Sarcoidosis presenting with and without Löfgren's syndrome: Clinical, radiological and behavioral differences observed in a group of 691 patients

Rubio‐Rivas, Manuel; Franco, Jonathan; Corbella, Emili

doi:10.1016/j.jbspin.2019.10.001

Cited by 26 publications

(27 citation statements)

References 41 publications

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“…Risk factors related to chronicity were older age, stage II at diagnosis, and need of treatment. 19 Serum angiotensin-converting enzyme level, PFT results, and gallium-67 scan activity at diagnosis were not related to the persistence of disease activity or recurrence in a Spanish study with 186 subjects. 38 Sarcoidosis is a genetically complex disease, with many genes contributing, both as risk factors and with an influence on the disease course.…”

Section: General Factorsmentioning

confidence: 78%

“…36,38 Respiratory symptoms and abnormalities in lung function tests are infrequent and less prevalent in LS in comparison to "non-LS" patients. 19,38 Most patients present with radiographic stage I, bilateral hilar lymphadenopathy (BHL). Additional parenchymal involvement (stage II) occurs in approximately 20% of the patients.…”

Section: General Factorsmentioning

confidence: 99%

“…36 Young to middle-aged adults are more likely to present with this manifestation, with a median age of 37 years, and a second peak around 40 to 60 years. 19,29,38 Specific components of LS differ based on gender: whereas EN (together with ankle arthritis or periarticular inflammation) is found predominantly in women (3-6 times more frequently than in men), a marked articular presentation without EN is seen more commonly in men. 29,36 In contrast to "non-LS" patients, who commonly display an insidious onset and slower progression, often culminating in a chronic disease state, LS portends a favorable prognosis and frequently experiences spontaneous recovery, with a high rate of resolution within the first 2 years (approximately 85%).…”

Section: General Factorsmentioning

confidence: 99%

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Nonlife-Threatening Sarcoidosis

Castro

Pereira

2020

Semin Respir Crit Care Med

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Sarcoidosis is a systemic granulomatous disease of unknown etiology. The outcome is quite variable and is mainly related to persistent inflammatory processes and the development of fibrosis. Many prognostic factors have been described, but the disease evolution is not yet entirely known. The nonthreatening course is characterized by spontaneous involution or stability after treatment withdrawal. Löfgren's syndrome is a subset within the spectrum of sarcoidosis phenotypes, composed of acute onset of fever, bilateral hilar lymphadenopathy, erythema nodosum and/or bilateral ankle periarticular inflammation/arthritis, specifically characterized by a self-limiting disease course. In contrast, advanced fibrotic sarcoidosis with pulmonary hypertension phenotype is correlated with a poor prognosis. Further studies are necessary to detail phenotypes to better understand the mechanisms of the disease and plan future clinical therapeutic studies.

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Section: General Factorsmentioning

confidence: 78%

Section: General Factorsmentioning

confidence: 99%

Section: General Factorsmentioning

confidence: 99%

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Nonlife-Threatening Sarcoidosis

Castro

Pereira

2020

Semin Respir Crit Care Med

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“…Joint involvement in sarcoidosis ranges from 6 to 35% (2,11,(13)(14)(15)(17)(18)(19). Clinical features of acute and chronic sarcoid arthropathies have rarely been analyzed (20,21). The chronic form is usually associated with parenchymal lung or other organ involvements and is relatively rare (up to 7%) (4,18,22,23).…”

Section: Discussionmentioning

confidence: 99%

Acute and Chronic Sarcoid Arthropathies: Characteristics and Treatments From a Retrospective Nationwide French Study

et al. 2020

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Introduction: We aimed to analyze patients with acute and chronic joint involvements in sarcoidosis.Methods: This is a retrospective multicenter analysis of patients with proven sarcoidosis, as defined by clinical, radiological, and histological criteria, with at least one clinical and/or ultrasonographic synovitis.Results: Thirty-nine patients with sarcoid arthropathy were included, and among them 19 had acute sarcoidosis (Lofgren's syndrome). Joint involvement and DAS44-CRP were not significantly different in acute and chronic sarcoid arthropathies. Acute forms were more frequent than chronic sarcoid arthropathy in Caucasians, without any difference of sex or age between these 2 forms. Joint involvement was frequently more symmetrical in acute than chronic forms (100 vs. 70%; p < 0.05), with a more frequent involvement in wrists and ankles in acute forms, whereas the tender and swollen joint counts and the DAS44-CRP were similar between the 2 groups. Skin lesions were significantly more frequent in patients with acute forms [17 (89%) vs. 5 (25%); p < 0.05] and were erythema nodosum in all patients with Löfgren's syndrome and sarcoid skin lesions in those with chronic sarcoidosis. Among 20 patients with chronic sarcoidosis, treatment was used in 17 (85%) cases, and consisted in NSAIDs alone (n = 5; 25%), steroids alone (n = 5; 25%), hydroxychloroquine (n = 2; 20%), methotrexate (n = 3; 15%), and TNF inhibitors (n = 2; 10%). A complete/partial joint response was noted in 14 (70%) cases with a DAS44-CRP reduction of 2.07 [1.85–2.44] (from 3.13 [2.76–3.42] to 1.06 [0.9–1.17]; p < 0.05).Conclusion: Sarcoid arthropathies have different clinical phenotypes in acute and chronic forms and various treatment regimens such as hydroxychloroquine and methotrexate could be used in chronic forms.

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“…Löfgren's syndrome follows many of the same epidemiologic trends of sarcoidosis. It affects women more than men, with Scandinavian ethnicity having the highest incidence [6,7]. There also seems to be a temporal clustering of incidence, with rates highest in the months from March to July [6].…”

Section: Epidemiologymentioning

confidence: 99%

Löfgren’s Syndrome

Wang¹,

Singh²

2021

Rare Diseases - Diagnostic and Therapeutic Odyssey

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Löfgren’s syndrome presents as acute sarcoid arthritis, with a triad of hilar adenopathy, acute polyarthritis and erythema nodosum. Löfgren’s syndrome is self-limited, erythema nodosum, hilar adenopathy and acute polyarthritis usually resolve within a few weeks to months, however polyarthritis can last for up to 2 years. Treatment involves symptomatic control with NSAIDs/colchicine or oral glucocorticoids until symptoms resolve, if disease is resistant to these therapies, hydroxychloroquine, methotrexate or infliximab can be used. Löfgren’s syndrome is a rare presentation of sarcoidosis occurring in only about 5–10% of sarcoid patients. It is, however, important to recognize as it is the most common form of acute sarcoid arthritis and prompt treatment can prevent unnecessary prolonged discomfort for patients.

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Sarcoidosis presenting with and without Löfgren's syndrome: Clinical, radiological and behavioral differences observed in a group of 691 patients

Cited by 26 publications

References 41 publications

Nonlife-Threatening Sarcoidosis

Nonlife-Threatening Sarcoidosis

Acute and Chronic Sarcoid Arthropathies: Characteristics and Treatments From a Retrospective Nationwide French Study

Löfgren’s Syndrome

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