The role of chest X-ray in the early diagnosis and staging of sarcoidosis: Is it really should be done?

BACKGROUND: Sarcoidosis is a chronic granulomatous disease characterized by non-caseating granuloma. The conventional chest X-ray (CXR) has important role in the diagnosis, staging and follow-up of disease. Computed tomography (CT) is a second-line imaging method used to determine the extent, complications and differential diagnosis of sarcoidosis. OBJECTIVES: To determine the role of CXR in the early diagnosis and staging of sarcoidosis and to compare with CT imaging. METHODS: One hundred and nine sarcoidosis patients followed at a single center were included in the study. Demographic, radiological, and clinical data of 81 patients were obtained from a total of 109 patients, and the record data of these 81 patients were evaluated. Patients who could not be reached for all tests were excluded from the study. CXR and CT imaging taken at diagnosis were evaluated retrospectively independently from two radiologists and one rheumatologist. RESULTS: Among 109 patients, eighty-one patients CXR and CT imaging taken at the same center has been reached. Among 81 sarcoidosis patients 23 (28.4%) were male, 58 (71.6%) were female. The mean patients age was 46.4 years and the mean disease duration was 3.8 years. CXR is regarded as normal at diagnosis in 30 patients (37%), while all of these patients had findings consistent with sarcoidosis on CT imaging. CT imaging are more superior than CXR in the early diagnosis and staging of sarcoidosis (p=0.001). Also CT imaging is more superior for detection of disease extent and complications. CONCLUSIONS: In this study, we observed that CT imaging outperforms CXR in terms of early detection and staging of sarcoidosis. The use of CT imaging is important for early diagnosis and staging of sarcoidosis. The low performance of CXR is a condition that requires the discussion of this method. Multicenter prospective study is needed in this regard.

The role of chest X-ray in the early diagnosis and staging of sarcoidosis: Is it really should be done? / El papel de la radiografía de tórax en el diagnóstico precoz y estadificación de la sarcoidosis: ¿realmente se debe hacer?

Background: Sarcoidosis is a chronic granulomatous disease characterized by non-caseating granuloma. The conventional chest X-ray (CXR) has important role in the diagnosis, staging and follow-up of disease. Computed tomography (CT) is a second-line imaging method used to determine the extent, complications and differential diagnosis of sarcoidosis. Objectives: To determine the role of CXR in the early diagnosis and staging of sarcoidosis and to compare with CT imaging. Methods: One hundred and nine sarcoidosis patients followed at a single center were included in the study. Demographic, radiological, and clinical data of 81 patients were obtained from a total of 109 patients, and the record data of these 81 patients were evaluated. Patients who could not be reached for all tests were excluded from the study. CXR and CT imaging taken at diagnosis were evaluated retrospectively independently from two radiologists and one rheumatologist. Results: Among 109 patients, eighty-one patients CXR and CT imaging taken at the same center has been reached. Among 81 sarcoidosis patients 23 (28.4%) were male, 58 (71.6%) were female. The mean patients age was 46.4 years and the mean disease duration was 3.8 years. CXR is regarded as normal at diagnosis in 30 patients (37%), while all of these patients had findings consistent with sarcoidosis on CT imaging. CT imaging are more superior than CXR in the early diagnosis and staging of sarcoidosis (p=0.001). Also CT imaging is more superior for detection of disease extent and complications. Conclusions: In this study, we observed that CT imaging outperforms CXR in terms of early detection and staging of sarcoidosis. The use of CT imaging is important for early diagnosis and staging of sarcoidosis. The low performance of CXR is a condition that requires the discussion of this method. Multicenter prospective study is needed in this regard.(AU)

Antecedentes: La sarcoidosis es una enfermedad granulomatosa crónica caracterizada por un granuloma no caseificante. La radiografía de tórax convencional (CXR) tiene un papel importante en el diagnóstico, estadificación y seguimiento de la enfermedad. La tomografía computarizada (TC) es un método de imagen de segunda línea que se utiliza para determinar la extensión, las complicaciones y el diagnóstico diferencial de la sarcoidosis. Objetivos: Determinar el papel de la radiografía de tórax en el diagnóstico temprano y la estadificación de la sarcoidosis y compararlo con la tomografía computarizada. Métodos: Se incluyeron en el estudio 109 pacientes con sarcoidosis seguidos en un solo centro. Se obtuvieron datos demográficos, radiológicos y clínicos de 81 sujetos de un total de 109 pacientes, y se evaluaron los datos de registro de estos 81 individuos. Los pacientes que no pudieron ser contactados para todas las pruebas fueron excluidos del estudio. Las imágenes de CXR y CT tomadas en el momento del diagnóstico fueron evaluadas retrospectivamente de forma independiente por 2 radiólogos y un reumatólogo. Resultados: De un total de 109 pacientes se han obtenido imágenes de CXR y CT, tomadas en el mismo centro, de 81 individuos. De esos 81 pacientes con sarcoidosis 23 (28,4%) eran hombres y 58 (71,6%) eran mujeres. La edad media de los pacientes fue de 46,4 años y la duración media de la enfermedad fue de 3,8 años. La CXR se considera normal en el momento del diagnóstico en 30 pacientes (37%), mientras que todos estos pacientes tenían hallazgos consistentes con sarcoidosis en la TC. La TC es superior a la radiografía de tórax en el diagnóstico temprano y la estadificación de la sarcoidosis (p=0,001) y en la detección de la extensión de la enfermedad y las complicaciones. Conclusiones: En este estudio observamos que la TC supera a la radiografía de tórax en términos de detección temprana y estadificación de la sarcoidosis...(AU)

SARS-CoV-2 Infection-Induced Necrotising Sarcoid Granulomatosis.

SARS-CoV-2 infection is a pandemic that affects predominantly upper airways and lungs. It may lead to reactivation of known inflammatory rheumatic diseases and/or initiation of various granulomatous disorders. Necrotising sarcoid granulomatosis (NSG) is a rare condition that can be confused with malignancy, granulomatosis with polyangiitis, and sarcoidosis. Herein we reported the development of NSG following a SARS-CoV-2 infection which mimicked granulomatosis with polyangiitis.

Increased circulating interleukin-23 level in patients with sarcoidosis.

BACKGROUND: Sarcoidosis is a Th1-mediated chronic inflammatory disease characterized by non-caseating granulomas. Its pathogenesis is not yet clear, but the possible role of various proinflammatory cytokines is being discussed. AIM: This study aims to determine serum cytokine (IL-6, IL-12, IL-17, and IL-23) levels in patients with sarcoidosis, and to determine a possible correlation with clinical and laboratory findings of the disease. MATERIAL AND METHOD: Forty-four biopsy-proven sarcoidosis patients followed up at a single centre and 41 healthy volunteers were included in the study. Demographic, clinical, laboratory, and radiological data of all patients were recorded. Serum samples from the patients and the control group were taken and IL-6, IL-12, IL-17, IL-23 were measured by ELISA method. RESULTS: Of the 44 sarcoidosis patients, 13(29.5%) were male and 31(70.5%) were female. Average patient age was 47.4 years, mean disease duration was 3.2 years. Twenty-one (47.7%) patients had erythema nodosum, three (6.8%) had uveitis, 40(90.9%) had arthralgia, 23(52.3%) had ankle arthritis, 15(34.1%) had enthesitis. Laboratory evaluation showed increased serum ACE levels in 24(54.5%) patients, increased serum calcium levels in 11 (25%) patients, increased serum D3 levels in 5(11.4%) patients, increased ESR and CRP levels in 22(50%) and 23(52.3%) patients, respectively. Compared with the control group higher serum IL-23 levels were found in the patients with sarcoidosis (p=.01). Serum IL-23 was associated with ankle arthritis (p=.02). Serum IL-6, IL-12, and IL-17 levels were similar in the sarcoidosis patients and the control group (p=.128, p=.212, p=.521 respectively). CONCLUSION: In our study, we found increased serum IL-23 in patients with sarcoidosis, while serum IL-6, IL-12, and IL-17 were detected as normal. Although our results are somewhat contradictory to other studies in the literature, the question should still be whether sarcoidosis is a Th1/Th17 disease. Multicentre studies are needed in this regard.

Increased circulating interleukin-23 level in patients with sarcoidosis / Incremento del nivel circulante de interleucina 23 en los pacientes con sarcoidosis

Background: Sarcoidosis is a Th1-mediated chronic inflammatory disease characterized by non-caseating granulomas. Its pathogenesis is not yet clear, but the possible role of various proinflammatory cytokines is being discussed. Aim: This study aims to determine serum cytokine (IL-6, IL-12, IL-17, and IL-23) levels in patients with sarcoidosis, and to determine a possible correlation with clinical and laboratory findings of the disease. Material and method: Forty-four biopsy-proven sarcoidosis patients followed up at a single centre and 41 healthy volunteers were included in the study. Demographic, clinical, laboratory, and radiological data of all patients were recorded. Serum samples from the patients and the control group were taken and IL-6, IL-12, IL-17, IL-23 were measured by ELISA method. Results: Of the 44 sarcoidosis patients, 13(29.5%) were male and 31(70.5%) were female. Average patient age was 47.4 years, mean disease duration was 3.2 years. Twenty-one (47.7%) patients had erythema nodosum, three (6.8%) had uveitis, 40(90.9%) had arthralgia, 23(52.3%) had ankle arthritis, 15(34.1%) had enthesitis. Laboratory evaluation showed increased serum ACE levels in 24(54.5%) patients, increased serum calcium levels in 11 (25%) patients, increased serum D3 levels in 5(11.4%) patients, increased ESR and CRP levels in 22(50%) and 23(52.3%) patients, respectively. Compared with the control group higher serum IL-23 levels were found in the patients with sarcoidosis (p=.01). Serum IL-23 was associated with ankle arthritis (p=.02). Serum IL-6, IL-12, and IL-17 levels were similar in the sarcoidosis patients and the control group (p=.128, p=.212, p=.521 respectively). Conclusion: In our study, we found increased serum IL-23 in patients with sarcoidosis, while serum IL-6, IL-12, and IL-17 were detected as normal.(AU)

Antecedentes: La sarcoidosis es una enfermedad inflamatoria crónica mediada por Th1, caracterizada por granulomas no caseificantes. Su patogenia no está clara todavía, aunque se está debatiendo el posible rol de las diversas citocinas proinflamatorias. Objetivo: El objetivo de este estudio es determinar los niveles de citocinas séricas (IL-6, IL-12, IL-17 e IL-23) en los pacientes con sarcoidosis, así como establecer una posible correlación con los hallazgos clínicos y de laboratorio de la enfermedad. Material y método: Se incluyó en el estudio a 44 pacientes con sarcoidosis verificada mediante biopsia, cuyo seguimiento se realizó en un único centro, y 41 voluntarios sanos. Se registraron los datos demográficos, clínicos, de laboratorio y radiológicos de todos los pacientes. Se tomaron muestras séricas de los pacientes y el grupo control, midiéndose los niveles de IL-6, IL-12, IL-17 e IL-23 mediante el método ELISA. Resultados: De los 44 pacientes con sarcoidosis, 13 (29,5%) fueron varones y 31 (70,5%) fueron mujeres. La edad media de los pacientes fue de 47,4 años, y la duración media de la enfermedad fue de 3,2 años. Veintiún (47,7%) pacientes tenían eritema nudoso, 3 (6,8%) tenían uveítis, 40 (90,9%) tenían artralgia, 23 (52,3%) tenían artritis de tobillo y 15 (34,1%) tenían entesitis. La evaluación de las pruebas de laboratorio reflejó un incremento de los niveles séricos de ECA en 24 (54,5%) pacientes, de los niveles séricos de calcio en 11 (25%) pacientes, de los niveles séricos de D3 en 5 (11,4%) pacientes y de los niveles de ESR y PCR en 22 (50%) y 23 (52,3%) pacientes, respectivamente. En comparación con el grupo control, se encontraron niveles séricos de IL-23 más elevados en los pacientes con sarcoidosis (p=0,01). Los niveles séricos de IL-23 estuvieron asociados a artritis de tobillo (p=0,02)...(AU)

VEXAS syndrome: Current clinical, diagnostic and treatment approaches.

VEXAS syndrome, is a hemato-inflammatory chronic disease characterized with predominantly rheumatic and hematologic systemic involvement. It was first described in 2020 by a group of researchers in the United States. VEXAS syndrome is a rare condition that primarily affects adult males and is caused by a mutation in the UBA1 gene located on the X chromosome. Its pathogenesis is related to the somatic mutation affecting methionine-41 (p.Met41) in UBA1, the major E1 enzyme that initiates ubiquitylation. Mutant gene lead to decreased ubiquitination and activated innate immune pathways and systemic inflammation occur. The specific mechanism by which the UBA1 mutation leads to the clinical features of VEXAS syndrome is not yet fully understood. VEXAS is a newly define adult-onset inflammatory syndrome manifested with treatment-refractory fevers, arthritis, chondritis, vasculitis, cytopenias, typical vacuoles in hematopetic precursor cells, neutrophilic cutaneous and pulmonary inflammation. Diagnosing VEXAS syndrome can be challenging due to its rarity and the overlap of symptoms with other inflammatory conditions. Genetic testing to identify the UBA1 gene mutation is essential for definitive diagnosis. Currently, there is no known cure for VEXAS syndrome, and treatment mainly focuses on managing the symptoms. This may involve the use of anti-inflammatory medications, immunosuppressive drugs, and supportive therapies tailored to the individual patient's needs. Due to the recent discovery of VEXAS syndrome, ongoing research is being conducted to better understand its pathogenesis, clinical features, and potential treatment options. In this review article, the clinical, diagnostic and treatment approaches of VEXAS syndrome were evaluated in the light of the latest literature data.

Atezolizumab Lung Toxicity and Importance of Combination Treatment: A Case Report.

BACKGROUND: Lung cancer is one of the most common and mortal cancers worldwide. According to pathological and clinical groups, treatments vary, and a tailored approach is considered. Adjuvant therapies, such as chemotherapy, radiation, and immune checkpoint inhibitors (ICI), are recommended by recent guidelines for patients with locally advanced cancer. OBJECTIVE: This study aimed to report the case of a patient with stage 2B squamous cell lung carcinoma who was managed for pulmonary toxicity after receiving adjuvant chemotherapy and atezolizumab treatment. CASE REPORT: A 66-year-old male patient received chemotherapy and immunotherapy after surgery for squamous cell lung cancer. A diagnosis of atezolizumab-associated pneumonitis was made using laboratory tests and imaging due to the patient's worsening dyspnea after treatment. Due to the patient's rapid progression, pulse steroid and MMF therapy were administered concurrently. When Klebsiella pneumoniae growth was detected in the sputum culture during the follow-up, IVIg was used to supplement the medication. The patient showed significant clinical and radiological improvement. CONCLUSION: In this study, we present an atezolizumab-induced pneumonitis case of a squamous cell lung cancer patient. It may be life-saving not to avoid aggressive treatment approaches by combining the steps of guideline recommendations in patients with rapidly progressive pneumonitis.

Stairway to Heaven: Do head-to-head Trials Indicate a Need for Definite Criteria for Choosing Biologic Drugs in Rheumatoid Arthritis?

Rheumatoid arthritis (RA) is a chronic disease characterized by joint and systemic involvement that develops with different pathogenetic mechanisms. Treatment of the disease is undertaken with disease-modifying anti-rheumatic drugs (DMARDs). The mechanisms of action of conventional DMARDs generally are based on the inhibition of T and B-cells in the immune system. In recent years, biologic and targeted smart molecules have been used in the treatment of RA. Targeting different cytokines and inflammatory pathways, these drugs have ushered in a new era in RA treatment. The efficacy of these drugs has been demonstrated in many studies; and in the postmarketing period, that is, as the patients who use them say, they are like a "stairway to heaven". However, as every "road to heaven" is challenging and "thorny", the efficacy and reliability of these drugs and whether any one of them is superior to the others, remains a matter of debate. However, the use of biologic drugs with or without cDMARDs, the preference for original vs. biosimilar molecules, and discontinuation of the drugs after achieving sustained remission are other questions that need to be explored. When it comes to the choice of biological drugs by rheumatologists, it is not yet clear on which criteria they base their choices on. Due to the limited comparative studies of these biological drugs, the subjective criteria of the physician gains importance. The selection of these drugs, however, should be based on objective criteria such as efficacy, safety, superiority over each other, and cost. In other words, the determinant of the "path to heaven" should be based on objective criteria and recommendations according to the scientific data generated by controlledprospective studies, not on the initiative of a single physician. In this review, a head-to-head comparison of biological drugs used in the treatment of RA, their efficacy, safety, and which are superior are discussed in light of recent literature data.

Treat to target and tight control: Could be a new approach in the treatment of sarcoidosis?

Sarcoidosis is a chronic granulomatous disease with multisystemic involvement. Although it is accepted as a benign disease, it can sometimes cause life-threatening organ (heart, brain) involvement that determines the prognosis of the disease. There are conflicting opinions about the treatment of the disease. In the generally accepted treatment approach the "step-by-step" model has gained weight. According to this approach, corticosteroids (CS) drugs alone are preferred in the first step in patients who require treatment. In the second step, immunosuppressive drugs (IS) are used in patients who do not respond to CS and/or have contraindications to CS use, and biologics (TNF-alpha inhibitors) are used in the third step. This treatment approach may be valid in cases with mild sarcoidosis. However, although sarcoidosis is considered a benign and self-limiting disease in some major organ involvement, the "step-by-step" approach may be a treatment option that puts the patient's life in danger. In such selected patients, much more rigorous, early and combined treatment approaches that definitely include CS, IS or biologic drugs may be required. In selected sarcoidosis patients with high risk, early diagnosis, "treat-to-target" (T2T) and "tight control" follow-up of patients seems to be a rational approach. This article reviews the "step-down" treatment regimens in light of recent literature data and hypothesizes that the T2T model may be a probable new treatment approach in patients with sarcoidosis.

Serum salusin-α and salusin-ß levels in patients with psoriatic arthritis.

Introduction: Psoriatic arthritis (PsA) is a chronic inflammatory disease characterized by skin lesions and joint involvement. Salusin-α and salusin-ß are two new bioactive molecules. It is reported that salusins may have role in regulation of the immune system and inflammation. The aim of our study was to evaluate the serum salusin-α and salusin-ß levels in PsA patients and to establish the possible relationship with the disease features. Material and methods: Our study included 40 PsA patients who fulfilled the CASPAR criteria and 40 healthy volunteers. Demographic, clinical, laboratory and radiological data and disease activity indices (PASI, BASDAI, BASFI, HAQ) were recorded in all patients. The enzyme-linked immunosorbent assay (ELISA) method was used to measure serum salusin-α and salusin-ß levels. Results: The demographic data were as follows: 13 patients (32.5%) were males and 27 (67.5%) were female, mean age was 48.5 years and mean disease duration was 2.4 years. Patients' history was taken and clinical assessment was performed; 20 (50%) patients had a family history, 18 (45%) patients were smoker, 19 (47.5%) patients had HLA-B27 positivity, 33 (82.5%) had sacroiliitis, 36 (90%) had enthesitis, 23 (57.5%) had distal interphalangeal (DIP) joint and nail involvement, 26 (65%) had wrist involvement, and 11 (27.5%) had ankle involvement. Laboratory data of the patients were recorded; 20 (50%) patients had elevated CRP level and 25 (62.5%) patients had an elevated ESR level. The study results showed that PsA patients had an elevated serum salusin-α level when compared with the control group (p = 0.004). The association between serum salusin-α level and ankle arthritis was found (p = 0.04). Serum levels of salusin-ß were similar in PsA patients and controls both (p = 0.285). Conclusions: We found elevated serum salusin-α in PsA patients while the serum salusin-ß levels were normal. Salusin-α may have a possible role in disease pathogenesis and it may be use as a reliable biomarker in PsA patients. Multicenter prospective studies are needed in this regard.

Budd-Chiari syndrome in Behçet's disease: a retrospective multicenter study.

OBJECTIVE: To compare the clinical features, laboratory findings, and prognosis of Behçet's disease (BD) patients with and without Budd-Chiari syndrome (BCS). METHODS: This multicenter retrospective study investigated 61 (M/F: 41/20) patients with BD, having coexistent BCS, and 169 (M/F:100/69) BD patients as the control group without BCS from 22 different centers of Turkey diagnosed between 1990 and 2017. RESULTS: Of the total 61 BD patients with BCS, the onset of the first symptom and the median age of diagnosis were earlier in contrast to BD patients without BCS (p = 0.005 and p = 0.007). Lower extremity deep vein and inferior vena cava (IVC) thrombosis were more common in patients with BCS (all; p < 0.01) compared to the control group. Mortality was significantly higher in BD-BCS patients with IVC thrombosis than in the controls (p = 0.004). Since most of the cases in our cohort had chronic and silent form of BCS, mortality rate was 14.8%, which was on the lower range of mortality rate reported in literature (14-47%). While all BD-BCS patients received immunosuppressive (IS) agents, only half of them received additional anticoagulant treatments. Among IS agents, interferon treatment was more frequently used in this cohort (19%), compared to other series reported in literature (2.3%). CONCLUSION: To our knowledge, this is the largest series of BD patients with BCS. Our patients had earlier disease onset and diagnosis, higher frequency of IVC thrombosis, and higher mortality rate, compared to BD patients without BCS. Mortality was significantly higher in BD-BCS patients with IVC thrombosis compared to controls. Key Points • Mortality rate is higher in BD-associated BCS patients with IVC involvement. • Chronic and silent form of BD-associated BCS has a better prognosis. • The main treatment options are corticosteroids and immunosuppressive agents, whereas anticoagulant treatment remains controversial.

Serum galectin-3 and TGF-beta levels in patients with sarcoidosis / Niveles séricos de galectina-3 y TGF-beta en pacientes con sarcoidosis

Introduction: Sarcoidosis is a chronic granulomatous disease that develops with non-caseified granuloma formation. Galectin-3 is a multifunctional protein operating in biological processes such as fibrosis, angiogenesis, and immune activation. Purpose: This study evaluates the levels of serum galectin-3 and TGF-beta in sarcoidosis patients to determine a possible correlation with clinical findings. Material and method: Forty-four biopsy-proven sarcoidosis patients followed in a single centre and 41 age and sex-matched healthy volunteers were included in the study. The levels of serum galectin-3 and TGF-beta were evaluated by ELISA method. Results: Among the 44 sarcoidosis patients, 13(29.5%) were male and 31(70.5%) were female. The average patient age was 47.4 and the average disease duration was 3.2 years. The level of serum galectin-3 was found to be the same as in the control group and had no significance statistically (p=.977). No correlation was determined between the level of serum galectin-3 and clinical and laboratory findings of sarcoidosis (p>.05). The level of serum TGF-beta was found to be higher in the sarcoidosis patients when compared to that of the control group (p=.005). While a correlation was found between serum TGF-beta and enthesitis, sacroiliitis, and arthralgia (p=.006, p=.034, p=.02), no correlation was determined on the other clinical and laboratory findings (p>.05). Conclusion: While the level of serum galectin-3 was determined to be normal in sarcoidosis patients, a high level of serum TGF-beta was found. These findings show that TGF-beta may play an important role in sarcoidosis pathogenesis and the formation of granuloma.(AU)

Introducción: : La sarcoidosis es una enfermedad granulomatosa crónica que se desarrolla con una formación de granuloma no caseificado. Galectina-3 es una proteína multifuncional que opera en procesos biológicos como la fibrosis, la angiogénesis y la activación inmune. Propósito: Este estudio evalúa los niveles séricos de galectina-3 y TGF-beta en pacientes con sarcoidosis para determinar una posible correlación con los hallazgos clínicos. Material y método: Fueron seguidos en un solo centro 44 pacientes con sarcoidosis probados por biopsia y se incluyeron en el estudio 41 voluntarios sanos de la misma edad y sexo. Los niveles séricos de galectina-3 y TGF-beta fueron evaluados por el método ELISA. Resultados: Entre los 44 pacientes con sarcoidosis, 13 (29,5%) eran hombres y 31 (70,5%) eran mujeres. La edad promedio de los pacientes fue de 47,4 y la duración promedio de la enfermedad fue de 3,2 años. Se encontró que el nivel de galectina-3 en suero era el mismo que en el grupo control y no tenía significancia estadística (p = 0,977). No se determinó correlación entre el nivel sérico de galectina-3 y los hallazgos clínicos y de laboratorio de sarcoidosis (p > 0,05). El nivel de TGF-beta en suero se encontró más alto en los pacientes con sarcoidosis, en comparación con el del grupo control (p = 0,005). Si bien se encontró una correlación entre el TGF-beta sérico y la entesitis, sacroileítis y artralgia (p = 0,006, p = 0,034, p = 0,02), no se determinó correlación en los otros hallazgos clínicos y de laboratorio (p > 0,05). Conclusión: Si bien se determinó que el nivel sérico de galectina-3 era normal en pacientes con sarcoidosis, se encontró un alto nivel de TGF-beta en suero. Estos hallazgos muestran que el TGF-beta puede desempeñar un papel importante en la patogénesis de la sarcoidosis y la formación de granuloma.(AU)

Serum galectin-3 and TGF-beta levels in patients with sarcoidosis.

INTRODUCTION: Sarcoidosis is a chronic granulomatous disease that develops with non-caseified granuloma formation. Galectin-3 is a multifunctional protein operating in biological processes such as fibrosis, angiogenesis, and immune activation. PURPOSE: This study evaluates the levels of serum galectin-3 and TGF-beta in sarcoidosis patients to determine a possible correlation with clinical findings. MATERIAL AND METHOD: Forty-four biopsy-proven sarcoidosis patients followed in a single centre and 41 age and sex-matched healthy volunteers were included in the study. The levels of serum galectin-3 and TGF-beta were evaluated by ELISA method. RESULTS: Among the 44 sarcoidosis patients, 13(29.5%) were male and 31(70.5%) were female. The average patient age was 47.4 and the average disease duration was 3.2 years. The level of serum galectin-3 was found to be the same as in the control group and had no significance statistically (p=.977). No correlation was determined between the level of serum galectin-3 and clinical and laboratory findings of sarcoidosis (p>.05). The level of serum TGF-beta was found to be higher in the sarcoidosis patients when compared to that of the control group (p=.005). While a correlation was found between serum TGF-beta and enthesitis, sacroiliitis, and arthralgia (p=.006, p=.034, p=.02), no correlation was determined on the other clinical and laboratory findings (p>.05). CONCLUSION: While the level of serum galectin-3 was determined to be normal in sarcoidosis patients, a high level of serum TGF-beta was found. These findings show that TGF-beta may play an important role in sarcoidosis pathogenesis and the formation of granuloma.

Ustekinumab-induced chronic lymphocytic leukemia in a patient with psoriatic arthritis.

Psoriatic arthritis (PsA) is a chronic inflammatory disease characterized by skin and joint involvement. The disease may present with various joint pattern involvement, which sometimes may lead to joint destruction and deformity. Early diagnosis and treatment with disease-modifying anti-rheumatic drugs may prevent joint deformity. Recently there are many new treatment options including biologic drugs. Ustekinumab, an interleukin 12/23 inhibitor, has proven efficacy in the treatment of psoriatic arthritis. Like other biologic drugs (anti-TNF-α), there are contradictory data about the safety of ustekinumab and possible relationship with cancer development. Herein we report the development of chronic lymphocytic leukemia in a patient with PsA treated with ustekinumab.

Malignancy in patients with sarcoidosis / Malignidad en pacientes con sarcoidosis

The relationship between sarcoidosis and malignancy is not clear yet. We retrospectively evaluated 131 sarcoidosis patients followed-up at the single Rheumatology center. The incidence of malignancies was investigated in this cohort. A total of 6 (4.6%) patients with malignancy were identified in our cohort of 131 patients with sarcoidosis. Hodgkin lymphoma (HL) was detected in three patients, followed by one patient with breast cancer, one patient with thyroid cancer and one patient with testicular cancer. All patients had chronic sarcoidosis with pulmonary involvement, and only 1 patient had acute sarcoidosis with Löfgren's syndrome. HL developed concomitantly with sarcoidosis in one patient while other two patients developed disease before and after sarcoidosis diagnosis. Two patients with solid tumors developed malignancy years before sarcoidosis diagnosis, while one patient developed thyroid cancer during sarcoidosis follow-up. All 6 sarcoidosis–malignancy patients survived after six year years follow up. We found low incidence of malignancy in patients with sarcoidosis in our small cohort. The sarcoidosis–malignancy relationship can only be a coincidence and/or can be explained by a common pathogenesis. New prospective studies involving large patients series are needed in this regard.(AU)

La relación entre sarcoidosis y malignidad no está clara todavía. Evaluamos retrospectivamente 131 pacientes con sarcoidosis seguidos por un centro de reumatología. En esta cohorte se investigó la incidencia de neoplasias malignas. Se identificó un total de 6 (4,6%) pacientes con neoplasia maligna en esta cohorte. El linfoma de Hodgkin (LH) se detectó en 3 pacientes, seguido de un paciente con cáncer de mama, un paciente con cáncer de tiroides y un paciente con cáncer testicular. El LH se desarrolló concomitantemente con sarcoidosis en un paciente, mientras que los otros 2 desarrollaron la enfermedad antes y después del diagnóstico de sarcoidosis. Dos pacientes con tumores sólidos desarrollaron malignidad años antes del diagnóstico de sarcoidosis, mientras que el paciente con cáncer de tiroides lo presentó durante el seguimiento. Los 6 pacientes con sarcoidosis y malignidad sobrevivieron durante 6 años de seguimiento. Encontramos baja incidencia de malignidad en pacientes con sarcoidosis en nuestra cohorte. La relación sarcoidosis-malignidad puede ser coincidental y/o puede explicarse por una patogénesis común.(AU)

Malignancy in patients with sarcoidosis.

The relationship between sarcoidosis and malignancy is not clear yet. We retrospectively evaluated 131 sarcoidosis patients followed-up at the single Rheumatology center. The incidence of malignancies was investigated in this cohort. A total of 6 (4.6%) patients with malignancy were identified in our cohort of 131 patients with sarcoidosis. Hodgkin lymphoma (HL) was detected in three patients, followed by one patient with breast cancer, one patient with thyroid cancer and one patient with testicular cancer. All patients had chronic sarcoidosis with pulmonary involvement, and only 1 patient had acute sarcoidosis with Löfgren's syndrome. HL developed concomitantly with sarcoidosis in one patient while other two patients developed disease before and after sarcoidosis diagnosis. Two patients with solid tumors developed malignancy years before sarcoidosis diagnosis, while one patient developed thyroid cancer during sarcoidosis follow-up. All 6 sarcoidosis-malignancy patients survived after six year years follow up. We found low incidence of malignancy in patients with sarcoidosis in our small cohort. The sarcoidosis-malignancy relationship can only be a coincidence and/or can be explained by a common pathogenesis. New prospective studies involving large patients series are needed in this regard.

Tofacitinib-induced Ramsay- Hunt Syndrome in a Patient with Rheumatoid Arthritis.

BACKGROUND: Rheumatoid arthritis (RA) is a chronic inflammatory disease characterized by joint and systemic involvement. Tofacitinib is a JAK- inhibitor that is an effective agent in the treatment of active RA. Varicella zoster virus(VZV) reactivation is among the most important adverse effects of tofacitinib. Ramsay-Hunt syndrome(RHS) is a rare clinical condition that develops as a result of VZV reactivation and progresses with hearing loss, dizziness, and facial nerve paralysis. OBJECTIVE: To present a case of Ramsay-Hunt syndrome due to varicella zoster reactivation in a RA patient using tofacitinib. CASE REPORT: A 63-year-old female RA patient under tofacitinib treatment was admitted to the rheumatology outpatient clinic due to widespread skin rashes on her face and ear, and hearing loss. On inspection widespread erythematous, vesicular rashes on the left side of the face, lips, around the eye and in the ear, and mild facial paralysis on the left side were detected. On laboratory investigations, acute phase reactants were increased. Serological study for specific antibodies against varicella zoster virus showed higher titers. Dermatology and ear nose throat specialist consultations were performed, and varicella zoster lesions on the left inner ear, face, and mild facial paresis were considered. According to clinical and laboratory findings, the patient was diagnosed with RHS triggered by tofacitinib. Tofacitinib and methotrexate were discontinued, and intravenous acyclovir was started. On the control examination, the patient's skin lesions and facial nerve paralysis regressed. CONCLUSION: Herein, we reported the first case of tofacitinib-induced RHS in a patient with RA. This may be another side effect of biologic treatment. New studies are needed on this subject.