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Sarcoidosis
Published in Lourdes R. Laraya-Cuasay, Walter T. Hughes, Interstitial Lung Diseases in Children, 2019
Erythema nodosum has been seen as part of Löfgren syndrome, with other manifestations of fever, fatigue, polyarthralgia and a Stage I chest film. Spontaneous resolution without therapy occurs within 6 months in 80% of cases. This is most commonly seen in young adult white women and is rare in children and black patients.1
Single best answer (SBA)
Published in Tristan Barrett, Nadeem Shaida, Ashley Shaw, Adrian K. Dixon, Radiology for Undergraduate Finals and Foundation Years, 2018
Tristan Barrett, Nadeem Shaida, Ashley Shaw, Adrian K. Dixon
Löfgren syndrome is a form of acute sarcoidosis with the triad of bihilar lymphadenopathy, erythema nodosum, and arthritis. It is associated with a good prognosis, with the majority having complete resolution of disease within two years. Serum ACE levels are often found to be elevated in sarcoid. The other listed options are all causes of bihilar lymphadenopathy.
Pulmonary sarcoidosis
Published in Muhunthan Thillai, David R Moller, Keith C Meyer, Clinical Handbook of Interstitial Lung Disease, 2017
There is wide variation in the reported frequency of sarcoidosis, with most studies finding incident rates ranging from 10–64/100,000 people (28). Higher frequencies are seen in Black populations in the United States and in women. Although these figures suggest sarcoidosis is a rare disease, lifetime estimates suggest the risk for Black populations may be >2% and ≥1% for White populations in Europe and the United States (29). Most cases are diagnosed between the ages of 20 and 50 years, with a later peak >65 years old; sarcoidosis is rarely diagnosed in children. The frequency of specific manifestations of sarcoidosis varies with ancestry. For example, lupus pernio which causes disfiguring facial lesions is more frequent in patients of African descent, while Löfgren syndrome (defined by the presence of constitutional symptoms, bilateral hilar lymphadenopathy, acute arthritis most commonly involving the ankles, feet, Achilles tendon, +/− erythema nodosum, and often uveitis) is more frequent in Scandinavian and Irish patients. Mortality rates approximate 1%–6% with advanced pulmonary, cardiac and neurologic manifestations responsible for most deaths.
An inflammatory triangle in Sarcoidosis: PPAR-γ, immune microenvironment, and inflammation
Published in Expert Opinion on Biological Therapy, 2021
Parnia Jabbari, Mona Sadeghalvad, Nima Rezaei
Sarcoidosis is a polygenic disorder, and several genes with different phenotypes are associated with disease development. Although further investigations are needed to determine, certain HLA-alleles have been distinguished to influence susceptibility to sarcoidosis [29]. In 2009, Grunewald J et al. showed that HLA- DRB1*03 (DR3) allele is associated with Löfgren syndrome, a type of acute sarcoidosis. In their study, 301 patients with Löfgren syndrome were included, and the significant association was found between HLA-DR3 allele and disease recovery within 2 years. However, 49% of HLA-DR3 negative patients developed a non-resolving disease [30]. In addition, HLA-DRB1*15 (DR15) and DRB1*14 (DR14) have been recognized to be associated with chronic non-resolving disease [31]. It has been mentioned that among the patients with sarcoid arthritis, HLA-B8 and HLA-B14 are more prevalent alleles and HLA-DRB1*04 is considerably rare. These patients are also carriers for HLA-DR3 allele [7].
Uveoparotid fever as a presentation of sarcoidosis
Published in Baylor University Medical Center Proceedings, 2019
Vignesh Ramachandran, Wasim Haidari, Christine Ahn, Rechelle Tull, Joseph L. Jorizzo
On exam, a 2-cm erythematous plaque without scale was noted on the left lateral arm (Figure 1b). Additionally, a 1.5-cm subcutaneous nodule of the right mandibular area and a skin-colored papule of the right nasal ala region were noted. A 6-mm punch biopsy of the left arm lesion was subsequently performed. The pathology report indicated noncaseating granulomas, which in this clinical context were pathognomonic for systemic sarcoidosis (Figure 1c). The patient’s cutaneous lesions were clinically interpreted as progression of her systemic sarcoidosis. As such, she was started on methotrexate 10 mg. A retrospective diagnosis of incomplete uveoparotid fever, a rare manifestation of sarcoidosis, was made. Lack of joint symptoms or erythema nodusum ruled out Löfgren syndrome, another acute sarcoidosis presentation.3
Clinical characteristics of sarcoidosis patients in Belgium
Published in Acta Clinica Belgica, 2022
Jolien De Ridder, Steven Ronsmans, Steven Vanderschueren, Wim Wuyts, Jonas Yserbyt
In 217 cases (93%) an attempt to obtain histopathological confirmation of a clinical suspicion of sarcoidosis was made. In 17 cases (7%) no data on histopathological examination were obtained from the patient file, among whom 6 patients with Löfgren syndrome, 6 patients with asymptomatic mediastinal/hilar lymph node enlargement, 3 patients with sarcoidosis of the eye and 2 cases of isolated sarcoid of the skin. However, seven of these patients showed a significant lymphocytosis on BAL. In some patients (8%) histological samples were obtained from more than 1 anatomical location.