Manifestation Of Sarcoidosis Research Articles

CARD-SARC: mixed-method study to develop a disease-specific health-related quality of life tool for cardiac sarcoidosis

Abstract Introduction Sarcoidosis is a rare inflammatory condition caused by the formation of lumps (granulomas) of abnormal tissue in any organ, and can affect physical, functional, mental, social and economic aspects of life. People living with cardiac sarcoidosis (CS) are likely to have worse clinical outcomes and greater impairment on health-related quality of life (HRQoL) than other sarcoidosis manifestations. HRQoL is the main concern for people living with sarcoidosis. However, measuring HRQoL in people with CS is difficult as existing tools focus on other forms of sarcoidosis (for example, lung, eye or skin involvement) or for specific cardiac symptoms (for example, atrial fibrillation, angina or heart failure), but not specifically for CS. Purpose To develop a disease-specific patient-reported outcome measure (CARD-SARC) to assess the impact of CS on HRQoL. Methods A sequential exploratory mixed-method, multicentre observational cohort study using a clinimetric approach was conducted. The McMaster Framework for evaluative instruments involved three steps (item selection, item scaling and item reduction) which were complemented with recommendations from the consensus-based standards for the selection of health measurement instruments (COSMIN). The item-generation process connected data from quantitative (theory, research findings and systematic review) and qualitative components (qualitative semistructured interviews, clinical observations and experts’ opinion). A simultaneous triangulation (QUAN+qual) method was followed to ensure a comprehensive list of preliminary items. The formatting of items was guided by patient and public involvement (PPI) with a 2-weeks recall period. The item reduction strategy was led by PPI and an international multidisciplinary clinical CS-experts. Results 740 potential items were generated by converging all the different sources and techniques. After removal of duplicates and simultaneous triangulation, a preliminary list with 111 items was reviewed by the CS-expert panel with 26 members (including 11 pulmonologists, 7 cardiologists, 5 CS-patients and 3 specialist-nurses). After a consensus meeting, the CARD-SARC included 61 relevant items in six sections for pilot-testing: "functioning", "social, leisure and occupation", "emotional", psychological", "physical" and "healthcare/self-care". Conclusions This study combined a robust methodology with active PPI engagement to develop the first disease-specific HRQoL tool for CS. Each data source was planned independently, however the input from PPI members was critical during the simultaneous triangulation for item-generation. The contributions of CS-experts (members of the PPI group and clinical multidisciplinary experts) enhanced the content validity of the CARD-SARC. The CARD-SARC has the potential to influence clinical management and treatment options, future research and health-economic stakeholders’ decisions for CS.

Oral Sarcoidosis Preceding Sudden Cardiac Arrest: A Case Report

Abstract Background Sarcoidosis is a disease characterized by non-caseating granulomas and may affect any organ system. Cardiac involvement may lead to conduction abnormalities, heart failure, or malignant ventricular arrhythmias. As sarcoidosis may present with heterogeneous manifestations, a detailed past medical history may provide clues that help guide further workup. We present a rare case of a patient with undiagnosed oral sarcoidosis who subsequently experienced cardiac arrest from cardiac involvement. Case Summary A 43-year-old male with a history of palpitations and periodontitis consistent with oral sarcoidosis presents after experiencing sudden cardiac arrest. He was subsequently diagnosed with cardiac and pulmonary sarcoidosis. With contemporary management (both immunosuppression and antiarrhythmics), he has not experienced any recurrent arrhythmias. Discussion In the setting of cardiac arrest and non-ischaemic cardiomyopathy, a careful clinical history and targeted cardiac testing may help clinicians determine when to consider cardiac sarcoidosis as a diagnosis. While oral sarcoidosis is a very rare condition, this case highlights how infrequent manifestations of sarcoidosis may be encountered in the clinical setting.

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.

12268 Hypercalcemia Alert: Exploring The Link To Isolated Bone Marrow Sarcoidosis

Abstract Disclosure: M. Renzu: None. C.M. Hubers: None. V. mehta: None. A. Qazi: None. D.K. Yerasuri: None. Introduction: Sarcoidosis, a complex disorder marked by granuloma formation in diverse organs, often affects the lungs, but can involve various systems as well. Rare cases of bone marrow involvement may mimic conditions like multiple myeloma, emphasizing the need to consider sarcoidosis in patients with bone marrow abnormalities, especially those without typical myeloma features. Case Presentation: A 79-year-old white male with a medical history of hypertension, hyperlipidemia, deep venous thrombosis, prostate cancer in remission, chronic knee pain, and basal cell carcinoma presented for pre-operative evaluation, as he was scheduled for an elective orthopedic procedure. On routine lab work, he was found to have acute hypercalcemia and normocytic anemia. This finding raised suspicion for an underlying systemic pathology. He reported that he did not take any calcium or vitamin D supplements at home. Subsequent endocrine assessment, including evaluation of parathyroid hormone and vitamin D levels, revealed a non-PTH-mediated hypercalcemia. The persistence of hypercalcemia prompted further investigations, including assessments for multiple myeloma by oncology. The workup was negative for multiple myeloma but significant for granulomas on bone marrow biopsy, a finding consistent with sarcoidosis. Additionally, angiotensin converting enzyme levels were elevated at the time of diagnosis. Interventions led to the resolution of hypercalcemia following steroid therapy of prednisone 20 mg daily and close follow up with endocrinology. As part of his follow up, ACE levels are being regularly monitored, with the patient going for lab work every other month. Discussion: Bone marrow biopsies rarely reveal granulomas and sarcoidosis accounts for only a very small percentage of these findings. The standard workup for sarcoidosis typically does not include bone marrow biopsies. By integrating biopsies into the standard diagnostic protocol researchers may gain insights into the etiology and mechanisms driving this manifestation of sarcoidosis, ultimately improving diagnostic accuracy and patient care. Distinguishing sarcoidosis from multiple myeloma also poses diagnostic challenges, necessitating thorough evaluation in patients with bone marrow abnormalities. Despite its rarity, clinical suspicion for sarcoidosis should be considered in patients with underlying hematologic disorders or malignancies. Additionally, sarcoidosis exhibits geographical variation, being less common in Japanese men and more prevalent in African American women. This highlights the importance of considering demographic factors when evaluating patients for sarcoidosis and its extrapulmonary manifestations. Long-term outcomes in isolated bone marrow sarcoidosis remain unclear, warranting surveillance for progression to multiple myeloma or other lymphoproliferative disorders. Presentation: 6/2/2024

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.

Position paper of the Austrian Society for Rheumatology and the Austrian Society for Pneumology on the diagnosis and treatment of sarcoidosis 2024

In many cases sarcoidosis is a multisystemic disease that requires interdisciplinary medical cooperation in the diagnostics, treatment and medical care during follow-up. Due to the often chronic course, it is of utmost importance to include patients with their priorities and wishes at an early stage and extensively in disease management and to establish a shared decision making whenever possible. In the process of writing this joint position paper, the expert group on interstitial and orphan lung diseases of the Austrian Society for Pulmonology and the working group on rheumatological lung disorders of the Austrian Society for Rheumatology and Rehabilitation sought to include patient advocacy groups as well as experts for rare organ manifestations of sarcoidosis. This position paper is not only meant to reflect current scientific and clinical standards but should also focus the national expertise and by networking and exchange to be a first step to strengthen cooperation between stakeholders to ultimately improve care for patients with sarcoidosis.

Elevated serum angiotensin converting enzyme correlates with specific HLA-DRB1 alleles and extrapulmonary manifestations in sarcoidosis

BackgroundGenetics influence the clinical picture in sarcoidosis, a granulomatous heterogeneous disease often accompanied by elevated serum angiotensin converting enzyme (s-ACE). We aimed to investigate if certain HLA-DRB1 alleles correlate with the levels of s-ACE, known as a marker of the granuloma burden. MethodsMedical journals of patients with sarcoidosis from a Swedish clinical registry were retrospectively examined to extract the highest recorded s-ACE value and analysed in relation to patient characteristics including phenotype [Löfgren syndrome (LS)/ non-LS], chest X-ray staging according to Scadding, treatment with immunosuppressants, presence of extrapulmonary manifestations (EPM), HLA-DRB1 alleles and prognosis (resolving vs. non-resolving disease within 2 years). Data were analysed with Fisher's exact test and Bonferroni correction was applied for HLA analyses. ResultsOf 1204 patients included, 40% had s-ACE levels above reference value. In comparison with patients with normal s-ACE, those with elevated levels were more often classified into non-LS (78% vs 59%, p < 0.001), and Scadding stage II (50% vs 38%, p < 0.001) but less often Scadding stage I (33% vs 46%, p < 0.001) and had more often EPM (45% vs 23%, p < 0.001). The patients with HLA-DRB1×04 had more often elevated s-ACE (p < 0.01) while those with HLA-DRB1×03 commonly had normal levels (p < 0.001). ConclusionsIn this retrospective study, HLA alleles associated with s-ACE levels in sarcoidosis patients, which in turn correlated with occurrence of EPM. These findings shed some new light on possible mechanisms behind differences in s-ACE levels.

The Role of Video Thoracoscopy in the Diagnosis of Sarcoidosis

Sarcoidosis is a systemic autoimmune disease associated with the development of non-caseous granulomas. Early and accurate diagnosis of sarcoidosis is an important and difficult task, since the initial manifestations are diverse, nonspecific, and many patients are asymptomatic. Objective — to study the role of video thoracoscopy and to present our own experience of invasive diagnosis of sarcoidosis. Materials and methods. Patients included in the study were divided into 2 clinical groups depending on whether the diagnosis at admission coincided with the final diagnosis established after the morphological examination of lung biopsies or not. Group I — 74 patients with PDS and ML (41.6 %), whose diagnosis at admission coincided with the final clinical diagnosis (established on the basis of morphological examination of lung biopsies). Group II — 104 patients with PDS and ML (58.4 %), whose diagnosis upon admission did not coincide with the final clinical diagnosis. Results and discussion. Insufficient diagnosis of lung cancer (lung carcinomatosis) — 3 (2.9 %) cases at admission and 31 (29.8 %) cases after lung biopsy. The small percentage of diagnosis of interstitial, granulomatous lesions of the lungs or pneumonitis with systemic connective tissue pathology is also noteworthy - 3 (2.9 %) cases before lung biopsy and 19 (18.3 %) after. In patients who were diagnosed with pulmonary sarcoidosis without histological confirmation, such a pathology as tuberculosis was found in 14 (13.5 %), oncological lesion — 23 (22.1 %), interstitial or granulomatous lesion together with pneumonia in systemic pathology — 17 (16.3 %) and «other» — 20 (19.2 %) cases. We performed 178 VATS biopsies, among which lung biopsy prevailed — 67 (37.6 %) cases, pleural biopsy was the least performed — 18 (10.1 %) cases. Intraoperative complications were recorded in 2 (1.1 %) cases, in the form of bleeding from a lymph node, the elimination of which required the use of electrocoagulation, the use of local and systemic hemostatic agents. Postoperative complications were observed in 5 (2.8 %) cases and were caused by the lack of lung tightness and the resulting long-term discharge of air through the drainage. Conclusions. Manifestations of sarcoidosis often simulate other disseminated processes, so its invasive diagnosis is an effective and safe way of establishing the diagnosis, especially in complex cases and atypical clinical and radiological picture. Among invasive methods, preference should be given to mini-invasive methods, therefore, the optimal choice is VATS, which has high informativeness and a low percentage of postoperative complications and allows to evaluate a wide range of tissues such as the pleura, lymph nodes and lungs.

Cystic lung in sarcoidosis: Clinico-radiologic characteristic and evolution.

Sarcoidosis can manifest with atypical findings on chest computed tomography (CT). Cysts are a rare manifestation of lung sarcoidosis. The aim of the study was to describe a series of patients with cystic sarcoidosis and their clinical-radiological characteristics and progression. In this retrospective, bicentric study we recruited all patients affected by sarcoidosis with lung cystic lesions at chest CT. We collected clinical characteristics, pulmonary tests and tracked number, distribution and size of the cysts at diagnosis and at the last evaluation. Twelve patients (6 males, median age 53 years) were identified (prevalence: 1.9%; 95% Confidence Interval: 0.8%-2.9%). All patients presented multiple cystic lesions (median number: 14 [range: 2-216]) with a bilateral distribution in 10/12, micronodules and nodules in 11/12 and fibrotic lesions in 4/12. Seven patients had normal lung function test, three had an obstructive syndrome, one had a restrictive syndrome and one had coexistence of both. During follow-up (median: 10 years [range 1-16 years]), an increase of the number of cysts was observed in four patients. At last evaluation, 3/12 patients experienced a decline of forced vital capacity >10% and 3/12 patients a decline of diffusing capacity for carbon monoxide (DLCO) >10%. A lower DLCO at diagnosis, and the presence of nodules or fibrotic lesions on CT were associated with an increase in the number of cysts. Cystic lung lesions are rare in patients with sarcoidosis and do not influence long term prognosis.

Aseptic meningitis with recurrent headache episodes, vomiting, and central fever as first manifestation of isolated neurosarcoidosis: a case report

BackgroundNeurosarcoidosis is a rare entity, usually within the context of systematic sarcoidosis. Isolated neurosarcoidosis and especially a manifestation with pachymeningitis is a notable rarity.Case ReportA 26-year-old patient presented to the emergency department with acute onset, recurrent episodes of occipital headaches spreading over the whole cranium and vomiting without food consumption, for three days. The clinical examination did not reveal any neurological deficits. The laboratory exams showed no pathological findings. A CT examination with angiography did not detect any acute intracranial or vessel pathology. A lumbar puncture was performed to rule out subarachnoid hemorrhage. The results showed a lymphocytic pleocytosis of 400/µL, elevated protein levels of 1077 mg/dL and reduced glucose levels (CSF: 55 mg/dL, Serum: 118 mg/dL). Extensive infectiological examinations did not reveal any signs of infection, including Borrelia spp. and M. tuberculosis. No positive auto-antibodies or vasculitis-related auto-antibodies were detected. The CSF analysis showed negative oligoclonal bands but an isolated increase in β2-microglobulin, neopterin, and IL-2R levels. The MRI examination revealed a dural gadolinium-enhancement, pronounced in the basal cerebral structures and the upper segment of the cervical spine, consistent with neurosarcoidosis. Corticosteroid treatment rapidly led to a significant improvement of the symptoms. No systemic manifestations of sarcoidosis were found.ConclusionsThis case report aims to highlight aseptic meningitis with atypical, acute onset headache attacks as a possible manifestation of isolated neurosarcoidosis. Neurosarcoidosis is a clinical entity that requires prompt treatment to avoid permanent neurological deficits.

Gastrointestinal Manifestations of Sarcoidosis: A State-of-the-Art, Comprehensive Review of the Literature-Practical Clinical Insights and Many Unmet Needs on Diagnosis and Treatment.

This comprehensive literature review explores the involvement of the gastrointestinal (GI) tract in sarcoidosis, a multisystem granulomatous disorder of unknown etiology. GI sarcoidosis presents a diagnostic and therapeutic challenge due to its rarity and nonspecific clinical manifestations, including overlap with other gastrointestinal diseases. We conducted a comprehensive screening of articles addressing the clinical features, diagnostic approaches, and treatment strategies for GI sarcoidosis. Our findings reveal that GI sarcoidosis can affect any part of the gastrointestinal tract, with the stomach and small intestine being the most involved. Clinical presentations range from asymptomatic cases to severe complications such as obstruction and perforation, with reflux being a common symptom. Diagnosis is often delayed due to the nonspecific nature of symptoms and the need for histopathological confirmation. Therapeutic approaches are poorly defined, typically involving corticosteroids as the mainstay of treatment. However, the long-term efficacy and safety of these treatments remain uncertain in this patient group, given the significant risks and complications associated with prolonged glucocorticoid therapy. There is a clear need to develop accurate diagnostic protocols to distinguish GI sarcoidosis from other conditions and to establish standardized therapeutic guidelines to optimize patient outcomes. Further research is essential to enhance our understanding and management of this complex condition.

Neurologic Manifestations of Rheumatologic Disorders.

This article provides an overview of the neurologic manifestations of sarcoidosis and select rheumatologic disorders. An approach to the assessment and differential diagnosis of characteristic clinical presentations, including meningitis and vasculitis, is also reviewed. A review of treatment options is included as well as discussion of distinct areas of overlap, including rheumatologic disease in the setting of neuromyelitis spectrum disorder and demyelinating disease in the setting of tumor necrosis factor-α inhibitors. An increased understanding of the immune mechanisms involved in sarcoidosis and rheumatologic diseases has resulted in a greater diversity of therapeutic options for their treatment. Evidence directing the treatment of the central nervous system (CNS) manifestations of these same diseases is lacking, with a paucity of controlled trials. It is important to have a basic knowledge of the common CNS manifestations of rheumatologic diseases and sarcoidosis so that they can be recognized when encountered. In the context of many systemic inflammatory diseases, including systemic lupus erythematosus, IgG4-related disease, and sarcoidosis, CNS disease may be a presenting feature or occur without systemic manifestations of the disease, making familiarity with these diseases even more important.

Current Practices and Emerging Therapies to Optimize Heart Failure Management in Cardiac Sarcoidosis: A Systematic Review.

Cardiac sarcoidosis (CS) is a distinctive manifestation of sarcoidosis, a multisystemic inflammatory disorder that is characterized by non-necrotizing granulomas. CS can lead to arrhythmias, heart failure (HF), and sudden cardiac death. The diagnosis of CS involves imaging in the form of a two-dimensional echocardiogram, cardiac magnetic resonance imaging (MRI), an 18-fluoro-deoxyglucose positron emission tomography (FDG-PET) scan, and an endomyocardial biopsy. Treatment of CS entails corticosteroids, immunosuppressive agents, monoclonal antibodies, and, in advanced cases, heart transplantation (HTx). This systematic review follows Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) 2020 guidelines, focusing on HF in sarcoidosis patients. Eligibility criteria include recent (2019-2024) research papers on sarcoidosis-induced heart failure, excluding other causes. The databases searched were PubMed, Google Scholar, and ScienceDirect. From 36,755 initial articles, 2,060 remained after filtering, and 17 were selected for quality assessment. Based on quality assessment, 11 studies were included in the final review. In CS, a variety of treatment strategies can be implemented. Corticosteroids are the first-line therapeutic options, and in the majority of cases, they are very successful in controlling the disease progression. Immunosuppressive agents like methotrexate and azathioprine are used to avoid long-term steroid use. Both corticosteroids and immunosuppressives act by reducing inflammation and preventing myocardial scarring. Biological agents like infliximab and adalimumab prevent disease progression by targeting specific inflammatory pathways and are used in refractory cases. Regular HF management drugs like angiotensin-converting enzyme (ACE) inhibitors, angiotensin receptor blockers (ARBs), sodium-glucose transport protein 2 (SGLT2) inhibitors, beta-blockers, and diuretics help in optimizing cardiac function. In severe cases, a left ventricular assist device (LVAD) may be required. The ultimate treatment for end-stage CS is HTx, which has to be supplemented with a strong, individualized regimen of glucocorticoids and immunosuppressives to avoid graft rejection and to control sarcoidosis. Due to a lack of standard protocols for management and limited knowledge about CS, the ideal treatment of HF is still a matter of debate. Hence, further research and clinical trials need to be performed to optimize patient outcomes.

Hypercalcaemia in gastrointestinal stromal tumour and sarcoidosis: a case report

BackgroundHypercalcaemia is a common manifestation of sarcoidosis but is sparingly described in gastrointestinal stromal tumours (GISTs). We describe a case of acute kidney injury and hypercalcemia resulting from simultaneous diagnosis of GIST and sarcoidosis, the presentation of which has not yet been reported.Case PresentationA 61-year-old male presented with acute kidney injury and hypercalcemia, with elevated 1,25-dihydroxyvitamin D levels. Investigations demonstrated a large gastric antral mass which was resected and proven to be GIST. Histopathology of incidentally found liver nodules revealed non-necrotising epithelioid granulomas consistent with concomitant sarcoidosis. The hypercalcemia was successfully treated with bisphosphonate therapy, resection of the GIST and a four month course of corticosteroids, which was truncated due to a mycobacterial infection.ConclusionsOur case report is the first to describe hypercalcemia due to GIST and biopsy-proven sarcoidosis, thereby raising the possibility of a common pathophysiological pathway relating the two entities. We review the literature describing the mechanisms of hypercalcaemia in GIST and the association between GIST and sarcoidosis.

The development of a disease-specific patient-reported outcome measure (CARD-SARC) to assess the impact of cardiac sarcoidosis on health-related quality of life

Abstract Introduction Sarcoidosis is a rare inflammatory condition caused by the formation of lumps (granulomas) of abnormal tissue in any organ, and can affect physical, functional, mental, social and economic aspects of life. People living with cardiac sarcoidosis (CS) are likely to have worse clinical outcomes and greater impairment on health-related quality of life (HRQoL) than other sarcoidosis manifestations. HRQoL is the main concern for people living with sarcoidosis. However, measuring HRQoL in people with CS is difficult as existing tools focus on other forms of sarcoidosis (for example, lung, eye or skin involvement) or for specific cardiac symptoms (for example, atrial fibrillation, angina or heart failure), but not specifically for CS. Purpose To develop a disease-specific patient-reported outcome measure (CARD-SARC) to assess the impact of CS on HRQoL. Methods A sequential exploratory mixed-method, multicentre observational cohort study using a clinimetric approach was conducted. The McMaster Framework for evaluative instruments was combined with the consensus-based standards for the selection of health measurement instruments (COSMIN) recommendations, involving three steps: item selection, item scaling and item reduction. The item-generation process used simultaneous triangulation (QUAN+qual) method, connecting quantitative data (theory, research findings and systematic review) with complementary qualitative components (interviews, clinical observations and experts’ opinion). The formatting of items was guided by patient and public involvement (PPI). The item reduction strategy was led by PPI and an international multidisciplinary clinical CS-experts. Results 740 potential items were generated by converging all the different sources and techniques. After removal of duplicates and simultaneous triangulation, a preliminary list with 111 items was reviewed by the CS-expert panel (including 11 pulmonologists, 7 cardiologists, 5 CS-patients and 3 specialist-nurses). After a consensus meeting, the CARD-SARC included 61 relevant items in six sections for pilot-testing: "functioning", "social, leisure and occupation", "emotional", psychological", "physical" and "healthcare/self-care". Conclusions This study used a robust methodology and active PPI involvement to develop the first disease-specific HRQoL tool for CS. The engagement and contributions of the PPI group in each step of the framework enhanced the content validity of the CARD-SARC. The CARD-SARC has the potential to influence clinical management and treatment options, future research and health-economic stakeholders’ decisions for CS.

A Comprehensive Review on the Electrocardiographic Manifestations of Cardiac Sarcoidosis: Patterns and Prognosis.

Cardiac sarcoidosis (CS) refers to cardiac involvement in sarcoidosis and is usually associated with worse outcomes. This comprehensive review aims to elucidate the electrocardiographic (ECG) signs and features associated with CS, as well as examine modern techniques and their importance in CS evaluation. The exact pathogenesis of CS is still unclear, but it stems from an abnormal immunological response triggered by environmental factors in individuals with genetic predisposition. CS presents with non-cardiac symptoms; however, conduction system abnormalities are common in patients with CS. The most common electrocardiographic (ECG) signs include atrioventricular blocks and ventricular tachyarrhythmia. Distinct patterns, such as fragmented QRS complexes, T-wave alternans, and bundle branch blocks, are critical indicators of myocardial involvement. The application of advanced ECG techniques such as signal-averaged ECG, Holter monitoring, wavelet-transformed ECG, microvolt T-wave alternans, and artificial intelligence-supported analysis holds promising outcomes for opportune detection and monitoring of CS. Timely utilisation of inexpensive and readily available ECG possesses the potential to allow early detection and intervention for CS. The integration of artificial intelligence models into ECG analysis is a promising approach for improving the ECG diagnostic accuracy and further risk stratification of patients with CS.

Vitiligo Associated With Sarcoidosis: Case Based Review

Sarcoidosis is characterized by non-caseating granulomatous inflammation that predominantly affects the lungs. Cutaneous involvement occurs in 20 to 35 percent of the patients and may emerge as the initial manifestation of the disease. Erythema nodosum and lupus pernio are the specific skin manifestations of cutaneous sarcoidosis. Many different skin lesions including hypopigmentation or hyperpigmentation that mimic other primary skin and systemic diseases may occur in sarcoidosis patients. Cutaneous involvement always poses a diagnostic dilemma in all patients with or without a previous sarcoidosis diagnosis. In this review, we present two sarcoidosis patients who subsequently developed vitiligo. The main aim is to discuss the underlying pathologic mechanisms of sarcoidosis associated with vitiligo and to define the potential relationship between these two autoimmune disorders. The second target is to specify a diagnostic pathway in sarcoidosis patients associated with skin involvement in the light of the current literature data.

I Don't Think This Is Gout….

Osseous sarcoidosis is a well-recognized manifestation of systemic sarcoidosis; however, its radiological natural history is poorly described. A 29-year-old man presented with pain, swelling, and erythema at the site of a recent minimal-trauma fracture of the right index finger proximal phalanx.

Isolated cardiac sarcoidosis: a case report

Introduction. Ventricular arrhythmias are a risk factor for sudden cardiac death. Abnormalities of cardiac rhythm and conduction may be the only subjective manifestation of isolated cardiac sarcoidosis, which occurs in 25% of the total number of patients with this disease.Brief description. We present a case of isolated cardiac sarcoidosis in a young female patient, the main clinical manifestation of which was attacks of palpitations caused by ventricular tachycardia and episodes of cardiogenic shock without a cardiovascular history. Echocardiography and contrast-enhanced cardiac magnetic resonance imaging (MRI) revealed asymmetric left ventricular hypertrophy and focal myocardial masses. Cardiac tumor and sarcoidosis were suspected. The final diagnosis was based on the results of histological and immunohistochemical analysis of endomyocardial biopsies documenting sarcoidosis. No systemic manifestations of sarcoidosis were found.Discussion. The case demonstrates the potential of a comprehensive paraclinical study in the diagnosis of cardiac sarcoidosis and its differential diagnosis with other cardiac pathologies.Conclusion. Cardiac sarcoidosis is difficult to diagnose and requires histological verification in patients with LV hypertrophy and arrhythmias, and physicians should be aware about this rare disease with unfavorable prognosis without specific treatment.

Arrhythmias in Cardiac Sarcoidosis: Management and Prognostic Implications.

Cardiac sarcoidosis (CS) is characterized by various arrhythmic manifestations ranging from catastrophic sudden cardiac death secondary to ventricular arrhythmia, severe conduction disease, sinus node dysfunction, and atrial fibrillation. The management of CS is complex and includes not only addressing the arrhythmia but also controlling the myocardial inflammation resultant from the autoimmune reaction. Arrhythmic manifestations of CS carry significant prognostic implications and invariably affect long-term survival in these patients. In this review, we focus on management of arrhythmic manifestation of cardiac sarcoidosis as well as risk stratification for sudden cardiac death in these patients.