmonary sarcoidosis may prevent progression to pulmonary fibrosis, a serious and potentially life-threatening form of the disease. However, the lack of a gold-standard diagnostic test and specific radiographic findings poses challenges in diagnosing pulmonary sarcoidosis. Chest computed tomography (CT) imaging is commonly used but requires expert, chest-trained radiologists to differentiate pulmonary sarcoidosis from lung malignancies, infections, and other ILDs. In this work, we develop a multichannel, CT and radiomics-guided ensemble network (RadCT-CNNViT) with visual explainability for pulmonary sarcoidosis vs. lung cancer (LCa) classification using chest CT images. We leverage CT and hand-crafted radiomics features as input channels, and a 3D convolutional neural network (CNN) and vision transformer (ViT) ensemble network for feature extraction and fusion before a classification head. The 3D CNN sub-network captures the localized spatial information of lesions, while the ViT sub-network captures long-range, global dependencies between features. Through multichannel input and feature fusion, our model achieves the highest performance with accuracy, sensitivity, specificity, precision, F1-score, and combined AUC of 0.93 ± 0.04, 0.94 ± 0.04, 0.93 ± 0.08, 0.95 ± 0.05, 0.94 ± 0.04, and 0.97, respectively, in a five-fold cross-validation study with pulmonary sarcoidosis (n = 126) and LCa (n = 93) cases. A detailed ablation study showing the impact of CNN + ViT compared to CNN or ViT alone, and CT + radiomics input, compared to CT or radiomics alone, is also presented in this work. Overall, the AI model developed in this work offers promising potential for triaging the pulmonary sarcoidosis patients for timely diagnosis and treatment from chest CT.