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Sarcoidosis is a systemic disease of unknown etiology characterized by the presence of noncaseating granulomas in any organ, most commonly the lungs and intrathoracic lymph nodes. A diagnosis of sarcoidosis should be suspected in any young or middle-aged adult presenting with unexplained cough, shortness of breath, or constitutional symptoms, especially among blacks or Scandinavians. Diagnosis relies on three criteria: (1) a compatible clinical and radiologic presentation, (2) pathologic evidence of noncaseating granulomas, and (3) exclusion of other diseases with similar findings, such as infections or malignancy. An early and accurate diagnosis of sarcoidosis remains challenging, because initial presentations may vary, many patients are asymptomatic, and there is no single reliable diagnostic test. Prognosis is variable and depends on epidemiologic factors, mode of onset, initial clinical course, and specific organ involvement. The optimal treatment for sarcoidosis remains unclear, but corticosteroid therapy has been the mainstay of therapy for those with significantly symptomatic or progressive pulmonary disease or serious extrapulmonary disease. Refractory or complex cases may require immunosuppressive therapy. Despite aggressive treatment, some patients may develop life-threatening pulmonary, cardiac, or neurologic complications from severe, progressive disease. End-stage disease may ultimately require lung or heart transplantation for eligible patients.

Sarcoidosis is a systemic inflammatory disease with a predilection for the respiratory system. Although most patients enter remission and have good long-term outcomes, up to 20% develop fibrotic lung disease, whereby granulomatous inflammation evolves to pulmonary fibrosis. There are several radiographic patterns of pulmonary fibrosis in sarcoidosis; bronchial distortion is common, and other patterns, including honeycombing, are variably observed. The development of pulmonary fibrosis is associated with significant morbidity and can be fatal. Dyspnea, cough, and hypoxemia are frequent clinical manifestations. Pulmonary function testing often demonstrates restriction from parenchymal involvement, although airflow obstruction from airway-centric fibrosis is also recognized. Complications of fibrotic pulmonary sarcoidosis include pulmonary hypertension from capillary obliteration and chronic aspergillus disease, with hemoptysis a common and potentially life-threatening manifestation. Immunosuppression is not always indicated in end-stage sarcoidosis. Lung transplantation should be considered for patients with severe fibrotic pulmonary sarcoidosis, as mortality is high in these patients.

Rationale Health status is impaired in patients with sarcoidosis. There is a paucity of tools that assess health status in sarcoidosis. The objective of this study was to develop and validate the King's Sarcoidosis Questionnaire (KSQ), a new modular health status measure. Methods Patients with sarcoidosis were recruited from outpatient clinics. The development of the questionnaire consisted of three phases: item generation; item reduction, Rasch analysis to create unidimensional scales and validation; repeatability testing. Results 207 patients with sarcoidosis (organ involvement: 184 lung, 54 skin, 45 eye disease) completed a 65-item preliminary questionnaire. 36 items were removed due to redundancy or poor fit to the Rasch model. The final version of the KSQ consisted of five modules (General health status, Lung, Skin, Eye, Medications). Internal consistency assessed with Cronbach's α coefficient was 0.70–0.93 for KSQ modules. Concurrent validity of the Lung module was high compared with St George's Respiratory Questionnaire (r=−0.83) and moderate when compared to forced vital capacity (r=0.49). Concurrent validity with skin-specific and eye-specific measures ranged from r=−0.4 to 0.8. The KSQ was repeatable over 2 weeks (n=39), intraclass correlation coefficients for modules were 0.90–0.96. Conclusions The KSQ is a brief, valid, self-completed health status measure for sarcoidosis. It can be used in the clinic to assess sarcoidosis from the patients’ perspective.

Sarcoidosis, a multisystemic granulomatous disease with unknown etiology, is characterized by formation of noncaseating granulomas, which can affect all organs. Recent studies have made outstanding achievement in understanding the pathology, etiology, genetics, and immune dysregulation involved in granuloma formation of sarcoidosis. Antigen stimulation in genetically predisposed individuals enhances the phagocytic activity of antigen-presenting cells, including macrophages and dendritic cells. CD4 + T cells initiate dysregulated immune responses and secrete significant quantities of inflammatory cytokines, including interleukin (IL)-2 and interferon-gamma (IFN-γ), which play a crucial role in modulating the aggregation and fusion of macrophages to form granulomas. The current therapeutic strategies focus on blocking the formation and spread of granulomas to protect organ function and alleviate symptoms. The efficacy of traditional treatments, such as glucocorticoids and immunosuppressants, has been confirmed in the management of sarcoidosis. Promising therapeutic agents encompass inhibitors of cytokines, like those targeting tumor necrosis factor (TNF)-α, as well as inhibitors of signaling pathways, such as Janus kinase (JAK) inhibitors, which exhibit favorable prospects for application. Although there has been progress in the identification of biomarkers for the diagnosis, prognosis, activity and severity of sarcoidosis, specific and sensitive biomarkers have yet to be identified. This review outlines recent advancements in the molecular mechanisms and therapeutic strategies for the sarcoidosis.

Sarcoidosis is a multisystem disease characterized by noncaseating granulomas in the affected organs, including skin, heart, nervous system, and joints. Diagnosis of sarcoidosis is generally based upon a compatible history, demonstration of granulomas in at least two different organs, negative staining and culture for acid fast bacilli, absence of occupational or domestic exposure to toxins, and lack of drug-induced disease. Involvement of the hollow organs is rare. Rather than being due to sarcoidosis, some reported mucosal lesions may simply have incidental granulomas. Extrinsic compression from lymphadenopathy can occur throughout the gastrointestinal tract. The stomach, particularly the antrum, is the most common extrahepatic organ to be involved, while the small bowel is the least common. Liver involvement frequently occurs and ranges from asymptomatic incidental granulomas to portal hypertension from granulomas in the portal triad, usually with relatively preserved liver function. CT scans show hepatosplenomegaly and adenopathy, followed in frequency by focal low-attenuation lesions of the liver and spleen. Ascites is usually a transudate from right heart failure (because of pulmonary hypertension) or portal hypertension (because of biliary cirrhosis). Rarely, an exudative ascites may occur from studding of the peritoneum with nodules. Pancreatic involvement presents as a mass, usually in the head or a diffusely firm, nodular organ. Corticosteroids should be instituted when organ function is threatened, usually lungs, eyes, and central nervous system. Their role in the treatment of hepatic sarcoidosis is unclear. The overall prognosis is good although most patients will have some permanent organ impairment. Cardiac and pulmonary diseases are the main causes of death.

A patient with cutaneous sarcoidosis that had been resistant to conventional treatment had clinical improvement with tofacitinib and a relapse with withdrawal of the medication. RNA sequencing of skin-lesion samples showed dysregulation of JAK-STAT that was responsive to tofacitinib.

BACKGROUND: Sarcoidosis is a granulomatosis of variable manifestation and evolution. It is crucial to assess the disease’s extent, activity, and severity to effectively manage and monitor patients with sarcoidosis. Fluorine-18 fluorodeoxyglucose positron emission tomography/computed tomography ([18F]FDG PET/CT) is a nuclear medicine technique that proved to be of great use in oncology. Its role in extra-oncological indications, including sarcoidosis, has been reported. MATERIAL AND METHODS: Twenty-five patients with sarcoidosis were retrospectively included. All patients underwent at least one [18F]FDG PET/CT scan. Five patients underwent a second [18F]FDG PET/CT to assess their response to corticosteroid treatment. Clinical data were gathered from medical records. Radiological and PET data were obtained from reports and digital imaging and communications in medicine (DICOM) archives. RESULTS: [18F]FDG PET/CT revealed active inflammation in 76% of patients. All had intrathoracic activity, including 76% mediastinal and 44% pulmonary lesions. Additionally, 60% of patients displayed associated extrathoracic activity, mainly in peripheral lymph nodes, spleen, and bone. Cardiac activity was reported in two patients. [18F]FDG PET/CT prompted a diagnostic biopsy in 32% of patients. [18F]FDG PET/CT had an impact on therapeutic decision-making in 60% of patients. Follow-up [18F]FDG PET/CT showed a complete metabolic response in two cases and an incomplete metabolic response in one patient. Disease progression was noted in two patients. CONCLUSIONS: Providing a complete functional map of the extent of inflammatory active disease, [18F]FDG PET/CT proves to be particularly useful in the management of sarcoidosis patients, by explaining persistent disabling symptoms, revealing suitable sites for biopsy, guiding therapeutic decisions and assessing treatment response.

Sarcoidosis is a systemic disorder of unknown cause that is characterized by its pathological hallmark, the noncaseating granuloma. Its presenting features are protean, ranging from asymptomatic but abnormal findings on chest radiography in many patients to progressive multiorgan failure in an unfortunate minority. The illness can be self-limited or chronic, with episodic recrudescence and remissions. Because the lungs and thoracic lymph nodes are almost always involved, most patients report acute or insidious respiratory problems, variably accompanied by symptoms affecting the skin, eyes, or other organs. The diverse manifestations of this disorder help fuel the prevailing hypothesis that sarcoidosis has more . . .

Purpose Sarcoidosis is a chronic granulomatous inflammatory disease of unknown etiology with heterogeneous outcomes. This study reviewed the clinical outcome status (COS) and organ involvement of Israeli sarcoidosis patients during a five-year period. Further, we compared our results to the ‘World Association of Sarcoidosis and Other Granulomatous Disease’ (WASOG) COS and the ‘A Case Control Etiologic Study of Sarcoidosis’ (ACCESS) instruments in order to evaluate their relevance to the Israeli population. Methods The retrospective study group consisted of 166 sarcoidosis patients for the period of 2010–2015. Data on demographic characteristics, presenting symptoms, co-morbidities, disease duration, lung function tests, treatment program, chest X-ray, and chest high-resolution computed tomography were collected. Results The median patient age was 62 ± 14, which was significantly higher than the WASOG and ACCESS cohorts ( p  < 0.0001), and the average disease duration was 9.8 ± 7.5 years. Resembling the ACCESS cohort, most patients were women (67.5%). The majority of patients suffered from constitutional symptoms (86%), as well as from respiratory symptoms (38.5%). Similarly to the ACCESS cohort, 91% of patients presented with lung involvement. However, significant differences in the involvement of other organs were noted, including lymph nodes (3 vs. 15.2%), liver (3.6 vs. 11.5%), CNS (7.2 vs. 4.6%), and joints (3.6 vs. 0.5%). In addition, significant differences were observed in the COS of the Israeli population in comparison to the WASOG data ( p  < 0.01). Conclusions Sarcoidosis in Israel is a unique and challenging disease with its clinical presentations that differ from previously reported studies.

Sarcoidosis affects people of all racial and ethnic groups and occurs at any age, although usually before the age of 50 years. The incidence of sarcoidosis varies widely throughout the world, probably because of differences in environmental exposures, case-surveillance methods, and predisposing HLA alleles, along with other genetic factors. This article summarizes advances in our understanding of sarcoidosis and addresses pitfalls in its diagnosis and treatment. This article summarizes advances in our understanding of sarcoidosis and addresses pitfalls in its diagnosis and treatment. The modern history of sarcoidosis, an enigmatic multisystem disease, goes back to 1899, when the pioneering Norwegian dermatologist Caesar Boeck coined the term to describe skin nodules characterized by compact, sharply defined foci of “epithelioid cells with large pale nuclei and also a few giant cells.” 1 Thinking this resembled sarcoma, he called the condition “multiple benign sarcoid of the skin.” 1 Since sarcoidosis was last reviewed in the Journal 10 years ago, 2 more than 5000 articles related to this condition have been published. This review summarizes recent advances and addresses pitfalls in the diagnosis and treatment of sarcoidosis. Epidemiology Sarcoidosis affects . . .