Sarcoidosis, Vasculitis, and Diffuse Lung Diseases : Official Journal of WASOG

BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease with elevated mortality. Delay in diagnosis lead to worse outcomes. Guidelines developed at academic medical centers are difficult to replicate in the community. OBJECTIVES: Our primary objective was to ascertain consistency with the 2011 IPF guidelines. Our secondary objective was to conduct an interdisciplinary review to ascertain whether the evidence supported the original diagnosis of IPF or not...

Sarcoidosis is a multisystem inflammatory disease manifesting as noncaseating epithelioid cell granulomas. 25 to 30% of individuals with systemic sarcoidosis show variable cutaneous manifestations. A 59-year-old female was seen with reticular purplish-red nodules and plaques on the legs for three months. A skin biopsy of the livedo area revealed non-caseating epithelioid cell granulomas surrounding blood vessels in the dermis. She was diagnosed with sarcoidosis livedo, and cutaneous lesions subsided with oral prednisone...

INTRODUCTION:   activity tracker device usage can help analyze the impact of disease state and therapy on patients in clinical practice.  factors such as age, race, and gender may contribute to difficulties with using such technology.  Objective: we evaluated the effect of age, race, and gender on the usability of the Fitbit OneTM activity tracking device in sarcoidosis patients and the impact of device on sarcoidosis patients' activity. METHOD: patients participated in a six-month prospective study where were asked to wear a Fitbit OneTM activity tracker daily...

No abstract text is available yet for this article.

BACKGROUND AND AIM: Diffuse alveolar hemorrhage (DAH) is a life-threatening condition due to the extravasation of blood in the alveoli, resulting in hypoxemia and even acute respiratory distress syndrome. This study aimed to describe the clinico-radio-pathological profile of patients diagnosed with DAH and classify it into immune and nonimmune DAH. METHODS: This was a retrospective analytical study. Of a total of 1000 cases of bronchoalveolar lavage fluids (BALF) received for cytological examination, patients fulfilling the clinical, radiological, and laboratory details of cases satisfying the clinical and cytological criteria of DAH (n=47) were studied...

BACKGROUND AND AIM: Serum Soluble Interleukin-2 Receptor (sIL-2R) levels are used clinically as a disease activity marker for systemic sarcoidosis. Studies have investigated the diagnostic role of serum soluble interleukin-2 receptor (sIL-2R) level for sarcoidosis relative to biopsy. We performed a systematic review and meta-analysis of studies evaluating the diagnostic utility of sIL-2R. METHODS: We carried out an electronic search in Medline, Embase, Google Scholar, and Cochrane databases using keyword and Medical Subject Heading (MeSH) terms: sarcoidosis and sIL-2R...

Background and aim Prednisone is used as first-line therapy for patients with pulmonary sarcoidosis. There is however no clear association between prednisone dose and FVC change in patients with pulmonary sarcoidosis. In order to improve our standard of care we introduced a more conservative prednisone protocol. Methods This study is a single centre observational study, applying value-based healthcare (VBHC) and quality improvement (QI) principles. Prednisone intake was reduced from a starting dose of 40 mg to a starting dose of 20 mg...

OBJECTIVE AND AIM:  Sarcoidosis, a multisystemic granulomatous disease, generally results in a lower quality of life (QoL) because of its unexpected course and diverse clinical symptoms. The Sarcoidosis Health Questionnaire (SHQ) evaluates the QoL for people with sarcoidosis in terms of their health. This study set out to validate the SHQ in a group of Turkish sarcoidosis patients. METHODS:  The study included a total of 146 adult sarcoidosis patients (63 male and 83 female; mean age, 44±3...

BACKGROUND AND AIM: To outline the observations of studies evaluating the prominence of Neutrophil to Lymphocyte Ratio (NLR) in sarcoidosis. METHODS: The search was performed on PubMed, Scopus, and web of science up until November 21, 2021. Eventually, a number of 17 papers were incorporated into this review. RESULTS: The results of this analysis showed no significant difference of NLR values between sarcoidosis patients and tuberculosis patients (SMD=-0...

Efzofitimod is a first-in-class biologic based on a naturally occurring splice variant of histidyl-tRNA synthetase (HARS) that downregulates immune responses via selective modulation of neuropilin-2 (NRP2). Preclinical data found high expression of NRP2 in sarcoidosis granulomas. Treatment with efzofitimod reduced the granulomatous inflammation induced by P. acnes in an animal model of sarcoidosis. A dose escalating trial of efzofitimod in sarcoidosis with chronic symptomatic pulmonary disease found that treatment with efzofitimod was associated with improved quality of life with a trend towards reduced glucocorticoid use and stable to improved pulmonary function...

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...

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...

Background   Combined pulmonary fibrosis and emphysema (CPFE) has been recognised as a phenotype of pulmonary fibrosis. We aimed to compare serum surfactant protein-A (SP-A), surfactant protein-D (SP-D) and Krebs von den Lungen-6 (KL-6) levels, functional parameters, in CPFE and  IPF (idiopathic pulmonary fibrosis) patients. Methods Patients diagnosed with 'CPFE' and 'IPF' were consecutively included in 6 months as two groups. The patients with connective tissue diseases are excluded. Results           In this study, 47 patients (41 males, 6 females) with CPFE (n = 21) and IPF (n = 26) with a mean age of 70...

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...

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...

No abstract text is available yet for this article.

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...

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...

No abstract text is available yet for this article.

Sarcoidosis is a multisystem disease of unknown origin. Diagnosis remains challenging, based on organ site involvement, histological confirmation of non-caseating granuloma and an appropriate clinical syndrome. Granulomatous bone involvement is rare and may be ignored because it is usually asymptomatic. Vertebrae, ribs and skull localizations are rarely reported. We described an interesting case of a woman with chronic and multiorgan sarcoidosis with unusual bone localizations.