Sarcoid Arthropathy Research Articles

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

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.

EP22 A manifestation of severe sarcoid arthropathy in the distal interphalangeal joint

In 2011 a gentleman in his 50s presented with nasal blockage and bloody discharge. He was diagnosed with sarcoidosis and after 9 years of failed strategies to control his disease, he developed dactylitis. X-ray of the hands showed severe arthropathy in the distal interphalangeal joints. This case demonstrates an uncommon extrapulmonary manifestation of sarcoidosis. Although most of his follow up was with a respiratory clinic, his main symptoms were not due to interstitial lung disease, highlighting the importance of a multidisciplinary approach. To reduce the need for steroids, several DMARDs were tried illustrating that there are limited treatment options.

EP17 Finding the road to sarcoidosis

Case report - IntroductionSarcoidosis is an autoimmune, multi-system condition in which the formation of non-caseating, granulomas is a key histological feature. Clinical presentation can be variable and may lead to a delay in recognition. Most cases will resolve with minimal or no intervention. Awareness of the condition and features helps guide long-term management and as illustrated in cases below, rheumatologists may often be involved in helping diagnose and coordinate the patient pathway.Case report - Case descriptionA 51- year old female ex-smoker experienced 6 months of fatigue, dry cough, mild exertional dyspnoea, sweats, mild weight loss and arthralgia after a cholecystectomy. She described lesions typical of erythema nodosum coinciding with a raised CRP (58g/L) which later normalised. Other than an elevated serum ACE (71 U/L), rest of tests were normal.Plain chest radiograph was normal but a co-incidental CT abdomen for non-specific abdominal discomfort showed small volume abdominal lymphadenopathy. Further imaging showed bilateral mediastinal and hilar lymphadenopathy. Pulmonary function tests and joint ultrasound were normal. EBUS sampling (August 2014) excluded malignancy but confirmed sarcoid granulomas. She briefly required non-steroidals for arthralgia. Four years later, she is still well with resolution of lymphadenopathy.A 41-year old male non-smoker presented with 6 weeks of bilateral heel pain followed by myalgia, weight loss, headaches, sweats, intermittent blurred vision, and a non-specific neck rash. He was afebrile with normal urinalysis, CRPs 24-39 mg/L, CCP, ANCA, ANA negative, (Serum ACE sample insufficient). Infection screening (including TB) was negative. Slit lamp examination was normal.Trans-bronchial sampling of hilar lymphadenopathy seen on imaging excluded lymphoma but showed granulomas typical of sarcoidosis. The patient fully recovered within a few months without medication or recurrence.A 63-year-old female was referred with ankle pain and swelling after 5 months of erythematous leg swelling treated initially as cellulitis. She also had bilateral, intermittent leg cramps and recent intermediate uveitis. She was positive for HLA B27 and ANA (homogenous speckled pattern) with a raised serum ACE (98 U). ANCA was negative, creatinine kinase normal. Background included treated squamous cell carcinoma and degenerative disc disease.Ankle problems had resolved when seen possibly due to prednisolone for uveitis. EBUS sampling of bilateral hilar lymphadenopathy confirmed sarcoid histology. Since commencing azathioprine (50mg) for recurrent uveitis, she stays well.Case report - DiscussionSarcoidosis is a granulomatous systemic disease thought to be Th-1 mediated but pathogenesis remains unclear. Heterogeneity in presentation and organ involvement may lead to delays or missed diagnoses. Like these cases, patients may have one or more presentations to various medical specialities before a link is made.Careful note of antecedent history, current symptoms and examination findings can point towards a differential of sarcoid particularly if bilateral ankle involvement or typical skin lesions are present. Erythema nodosum can occur which the first case had described. Given the smoking and weight loss history, the differential of malignancy had to be excluded first. Sarcoid arthropathy, as seen in these cases, typically presents as arthralgia, myalgia, or arthritis in either acute or chronic form. Sometimes myopathy and bone involvement are seen though erosive disease is uncommon.Cases often have minimal or no respiratory symptoms but chest imaging can pick up features including bilateral hilar lymphadenopathy (more than 75% of cases) and less commonly pulmonary parenchymal changes (nodules, ground glass changes, fibrosis) or pleural effusions.Most cases will resolve over time with minimal intervention as in the first two cases. Some require non-steroidal anti-inflammatories. Steroids may be required if there are more inflammatory features affecting joints or other organs. Disease modifying therapies (biologic and non-biologic) have been used in more chronic or resistant cases.Sarcoid may co-exist with or mimic other conditions. In the last case, the unifying diagnosis of uveitis, skin changes and joint involvement seems to be sarcoid. However, it was interesting that the patient had mixed serology and showed some features of a seronegative arthritis profile as spondyloarthritis and sacroiliitis have been reported with sarcoidosis.Rheumatologists are often familiar with features of the condition. Thus, they can help link symptoms to guide appropriate investigations and further management with good outcomes. Case report - Key learning points Sarcoidosis can have a heterogeneous presentation so may take a while for diagnosis to be made Respiratory symptoms may not be present despite findings on chest imagingRheumatologists are often involved in diagnosis and treatment when patients with sarcoid related arthralgia or arthritis type symptoms get referredMost cases will resolve with minimal interventionEarly recognition can streamline investigations and management subsequently improving the patient journeyIn cases with a mixed autoantibody profile, there may be a discussion on whether one or more conditions are present to explain all the features

Clinical Characteristics of Sarcoidosis in Asian Population: A 14-year Single Center Retrospective Cohort Study from Thailand.

Background:Little is known about epidemiology and clinical characteristics of sarcoidosis in Asian population.Objectives:This study aimed to examine the epidemiology and clinical characteristics of Thai patients with sarcoidosis, using databases of a tertiary care medical center.Methods:Potential cases of sarcoidosis were identified from two sources, the medical record-linkage system and the pathology database of Siriraj Hospital, Mahidol University in Bangkok, Thailand. Patients with ICD-10-CM codes for sarcoidosis were identified and retrieved from the medical record-linkage system from 2005 to 2018. Patients with histopathology positive for non-caseating granuloma were identified and retrieved from the pathology database from the same time period. All potential cases underwent individual medical record review to confirm the diagnosis of sarcoidosis which required compatible clinical pictures supported by presence of non-caseating granuloma, radiographic evidence of intrathoracic sarcoidosis and exclusion of other granulomatous diseases.Results:From 2005 to 2018, 89 confirmed cases of sarcoidosis were identified. 80.9% of them were female and mean age at diagnosis was 46.8 years (standard deviation (SD) 13.9 years). The majority of patients had intrathoracic disease (81 cases; 91.0%) but less than half had respiratory symptoms (34 cases; 41.9%). Extrathoracic disease was common in this cohort that pulmonary sarcoidosis was accompanied by extrathoracic involvement in 53 patients (65.4%). Sarcoid uveitis was the most common extrathoracic disease (35 cases; 39.3%), followed by cutaneous sarcoidosis (24 cases; 26.9%), extrathoracic lymphadenopathy (18 cases; 22.5%) and sarcoid arthropathy (4 cases; 4.5%).Conclusion:The current study examined clinical characteristics of sarcoidosis in an Asian population and found high prevalence of uveitis and marked female predominance. (Sarcoidosis Vasc Diffuse Lung Dis 2020; 37 (4): e2020011)

The clinical characteristics of sarcoid arthropathy based on a prospective cohort study.

Sarcoidosis is known as a Th1-mediated disease, which can mimic many primary rheumatologic diseases or sometimes co-exist with them. Clinical characteristics of sarcoid arthropathy are not well described and the studies reported in the literature so far are mostly based on data from referrals. The aim of this study was to evaluate the incidence and clinical characteristics of sarcoid arthropathy. All our patients were prospectively evaluated in our rheumatology outpatient center from 2011 to 2015. A total of 114 (32 male) patients with sarcoidosis who were admitted to our clinic were included in the study. Clinical, demographical, laboratory, radiological and histological data of these patients obtained during 4-year follow-up and treatment period were compiled and analyzed. The mean patient age was 48.1 years (range, 20-82 years), and the mean disease duration was 40.5 months (range, 1-300 months). Sarcoid arthritis was observed in 71 (62.3%), and arthralgia in 106 (92.9%) patients. Out of the 71 patients with arthritis, 61 (85.9%) had involvement of ankle, 7 (9.8%) knee, 2 (2.8%) wrist, MCP and PIP joints, and 1 (1.4%) had shoulder periarthritis. Oligoarthritis (two to four joints) was the most common pattern followed by monoarthritis and polyarthritis. Arthritis and erytjhema nodosum and arthritis and female sex was found to be correlated (p = 0.03 and p = 0.001). Again, in patients with arthritis, even higher levels of CRP/ESR as well as ANA and RF positivity were observed (p = 0.03, p = 0.01, p = 0.01, and p = 0.02, respectively). A total of 11 patients had another rheumatic pathology concurrent with sarcoidosis. Inflammatory arthritis occurs in a majority of patients with sarcoidosis. Acute arthritis with bilateral ankle involvement is the most common pattern of sarcoid arthropathy. Sarcoidosis can mimic many primary rheumatic diseases or may coexist with them. Sarcoidosis should be considered not only as a mimicker but also as a Th1-mediated primary rheumatologic pathology.

Clinical Characteristics of Sarcoid Arthropathy: A Population-Based Study.

The epidemiology and clinical characteristics of sarcoid arthropathy are not well described, as only referral-based studies have been reported. This study aimed to use the data from a geographically well-defined population to characterize the clinical characteristics of sarcoid arthropathy. An inception cohort of patients with incident sarcoidosis in the years 1976-2013 in a geographically well-defined population was identified based on comprehensive individual medical records review. Inclusion required physician diagnosis supported by histopathology and radiologic features of intrathoracic sarcoidosis, compatible clinical presentation, and exclusion of other granulomatous diseases. Patients with joint pain were then identified from this cohort. In 1976-2013, all 345 incident cases of sarcoidosis were identified. Symptoms of joint pain occurred in 42 patients (mean age 41.2 years, 57.1% female, and 95% white), and 35 patients had swollen joint(s) on physical examination. Most patients had arthralgia prior to the diagnosis of sarcoidosis, with an average time to diagnosis of 21 days. Of the 35 cases, oligoarthritis (2 to 4 joints) was the most common pattern (88% of cases), followed by monoarthritis (6%) and polyarthritis (6%). Ankles were involved in 91% of cases. In the majority of patients (88%), the arthritis resolved within 6 weeks. Classic Lofgren's syndrome was observed in 11 patients (26%). Inflammatory arthritis occurs in a minority of patients with sarcoidosis. Acute oligoarthritis with bilateral ankle involvement was the most common pattern of sarcoid arthropathy. It should be noted that the generalizability of the results may be limited, as the cohort was predominantly white.

THU0508 Incidence of Inflammatory Joint Diseases in Northern Savo Area in Finland in 2010

BackgroundMonitoring of the incidence of inflammatory joint diseases may provide new insight into the etiology of these diseases.ObjectivesAll rheumatologists practising in the Northern Savo rheumatological outpatient departments collected data on...

Radiographic Improvement in Sarcoid Arthropathy after Infliximab Treatment: Figure 1.

To the Editor: We describe our experience on the use of infliximab in sarcoid arthropathy and partial reversal of the typical radiographic changes seen in sarcoid arthropathy after treatment. A 35-year-old African American man presented 5 years ago with bilateral hand arthritis, dactylitis, and a shiny, fleshy growth over the tip of the nose. Examination revealed bilateral proximal interphalangeal joint synovitis and severe deformity and resorption of the distal interphalangeal joints with dystrophic nail changes and diffuse dactylitis. The fleshy growth was indicative of a sarcoid-specific “lupus pernio” presentation, confirmed by skin biopsy, showing noncaseating granulomas. Chest radiography showed paratracheal and bilateral hilar lymphadenopathy without parenchymal disease. Laboratory evaluation revealed elevation of … Address correspondence to Dr. P. Efthimiou, Rheumatology Division, Lincoln Medical and Mental Health Center, 234 East 149th St., New York, NY 10451, USA. E-mail: pe53{at}cornell.edu

Sarcoidosis and sacroiliitis, a case report

Sarcoidosis is a multisystem disorder of unknown etiology characterized by the presence of non-caseating granulomas in the organs affected. Sarcoid arthropathy is a rare manifestation, and sacroiliitis is an unusual first manifestation of the disorder.

Acute Sarcoid Arthropathy—An Infective Cause?

Acute Sarcoid Arthropathy—An Infective Cause?

Colchicine Therapy in Sarcoid Arthropathy

To the Editor.— We read with great interest the article by Lindsley and Godfrey1 but wish to disagree with one of their conclusions. They suggested that oral corticosteroid therapy is the treatment of choice in sarcoid arthropathy because salicylate and other nonsteroidal anti-inflammatory drugs have only limited success in such cases. We believe, however, that this is not the case, because low-dosage colchicine therapy has provided good results in the agement of both the acute and chronic forms of sarcoid arthropathy when there was no adequate response to nonsteroidal anti-inflammatory agents and bed rest. Colchicine was first used in the treatment of sarcoid arthropathy by Kaplan,2,3 who reported a dramatic improvement, usually within a few days, with the use of 0.5 mg of oral colchicine daily. Subsequently, maintenance oral therapy with colchicine, 1 to 2 mg/d, was introduced to treat the chronic form of the disease, with similar beneficial results.3-7

Testicular sarcoidosis

A 20 year old black man presented with shortness of breath, hilar adenopathy, monoarticular arthritis, polydipsia and polyuria, and bilateral testicular masses. Transbronchial biopsy revealed noncaseating granulomas consistent with sarcoidosis. Subsequent work-up demonstrated hypothalamic dysfunction, and joint fluid analysis was consistent with sarcoid arthropathy. Concern over the possibility of a testicular neoplasm led to right testicular biopsy which revealed noncaseating granulomas consistent with testicular sarcoidosis. A short course of steroid therapy resulted in clinical improvement and complete resolution of the remaining left testicular mass.