Objective
Bone sarcoidosis is usually rare. Imaging procedures such as fluorine-18 fluorodeoxyglucose positron emission tomography/computed tomography (
18
F-FDG PET/CT) can reveal bone sarcoidosis ...with better sensitivity than conventional radiography. We aimed to describe bone sarcoidosis involvement detected with
18
F-FDG PET/CT.
Methods
This was an observational retrospective study of individuals with pulmonary sarcoidosis who underwent
18
F-FDG PET/CT. According to the ATS/ERS/WASOG criteria, sarcoidosis was diagnosed by the presence of clinical and/or imaging features of sarcoidosis and evidence of non-caseating epithelioid granulomas on a biopsy. We assessed clinical and
18
F-FDG PET/CT characteristics.
Results
Data for 85 patients with sarcoidosis (56.5% female, median age 47 range 21–80 years) were analyzed. The median follow-up was 4 years. Among 56 patients, sarcoidosis occurred in more than three organs. According to ATS/ERS/WASOG criteria, bone sarcoidosis was diagnosed in 12 (14%) patients. The spine was the most commonly affected location (92%,
n
= 11), followed by the pelvis (67%,
n
= 8), sternum (33%,
n
= 4), humerus (25%,
n
= 3), and fingers (17%,
n
= 2). Only peripheral adenopathy was associated with bone sarcoidosis (
p
= 0.04). Seven patients had a
18
F-FDG PET/CT follow-up, all showing a decrease of bone lesions.
Conclusion
Bone sarcoidosis occurred in 14% of patients with sarcoidosis, affecting multiple bones and mostly the axial skeleton.
18
F-FDG PET/CT seems a sensitive tool for diagnosis and follow-up of bone sarcoidosis.
Key Points
• Bone sarcoidosis is not rare.
• Bone sarcoidosis affects mainly the axial skeleton.
•
18
F-FDG PET/CT is useful for diagnosis and follow-up of bone sarcoidosis.
Recent advances in deciphering the genetic architecture of RA-ILD support the hypothesis of RA-ILD as a complex disease with a heterogeneous phenotype encompassing at least the usual interstitial ...pneumonia (UIP) and non-UIP high-resolution CT patterns. The results of genetic studies support the hypothesis of a common genetic background between idiopathic pulmonary fibrosis (IPF) and RA-ILD, and more specifically RA-UIP, a subset of the disease associated with a poor prognosis. Overall, these findings suggest the existence of shared pathogenic pathways between IPF and RA-ILD providing new opportunities for future intervention in RA-ILD, particularly with drugs that have been shown to be active in IPF.
Los recientes avances en el desciframiento de la arquitectura genética de la artritis reumatoide asociada a enfermedad pulmonar intersticial (EPI-AR) apoyan la hipótesis de que la EPI-AR es una enfermedad compleja, con un fenotipo heterogéneo, que abarca al menos los patrones de neumonía intersticial habitual (NIU) y de TC de alta resolución no NIU. Los resultados de los estudios genéticos apoyan la hipótesis de un trasfondo genético común entre la fibrosis pulmonar idiopática (FPI) y la EPI-AR, y más concretamente la NIU-AR, un subconjunto de la enfermedad asociado a un mal pronóstico. En conjunto, estos hallazgos sugieren la existencia de vías patogénicas compartidas entre la FPI y la EPI-AR que proporcionan nuevas oportunidades para futuras intervenciones en la EPI-AR, particularmente con fármacos que han demostrado ser activos en la FPI.
To describe the disease course of patients with early arthritis without rheumatoid factor (RF) and anti-citrullinated protein auto-antibodies (ACPA) in an inception cohort. To determine baseline ...predictors of fulfilling 2010 ACR/EULAR criteria for rheumatoid arthritis (RA) for these patients within 3 years.
Patients included in the multicenter ESPOIR cohort were compared at baseline and 3 years by whether they were negative for RF and ACPA ("seronegative") or positive for RF and/or ACPA ("seropositive"). Univariate analysis was used to determine the association between baseline variables in seronegative patients and RA classification. Stepwise multiple logistic regression was used to identify predictors of RA classification within 3 years, estimating odds ratios (ORs).
Among 354 seronegative patients, 224/340 with available data (65.9%) fulfilled RA classification at baseline and 189/233 (81.1%) at 3 years. As compared with seropositive patients, seronegative patients had lower DAS28 (p = 0.002) and lower modified total Sharp score (mTSS; p = 0.026) at baseline; DAS28 remission was similar (p = 0.634), but radiographic progression rate was lower in seronegative patients (p < 0.001) at 3 years. In seronegative patients, factors predicting RA classification within 3 years were additive (OR = 3.61), bilateral (OR = 2.59) and hand, wrist or forefeet involvement (OR = 3.87); presence of a trigger event (OR = 3.57); pain at rest (OR = 2.76); morning stiffness (OR = 2.62); number of tender joints (OR = 23.73); and mTSS (OR = 2.56).
"Seronegative" patients have less active disease at baseline and less radiographic progression during follow-up than "seropositive" patients. With inflammatory pain, symmetric involvement of numerous small joints and erosive disease, a classification of RA is likely.
The genetics of interstitial lung diseases Borie, Raphael; Le Guen, Pierre; Ghanem, Mada ...
European respiratory review,
09/2019, Volume:
28, Issue:
153
Journal Article
Peer reviewed
Open access
Interstitial lung diseases (ILDs) are a set of heterogeneous lung diseases characterised by inflammation and, in some cases, fibrosis. These lung conditions lead to dyspnoea, cough, abnormalities in ...gas exchange, restrictive physiology (characterised by decreased lung volumes), hypoxaemia and, if progressive, respiratory failure. In some cases, ILDs can be caused by systemic diseases or environmental exposures. The ability to treat or cure these ILDs varies based on the subtype and in many cases lung transplantation remains the only curative therapy. There is a growing body of evidence that both common and rare genetic variants contribute to the development and clinical manifestation of many of the ILDs. Here, we review the current understanding of genetic risk and ILD.
We aimed to compare the efficacy of steroids alone or associated with immunosuppressive drugs for the prevention of relapse in cardiac sarcoidosis (CS).
In this monocentric multidisciplinary ...retrospective single center study, all consecutive patients with histologically proven sarcoidosis hospitalized from January 2012 to December 2016 were considered. All patients with symptomatic CS were studied. Patients received steroids or steroids plus immunosuppressive drugs (IS) for CS treatment at diagnosis. The efficacy of each treatment strategy (steroids vs steroids + IS) was assessed by the cardiac relapse rate over follow up.
326 consecutive patients with histologically proven sarcoidosis were screened. Among them, 36 (11%) had symptomatic CS (20 (55.5%) men, median age at diagnosis 48.5 22.8–76). Twenty-four patients received steroids and 12 received steroids + IS (azathioprine n = 5, methotrexate n = 5, cyclophosphamide n = 2) at CS diagnosis. Over a median follow up of 3.6 1–15.2 years, 13 (36.1%) patients suffered a cardiac relapse including reduced left ventricular ejection fraction (LVEF, n = 4), third degree heart block (n = 2), atrio-ventricular (n = 1) or ventricular (n = 1) tachycardia and sudden cardiac death (n = 1). Except for a higher frequency of black patients in patients receiving IS, CS features at diagnosis and median time to relapse did not significantly differ between patients who did or did not receive IS. Relapse rate was 45.8% in the steroids group versus 16.7% in the steroids + IS group (p = 0.048).
In cardiac sarcoidosis, the combination of steroids with immunosuppressive drugs might reduce the risk of cardiac relapse, as compared to steroids alone.
•Heart involvement is the leading cause of death in sarcoidosis•Evidence-based data regarding cardiac sarcoidosis treatment are poor•Steroids and immunosuppressive drugs may be more effective than steroids alone in preventing cardiac sarcoidosis relapse.
Objective
To determine risk factors for primary Sjögren's syndrome (SS)–associated lymphoma in a multicenter cohort of patients, with analysis of the predictive power of previously reported risk ...factors, including the presence of ectopic germinal center (GC)–like structures in minor salivary gland (MSG) biopsy tissue.
Methods
One hundred fifteen patients with primary SS were included, and MSG biopsy tissue from these patients was retrospectively examined, focusing on the presence of ectopic GC‐like structures. Epidemiologic, clinical, biologic, immunologic, and histologic data were collected at the time of diagnosis of primary SS. Patients with non‐Hodgkin's lymphoma (NHL) were compared with those without NHL during the follow‐up period, using a Cox proportional hazards multiple regression model.
Results
NHL was diagnosed in 8 patients (6.96%), and ectopic GC‐like structures in 19 patients (16.5%). The presence of ectopic GC‐like structures was associated with a 7.8‐fold increased risk of lymphoma occurrence (95% confidence interval 95% CI 1.73–34.86 P = 0.0075). Other independent predictors included a positive cryoglobulin test result (hazard ratio HR 7.10, 95% CI 1.74–28.92 P = 0.006), male sex (HR 28.73, 95% CI 4.46–144.87 P = 0.0004), sensorimotor neuropathy (HR 35.48, 95% CI 5.79–217.39 P = 0.0001), and splenomegaly (HR 19.9, 95% CI 4.4–90 P = 0.0001).
Conclusion
The presence of ectopic GC‐like structures in MSG biopsy tissue is associated with the risk of lymphoma in patients with primary SS. These data reinforce the major role of MSG biopsy tissue in primary SS, for the identification a priori of a subgroup of patients with the highest risk of lymphoma.
The aim of this study was to determine the prevalence and clinical significance of extra-articular manifestations (EAMs) at inclusion into a cohort of patients with recent-onset arthritis consistent ...with rheumatoid arthritis (RA).
The ESPOIR cohort included patients aged 18 to 70 years who had a definitive or probable diagnosis of RA. Symptoms consistent with EAMs were collected at baseline. We divided the patients into two groups, with vs. without baseline EAMs. We looked for associations linking the presence of EAMs at baseline to patient and disease characteristics at baseline and 5 years later, as well as to diagnostic certainty after 2 years. The analyses were adjusted for multiple comparisons using the Benjamini–Hochberg procedure to control the false discovery rate.
Of 798 patients, 330 (41.4%) had at least one symptom consistent with EAM at baseline, with the most common being sicca syndrome (28.4%) and Raynaud's phenomenon (17.3%). The EAM+ group had a higher mean baseline DAS-28 value (5.3 ± 1.3 versus 5.0 ± 1.3; corrected p value = 0.005) compared to the EAM- group. The final diagnosis did not differ between the two groups. After 5 years, the EAM+ group had significantly higher values for the tender joint count (3.9 ± 6.4 versus 1.8 ± 3.3, corrected p value = 0.005) and swollen joint count (1.3 ± 2.8 versus 1.1 ± 2.3, corrected p value =0.0005) compared to the EAM- group.
EAMs, particularly sicca syndrome and Raynaud's phenomenon, are very common in patients with early arthritis consistent with RA. In this population, several parameters reflecting disease activity were higher among patients with EAMs, at baseline and after 5 years.
Gout is a common arthritis that occurs particularly in patients who frequently have associated comorbidities that limit the use of conventional therapies. The main mechanism of crystal-induced ...inflammation is interleukin-1 production by activation of the inflammasome. We aimed to evaluate the efficacy and tolerance of anakinra in gouty patients.
We conducted a multicenter retrospective review of patients receiving anakinra for gouty arthritis. We reviewed the response to treatment, adverse events and relapses.
We examined data for 40 gouty patients (32 men; mean age 60.0 ± 13.9 years) receiving anakinra. Mean disease duration was 8.7 ± 8.7 years. All patients showed contraindications to and/or failure of at least two conventional therapies. Most (36; 90%) demonstrated good response to anakinra. Median pain on a 100-mm visual analog scale was rapidly decreased (73.5 (70.0 to 80.0) to 25.0 (20.0 to 32.5) mm, P < 0.0001), as was median C-reactive protein (CRP) level (130.5 (55.8 to 238.8) to 16.0 (5.0 to 29.5) mg/l, P < 0.0001). After a median follow-up of 7.0 (2.0 to 13.0) months, relapse occurred in 13 patients after a median delay of 15.0 (10.0 to 70.0) days. Seven infectious events, mainly with long-term use of anakinra, were noted.
Anakinra may be efficient in gouty arthritis, is relatively well tolerated with short-term use, and could be a relevant option in managing gouty arthritis when conventional therapies are ineffective or contraindicated. Its long-term use could be limited by infectious complications.
To compare the ultrasonography (US) assessment of the retinacula of ankles in patients with rheumatoid arthritis (RA) and psoriatic arthritis (PsA).
This cross-sectional study included RA or PsA ...patients with ankle pain and healthy controls. The following US features were recorded: presence of synovitis, tenosynovitis and abnormalities of two retinacula (the superior peroneal retinaculum SPR and the flexor retinaculum FR evaluated in mode B and power Doppler).
Among the 80 included patients, 37 (46%) and 23 (29%) had RA and PsA; 20 (25%) patients were healthy controls. The FR was thicker in PsA than RA ankles 0.96±0.39 vs. 0.64±0.15, P<0.001 with no difference between RA patients and HCs. Other FR abnormalities such as hypoechogenicity, PD positivity or periostosis were more frequent in PsA than RA patients, P<0.001. On receiver-operating-characteristic curve analysis, a cut-off of 1mm FR thickness provided a sensitivity of 49% and specificity of 97% for the diagnosis of PsA. Overall, 39 and 3% of PsA and RA ankles exhibited retinaculitis of FR (thickness≥1mm with hypervascularization or hypoechogenicity). The two disease groups did not differ in the evaluation of SPR.
US abnormalities of FR were more frequent in PsA than RA and appeared to be specific for PsA. US assessment of FR might be useful to distinguish RA and PsA.
PDF
