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Journal of Ayub Medical College,... 2023A multi-organ granulomatous disease with characteristic lung manifestations, sarcoidosis generally responds well to glucocorticoid therapy but 10% of cases are...
A multi-organ granulomatous disease with characteristic lung manifestations, sarcoidosis generally responds well to glucocorticoid therapy but 10% of cases are refractory necessitating immunosuppressive therapy. A 58-year-old lady presented with a dry cough and progressively worsening shortness of breath for the last 12 months. On investigation, her ESR was raised but cultures, malignancy screen and TB quantiferon were negative. HRCT chest demonstrated multiple pulmonary nodules with hilar lymphadenopathy and CT guided biopsy revealed non-caseating granuloma. She was diagnosed with Pulmonary Sarcoidosis and started on oral steroids with minimal improvement. Azathioprine was added but due to gastric intolerance switched to methotrexate. Her disease however continued to worsen and infliximab was started but she developed a severe allergic reaction. She was then started on mycophenolate mofetil but her chest imaging continued to worsen. After failing prednisone, azathioprine, methotrexate, infliximab and mycophenolate mofetil, the patient was started on rituximab.
Topics: Humans; Female; Middle Aged; Methotrexate; Infliximab; Mycophenolic Acid; Azathioprine; Sarcoidosis; Granuloma
PubMed: 38404097
DOI: No ID Found -
Clinics in Chest Medicine Mar 2024Cardiac involvement is a major cause of morbidity and mortality in patients with sarcoidosis. It is important to distinguish between clinical manifest diseases from... (Review)
Review
Cardiac involvement is a major cause of morbidity and mortality in patients with sarcoidosis. It is important to distinguish between clinical manifest diseases from clinically silent diseases. Advanced cardiac imaging studies are crucial in the diagnostic pathway. In suspected isolated cardiac sarcoidosis, it's key to rule out alternative diagnoses. Therapeutic options can be divided into immunosuppressive agents, guideline-directed medical therapy, antiarrhythmic medications, device/ablation therapy, and heart transplantation.
Topics: Humans; Cardiomyopathies; Sarcoidosis; Diagnostic Imaging; Heart Transplantation
PubMed: 38245360
DOI: 10.1016/j.ccm.2023.08.006 -
The Journal of Clinical Investigation Dec 2023Sarcoidosis is a disease of unknown etiology in which granulomas form throughout the body and is typically treated with glucocorticoids, but there are no approved...
Sarcoidosis is a disease of unknown etiology in which granulomas form throughout the body and is typically treated with glucocorticoids, but there are no approved steroid-sparing alternatives. Here, we investigated the mechanism of granuloma formation using single-cell RNA-Seq in sarcoidosis patients. We observed that the percentages of triggering receptor expressed on myeloid cells 2-positive (TREM2-positive) macrophages expressing angiotensin-converting enzyme (ACE) and lysozyme, diagnostic makers of sarcoidosis, were increased in cutaneous sarcoidosis granulomas. Macrophages in the sarcoidosis lesion were hypermetabolic, especially in the pentose phosphate pathway (PPP). Expression of the PPP enzymes, such as fructose-1,6-bisphosphatase 1 (FBP1), was elevated in both systemic granuloma lesions and serum of sarcoidosis patients. Granuloma formation was attenuated by the PPP inhibitors in in vitro giant cell and in vivo murine granuloma models. These results suggest that the PPP may be a promising target for developing therapeutics for sarcoidosis.
Topics: Humans; Animals; Mice; Pentose Phosphate Pathway; Sarcoidosis; Granuloma; Macrophages; Glucocorticoids
PubMed: 38038136
DOI: 10.1172/JCI171088 -
Ugeskrift For Laeger Aug 2023Tattoo-associated sarcoidosis is characterized by granulomas in tattoos with or without the involvement of other organ systems such as the lungs and eyes.... (Review)
Review
Tattoo-associated sarcoidosis is characterized by granulomas in tattoos with or without the involvement of other organ systems such as the lungs and eyes. 18F-fluorodeoxyglucose (18F-FDG PET is a nuclear medicine imaging study that can differentiate between metabolically over-active areas and normal tissue. Thus, this review finds that 18F-FDG-PET/CT imaging can be used to image inflammatory activity in tattoos and in case of papulonodular tattoo reaction be used to investigate possible systemic sarcoidosis.
Topics: Humans; Fluorodeoxyglucose F18; Positron Emission Tomography Computed Tomography; Sarcoidosis; Tattooing; Granuloma
PubMed: 37615154
DOI: No ID Found -
CMAJ : Canadian Medical Association... Jul 2023
Topics: Humans; Allopurinol; Disease Progression; Drug Hypersensitivity Syndrome
PubMed: 37460122
DOI: 10.1503/cmaj.221575-f -
Actas Dermo-sifiliograficas Sep 2023
Topics: Humans; Sarcoidosis
PubMed: 36273555
DOI: 10.1016/j.ad.2022.05.026 -
Journal of Drugs in Dermatology : JDD Jul 2023Cutaneous sarcoidosis presents in 25% of all sarcoidosis cases. African American populations, particularly African American women, are more likely to develop the...
Cutaneous sarcoidosis presents in 25% of all sarcoidosis cases. African American populations, particularly African American women, are more likely to develop the dermatologic manifestations of the disease. There are several types of skin manifestations of sarcoidosis, which can make it more difficult to diagnose it clinically. Given the higher incidence of sarcoidosis and the poorer outcomes in these populations, it is essential to understand and recognize the variety of dermatologic symptoms associated with sarcoidosis. By doing so, patients can be diagnosed and treated earlier in their disease progression. Williams JR, Frey C, Cohen GF. Cutaneous sarcoidosis in skin of color. J Drugs Dermatol. 2023;22(7):695-697. doi:10.36849/JDD.7008.
Topics: Female; Humans; Black or African American; Sarcoidosis; Skin; Skin Diseases
PubMed: 37410043
DOI: 10.36849/JDD.7008 -
Clinics in Chest Medicine Mar 2024Sarcoidosis frequently affects the eye and can do so in many different ways. Sarcoidosis causing uveitis can have distinctive features that facilitate identifying... (Review)
Review
Sarcoidosis frequently affects the eye and can do so in many different ways. Sarcoidosis causing uveitis can have distinctive features that facilitate identifying sarcoidosis as the cause of the uveitis. Progress is being made in elucidating ocular sarcoidosis, as for example, by transcriptomics, genetics, therapy, and imaging.
Topics: Humans; Sarcoidosis; Uveitis
PubMed: 38245371
DOI: 10.1016/j.ccm.2023.08.003 -
JAMA Dermatology Jan 2024Drug reaction with eosinophilia and systemic symptoms (DRESS) is a rare but potentially fatal drug hypersensitivity reaction. To our knowledge, there is no international...
IMPORTANCE
Drug reaction with eosinophilia and systemic symptoms (DRESS) is a rare but potentially fatal drug hypersensitivity reaction. To our knowledge, there is no international consensus on its severity assessment and treatment.
OBJECTIVE
To reach an international, Delphi-based multinational expert consensus on the diagnostic workup, severity assessment, and treatment of patients with DRESS.
DESIGN, SETTING, AND PARTICIPANTS
The Delphi method was used to assess 100 statements related to baseline workup, evaluation of severity, acute phase, and postacute management of DRESS. Fifty-seven international experts in DRESS were invited, and 54 participated in the survey, which took place from July to September 2022.
MAIN OUTCOMES/MEASURES
The degree of agreement was calculated with the RAND-UCLA Appropriateness Method. Consensus was defined as a statement with a median appropriateness value of 7 or higher (appropriate) and a disagreement index of lower than 1.
RESULTS
In the first Delphi round, consensus was reached on 82 statements. Thirteen statements were revised and assessed in a second round. A consensus was reached for 93 statements overall. The experts agreed on a set of basic diagnostic workup procedures as well as severity- and organ-specific further investigations. They reached a consensus on severity assessment (mild, moderate, and severe) based on the extent of liver, kidney, and blood involvement and the damage of other organs. The panel agreed on the main lines of DRESS management according to these severity grades. General recommendations were generated on the postacute phase follow-up of patients with DRESS and the allergological workup.
CONCLUSIONS AND RELEVANCE
This Delphi exercise represents, to our knowledge, the first international expert consensus on diagnostic workup, severity assessment, and management of DRESS. This should support clinicians in the diagnosis and management of DRESS and constitute the basis for development of future guidelines.
Topics: Adult; Humans; Drug Hypersensitivity Syndrome; Consensus; Delphi Technique; Eosinophilia; Surveys and Questionnaires
PubMed: 37966824
DOI: 10.1001/jamadermatol.2023.4450