Intravenous Anakinra for Treating Macrophage Activation Syndrome in Adult-Onset Still's Disease.

Background: Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disease characterized by fever, rash, arthritis, and multi-organ involvement. Macrophage activation syndrome (MAS), a serious complication of AOSD, poses significant diagnostic and therapeutic challenges.

Case Presentation: A 32-year-old male was diagnosed with AOSD in 2020 after being hospitalized for a fever of unknown origin and elevated liver enzymes.

Possible Association between Behçet's Disease and Periodontal diseases.

Aim: This study explores the connection between Behçet's disease (BD), characterized by persistent oral and genital ulcers alongside iritis, and periodontal disease. It examines the levels of tumor necrosis factor-α (TNF-α), interleukin-1β (IL-1β), and nitric oxide (NO) in gingival crevicular fluid (GCF) and saliva.

Methods: Forty Behçet's patients with gingivitis or periodontitis and 47 patients with either gingivitis or periodontitis but without BD were studied.

Long-term results of kidney transplantation in patients with familial mediterranean fever.

Introduction: Long-term kidney transplantation (KT) results in patients with familial Mediterranean fever (FMF)-related amyloidosis are not well studied. This study reviewed the long-term survival outcomes of FMF patients who underwent KT.

Methods: We compared the outcomes of 31 patients who underwent (KT) for biopsy-proven amyloidosis secondary to FMF with 31 control patients (five with diabetes mellitus and 26 with nondiabetic kidney disease) undergoing KT between 1994 and 2021 at BaÅŸkent University Hospital.

-allylcysteine inhibits chondrocyte inflammation to reduce human osteoarthritis via targeting RAGE, TLR4, JNK, and Nrf2 signaling: comparison with colchicine.

The discovery of new pharmacological agents is needed to control the progression of osteoarthritis (OA), characterized by joint cartilage damage. Human OA chondrocyte (OAC) cultures were either applied to -allylcysteine (SAC), a sulfur-containing amino acid derivative, or colchicine, an ancient anti-inflammatory therapeutic, for 24 h. SAC or colchicine did not change viability at 1 nM-10 µM but inhibited p-JNK/pan-JNK.

TLR4, RAGE, and p-JNK/JNK mediated inflammatory aggression in osteoathritic human chondrocytes are counteracted by redox-sensitive phenolic olive compounds: Comparison with ibuprofen.

Osteoarthritic chondrocytes show an over-activity of inflammatory catabolic mediators, and olive products have attracted attention because they were discovered to have some benefits on osteoarthritis patients. We investigated the mechanisms of action of olive leaf polyphenolic compounds in osteoarthritic chondrocytes (OACs) using a standardized leaf extract, ZeyEX, and its main phenolic component, oleuropein, also compared with anti-inflammatory drug ibuprofen. OACs, isolated from joint-cartilages of Grade 4 OA patients, were found to express COMP and MMP-9 throughout their culture period.

Comparison of clinical courses and mortality of connective tissue disease-associated interstitial pneumonias and chronic fibrosing idiopathic interstitial pneumonias.

Interstitial lung disease (ILD) is a common pulmonary manifestation of connective tissue diseases (CTD). Prognostic effect of radiological usual interstitial pneumonia (UIP) pattern in CTD-associated interstitial lung disease (CTD-ILD) is unknown. This study aimed to investigate the disease progression and mortality of patients with CTD-ILD and idiopathic interstitial pneumonias (IIP) including idiopathic pulmonary fibrosis (IPF) and idiopathic nonspecific interstitial pneumonia and the prognostic impact of the radiological UIP pattern on both disease groups.

Clinical characteristics of avascular necrosis in patients with Behçet disease: a case series and literature review.

Avascular necrosis (AVN), also known as osteonecrosis, is characterized by death of the osteocytes due to inadequate blood supply caused by various mechanisms. The hip is the most common affected joint followed by knee. Incidence of AVN in rheumatic diseases is variable and high corticosteroid (CS) therapy is a known major risk factor for development of AVN.

Serum ferritin as an activity marker for granulamotosis with polyangiitis.

Background: Serum ferritin correlates well with the activities of systemic lupus erythematosus (SLE) and dermatomyositis, but it has not been previously studied in patients with vasculitis.

Methods: Medical records of granulomatosis with polyangiitis (GPA, Wegener's granulomatosis) patients with at least six months of regular follow-up were evaluated. The activity of GPA was assessed with Birmingham Vasculitis Activity Score for Wegener's Granulomatosis (BVAS/WG).

Granulomatous interstitial nephritis associated with Primary Sjögren's syndrome.

Primary Sjögren's syndrome (pSS) is an autoimmune disease characterized by lymphocytic and plasmacytic infiltration of the exocrine glands. Tubulointerstitial nephritis (TIN) is the most common type of renal involvement in pSS. However, clinically significant renal involvement is uncommon.

Serum Vitamin B12, Homocysteine and Methylmalonic Acid Levels in Patients With Parenchymal Neuro-Behçet's Syndrome.

Objectives: This study aims to investigate the role of serum levels of vitamin B12 (VitB12), homocysteine (Hcy), and methylmalonic acid (MMA) in the development of parenchymal neuro-Behçet's syndrome (NBS) and to compare them with healthy controls and Behçet's syndrome (BS) patients without NBS.

Patients And Methods: Serum VitB12, Hcy, and MMA were measured using enzyme-linked immunosorbent assay in 64 patients (36 males, 28 females; mean age 36±8.3 years; range 20 to 56 years) who met the criteria of the International Study Group for Behçet's Disease and 30 matched healthy controls (17 males, 13 females; mean age 35.

The association between the parenchymal neurological involvement and posterior uveitis in Behçet's syndrome.

Objectives: Behçet's syndrome (BS) is a systemic vasculitis, which may involve multiple organ systems simultaneously. Clinical findings in BS often fit into well-recognized patterns, such as the association between papulo-pustular skin lesions and arthritis. We have recently observed a distinct pattern, in which a subtype of neuro-Behçet's syndrome (NBS) is often preceded by specific ophthalmic manifestations of the disease process.

Primary Sjögren's syndrome is associated with significant cognitive dysfunction.

Aim: Cognitive dysfunction is a neurologic manifestation in primary Sjögren syndrome (PSS). On the other hand, several antibodies are related to cognitive dysfunction. The aim of this study is to assess the cognitive dysfunction of PSS patients via detailed neurologic tests.

Differential diagnosis of elevated erythrocyte sedimentation rate and C-reactive protein levels: a rheumatology perspective.

Objective: In the case of high erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels, the diagnosis of the underlying disease can be challenging especially in serologically unrevealing patients who have nonspecific clinical findings. We aimed to investigate the final distribution of definitive diagnoses in patients who initially presented with nonspecific clinical findings and sustained elevations in serum ESR/CRP levels.

Material And Methods: The medical records of patients hospitalized in a rheumatology clinic between January 2010 and January 2011 were retrospectively analyzed.

The Association Between Neutrophil/Lymphocyte Ratio and Disease Activity in Rheumatoid Arthritis and Ankylosing Spondylitis.

Background: Elevated neutrophil count is associated with poor prognosis and increased mortality in many conditions. Neutrophil to lymphocyte ratio (NLR) has emerged as a marker of inflammation in neoplastic and cardiovascular disorders. Herein, we investigated utility of this simple tool in rheumatoid arthritis (RA) and ankylosing spondylitis (AS).

Retro-orbital granuloma associated with granulomatosis with polyangiitis: a series of nine cases.

Retro-orbital granuloma is a rare and devastating component of granulomatosis with polyangiitis (GPA). Current medical treatment protocols are falling short, and outcomes are poor. The aim of the study was to investigate the frequency, clinical features, and treatment outcomes of retro-orbital granuloma in patients with GPA.

An old disease with new insights: Update on diagnosis and treatment of gout.

Gout is an acute and chronic inflammatory disorder associated with high morbidity and impaired quality of life. There has been a substantial increase in the prevalence and incidence of gout in recent years. Novel diagnostic and therapeutic options have provided new insights into the pathogenesis and management of hyperuricemia and gout in the last decade.

Minimally invasive minor salivary gland biopsy for the diagnosis of amyloidosis in a rheumatology clinic.

Background. Systemic amyloidosis is a potentially fatal condition, unless diagnosed and treated before development of irreversible organ damage. Demonstration of amyloid deposits within tissue biopsies is only definitive diagnostic method, which makes appropriate selection of biopsy site essential.

Rapid resolution of protracted febrile myalgia syndrome with anakinra: Report of two cases.

Protracted febrile myalgia syndrome (PFMS) is a very rare but severe manifestation of familial Mediterranean fever (FMF) which is characterized by severe debilitating pain in large muscle groups that may last for several weeks. Colchicine is ineffective and treatment is largely supportive. Demonstration of crucial role of interleukin-1 (IL-1) in the pathogenesis of FMF has increased the use of IL-1 blockers in colchicine resistant or intolerant patients.

Turkish lupusPRO: cross-cultural validation study for lupus.

LupusPRO is a disease-targeted patient-reported outcome measure that was developed and validated from and among US patients with systemic lupus erythematosus (SLE). We herein report the results of the cross-cultural adaptation and validation study of the Turkish translated version of the LupusPRO. Turkish LupusPRO and the Medical Outcomes Study Short Form (SF-36) (Turkish) were administered to the Turkish lupus patients.

Real-time sonoelastography of Achilles tendon in patients with ankylosing spondylitis.

Objective: Real-time sonoelastography (SE) is a new ultrasound-based imaging technique that provides information on tissue elasticity and stiffness. We determined the efficacy of SE for assessing Achilles tendon abnormalities in patients with ankylosing spondylitis (AS).

Materials And Methods: Forty-one consecutive AS patients and 32 asymptomatic healthy subjects were enrolled.

Transverse myelitis in Behçet's disease: a series of four cases and review of the literature.

Objectives: Transverse myelitis (TM) is an uncommon neurologic condition characterised by the segmental involvement of the spinal cord. Although its etiology is unknown, a well established list of associations have been described, many items of which point towards an autoimmune and vasculitic process. TM is also a rare complication of Behçet's disease (BD), an autoimmune process.

An Unusual Case of Madelung's Disease with Multiple Atypical Fractures.

Madelung's disease is a rare acquired disorder of fat metabolism characterized by multiple symmetric lipomas with typical distribution mainly around the upper trunk, neck, and shoulders. The condition is strongly associated with chronic alcohol use and has various systemic manifestations like polyneuropathy, muscle weakness, and small bone fractures. Herein, we report a 56-year-old male patient with Madelung's disease and multiple fractures and discuss possible underlying factors leading to multiple fractures.

Enthesopathy in patients with familial Mediterranean fever: increased prevalence in M694 V variant.

Enthesopathy is pathology of bony insertions of tendons, ligaments or joint capsules. It is a frequent finding in rheumatic diseases, like ankylosing spondylitis (AS) and Behçet's disease. Musculoskeletal complaints are common in patients with familial Mediterranean fever (FMF), and these could be a clinical manifestation of enthesopathy.