Sarcoidosis, Vasculitis, and Diffuse Lung Diseases最新文献

Background and aim: Small fiber neuropathy (SFN) may present as complication in sarcoidosis.(1) SFN can potentially result into a large range of symptoms with a high impact on quality of life.(2) Although treatment of the underlying disease of SFN is paramount, little research has been performed to investigate SFN improvement as consequence of sarcoidosis treatment. This retrospective study investigates whether there is an association between the anti-inflammatory effects of infliximab and SFN-symptoms Methods: The Small Fiber Neuropathy Screening List (SFNSL) was used to measure changes in SFN symptoms during infliximab treatment. Maximal standardized uptake value (SUVmax) from Fluordeoxyglucose Positron Emission Tomography (FDG-PET) was used as a measure for inflammatory activity.

Results: 36 sarcoidosis patients were eligible for analysis. SFNSL-score showed a mean decrease of -1,9 points (p = 0.446). SUVmax did improve with a mean of -3.7 (p<0.001). No correlation between a decrease of SUVmax and SFNSL screening list could be found (p=0.610).

Conclusions: Our data reveal no association between anti-inflammatory effect of infliximab and SFN-related symptoms in patients with sarcoidosis, which contradicts previous case-reports and case-series.(3-6) Given the major negative impact of SFN-related symptoms on the quality of life in patients with sarcoidosis, it is necessary that the possible beneficial effect of anti-inflammatory therapy will be further addressed in future prospective studies.1.

Background and objectives: The present study aimed to find quantitative and semiquantitative methods to detect the development of fibroproliferative processes at an early stage and predict the severity and prognosis of the disease in interstitial lung diseases (ILDs) using High-Resolution Computed Tomography (HRCT), Pulmonary Function Tests (PFTs) and Complete Blood Count (CBC) parameters.

Materials and methods: A total of 63 patients (26 female and 37 male) who were admitted to our hospital between January 2014 and January 2018, whose follow-ups were regular and who underwent HRCT, PFT, and CBC examinations on the same day, were included in our study. The median age of the patients included was 65 years (range: 47-79).

Results: There were significant differences among the mild, moderate, and severe form ILD groups created using the Warrick scoring system for NLR, neutrophil count, and PNR values (p = 0.025, 0.035, 0.006, respectively). Also, there were significant differences among the groups for FVC, FEV1/FVC, PAD, RAA, RV/LV ratio, MLnMD, and MLnC values. Correlation analyses between the parameters revealed significant relationships between Warrick Score, and NLR and neutrophil count, PNR, FVC, FEV1/FVC, PAD, RAA, RV/LV ratio, MLnMD, and MLnC .

Conclusions: The results of the present study suggested that NLR, neutrophil count, and PNR values could be used as objective evaluation criteria to determine the severity and prognosis in interstitial lung diseases. Also, usage of Warrick Score, FVC, FEV1/FVC, PAD, RAA, RV/LV ratio, MLnMD, and MLnC values could provide quantitative and semiquantitative data for an objective evaluation. Carrying out multicenter studies and creating a scoring system using these parameters could create standardization in determining the prognosis of patients with ILD.

Objective: To review the medical literature regarding chylothorax associated with sarcoidosis.

Methods: A literature review of all reported cases of sarcoidosis-associated chylothorax, we included a novel case report to the analysis.

Results: Of sixteen cases included in the study, 10 were women (62.5%), mean age 47±17years. In 6 subjects (37.5%) chylothorax was part of the initial presentation of sarcoidosis. Four subjects (25%) additionally suffered from lymphedema and chylous ascites, and one from chylous ascites only. Thoracic lymphadenopathy was reported for 13/16 subjects (81.3%) and lung parenchymal disease in 8/16 (50%). Compression of the thoracic duct was considered as a causative factor in 10 cases (62.5%). One case was attributed to granulomatous pleural inflammation, one to generalized lymphangiectasia, and no specific causative factors were identified in 4 remaining cases (25%). Overall mortality rate was 18.8% (3/16 subjects). Of note, all the subjects treated with corticosteroids survived.

Conclusions: Since the association of sarcoidosis with chylothorax is exceedingly rare, alternative etiologies should be pursued even when chylothorax develops in a subject with preexisting sarcoidosis. However, the possibility of sarcoidosis should be entertained when other etiologies for a newly diagnosed chylothorax are ruled out. A multidisciplinary approach is required for optimal management, both for elucidating the diagnosis and for employing therapy, which could be multimodal. A trial of immunosuppressive therapy with corticosteroids should be considered.

Background and aim: Acute exacerbation (AE) of idiopathic pulmonary fibrosis (IPF) is a fatal condition with no established treatment. Intravenous immunoglobulin (IVIG) is a unique therapy with both anti-inflammatory and anti-infective effects. Therefore, we hypothesized that IVIG may have a positive effect on AE of interstitial pneumonia. This study aimed to determine the effect of IVIG in patients with AE of fibrotic idiopathic interstitial pneumonias (IIPs), including IPF.

Methods: We retrospectively analyzed consecutive patients who were diagnosed with AE of fibrotic IIPs and treated with pulse corticosteroid therapy (methylprednisolone 500-1000 mg/day for 3 days) between April 2018 and May 2021 at Kagawa Rosai Hospital and KKR Takamatsu Hospital.

Results: This study included 52 patients with AE of fibrotic IIPs (IPF,41; fibrotic IIPs other than IPF,11). Thirteen patients received IVIG (5 g/day for 3-5 days) concurrently with pulse corticosteroid therapy. The remaining 39 patients were assigned to the control group. The survival rate on day 90 was significantly higher in the IVIG group than that in the control group (76.9% vs. 38.5%, p = 0.02). IVIG administration (odds ratio [OR], 0.11; 95% confidence interval [CI], 0.02-0.69; p = 0.02) and C- reactive protein (OR, 1.19; 95% CI, 1.06-1.33, p < 0.01) were independently associated with 90-day mortality.

Conclusions: The results indicate that administration of IVIG may improve the survival of patients with AE of fibrotic IIPs. We are now conducting a prospective study to confirm the effect of IVIG on AE of IPF since May 2022 (jRCT1061220010).

Sarcoidosis is a systemic granulomatous disease, sometimes characterized by an extrapulmonary localization in 30 - 50% of cases. We describe a 60-year-old Italian man with an unexplained history of fatigue, ascitis and progressive renal function impairment. Diagnosis of hepatic and bone marrow sarcoidosis was established by histology, and fast improvement of renal function was obtained after starting  corticosteroid therapy. Atypical presentation and simultaneous involvement of liver, bone marrow and kidneys make diagnosis of extrapulmonary sarcoidosis still a diagnostic challenge. Delayed diagnosis could lead to serious organ damage like a progressive severe kidney failure.

Background:   Sarcoidosis is a multisystem granulomatous inflammatory disease of unclear etiology that involves the lung, skin and other organs, with an unknown antigenic trigger. Recently, evidence has been found in both immune deficient and immune competent patients for rubella virus in cutaneous granulomas. These granulomatous lesions share overlapping features with cutaneous sarcoidosis, raising the question of rubella virus in sarcoidosis.

Objective: To investigate the presence of rubella virus in sarcoidosis lung samples.

Methods: We employed metagenomic sequencing to interrogate extracellular virome preparations and cellular transcriptomes from bronchoalveolar lavage (BAL) of 209 sarcoidosis patients for rubella virus sequences.

Results: We found no evidence for rubella virus genomes in acellular fluid or rubella virus gene expression in BAL cells of sarcoidosis patients.

Conclusions: These findings argue against rubella virus infection or persistence within the lung at time of sampling as a sarcoidosis trigger.