Pulmonary sarcoidosis
Muhunthan Thillai, David R Moller, Keith C Meyer in Clinical Handbook of Interstitial Lung Disease, 2017
The initial clinical manifestations associate with different prognoses. For example, acute sarcoidosis or Löfgren syndrome is associated with remission in 70%–80% of patients, though in the authors’ experience, African Americans with this syndrome do not enjoy such a good prognosis. Other presentations associated with a good prognosis for remission include those with a stage 1 chest radiograph on initial diagnosis. Multiorgan disease (e.g. five or more organs), pulmonary fibrosis, and lupus pernio carry a poor prognosis with typically chronic, lifelong disease. True remitting/relapsing sarcoidosis is usually seen only in subsets of neurologic, ocular or non-lupus skin disease.
Test Paper 4
Teck Yew Chin, Susan Cheng Shelmerdine, Akash Ganguly, Chinedum Anosike in Get Through, 2017
Acute arthritis may be the first manifestation of sarcoidosis. It is mainly oligoarticular but occasionally polyarticular, and rarely monoarticular. Ankles are the most commonly involved joints. Enthesitis, tendinosis and tenosynovitis are well recognised, especially around the ankle. The triad of acute arthritis, bilateral hilar adenopathy and erythema nodosum is known as Lofgren syndrome. Chronic arthritis tends to involve the shoulders, hands, wrists, ankles and knees. Dactylitis can be seen; it is very similar to that seen in patients with psoriatic arthritis.
Clinical Cases
S. J. Copley, J. P. Kanne, D. M. Hansell in Thoracic Imaging, 2014
ii. The diagnosis is most likely to be sarcoidosis. Erythema nodosum (skin lesions typically involving the shins) often occurs at presentation in patients with sarcoidosis, together with arthralgia and iridocyclitis. The patient had Löfgren syndrome, a triad consisting of bilateral hilar lymphadenopathy, erythema nodosum and polyarticular arthritis/arthralgia. Löfgren syndrome is a characteristic acute form of presentation of sarcoidosis.
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
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
Non-HLA genes including TLRs, BTNL2, RAGE, IL23r, NOTCH4, ANXA11, NOD2/CARD15, and XAF1 are also identified to have polymorphisms in sarcoidosis [1,6]. A recent study by Levin et al., recognized one SNP, rs6502976, in intron 5 of gene X-linked Inhibitor of Apoptosis Associated Factor 1 (XAF1) with an impact on the transcriptional expression of XAF1, which regulates X-linked inhibitor of apoptosis protein (XIAP) negatively. The XIAP/XAF1 pathway takes part in apoptotic mechanisms. For sarcoidosis, if XAF1 and XIAP express differently, granuloma formation will be influenced [32]. On the other hand, the TNF gene encoding pro-inflammatory cytokine is essential for macrophage activation, cell migration, and leukocyte adhesion [33]. TNF-308 G/A variant mutation has been reported to be associated with Löfgren syndrome in Asian and Caucasian sarcoidosis patients [34].
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.
Related Knowledge Centers
- Chest Radiograph
- Erythema Nodosum
- Fever
- Lymph Node
- Sarcoidosis
- Arthritis
- Lymphadenopathy
- Lung
- Root of The Lung
- X-Ray