IL-1 Inhibitors in the Treatment of Familial Mediterranean Fever: Treatment Indications and Clinical Features in a Large Real-World Cohort.

Background: The accruing evidence about the efficacy of anti-IL-1 agents in Familial Mediterranean Fever (FMF) patients led to their widespread off-label use. Therefore, identifying precise indications and clinical characteristics of IL-1i-warranting patients are important. This study investigated the clinical characteristics and treatment indications of patients with FMF requiring interleukin 1 inhibition therapy (IL-1i). Methods: Hospital records of FMF patients attending a tertiary care center at the Department of Rheumatology, University of Health Sciences, Basaksehir Cam and Sakura City Hospital were retrospectively analyzed. Data on symptoms and disease manifestations, age of symptom onset, time to diagnosis, MEFV variants, type of treatment, and their indications were collected. Results: Between June 2020 and March 2023, 312 FMF patients were identified. The mean age at the onset of symptoms was 14.0, and the mean time to diagnosis was 11.9 years. In total, 87.1% of patients were receiving colchicine monotherapy, while the remaining 11.8% warranted IL-1i. Clinical symptoms and flare manifestations did not show a significant difference between the two groups. However, patients receiving IL-1i started having symptoms at younger age (11.5 vs. 14.5, p = 0.042) and time to diagnosis was longer (18.2 vs. 11.0, p < 0.01). M694V homozygosity was more common in patients receiving IL-1i. Indications for patients receiving IL-1i were colchicine resistance (8.0%), secondary amyloidosis (5.1%), and colchicine intolerance (2.2%). Conclusions: This study shows that a subset of FMF patients, particularly those with a more severe phenotype with an earlier disease onset and M694V homozygosity, require IL-1i treatment despite the overall good efficacy and tolerability of colchicine, primarily due to colchicine resistance, intolerance, or complications such as amyloidosis.

Production and oxidative stress responses in a cage designed for creep-feeding lambs.

This research aims to assess the production and oxidative stress reactions of lambs fed in a specially designed cage for creep feeding. For this purpose, the Akkaraman lambs (n = 60) were separated into four groups (15 individuals by two sexes and two groups), and all were fed the same ration. On the days that all the data was collected, the lambs were precisely 20, 34, 48, and 62 days old. The study included collecting data on body weights, body measures, and saliva samples from lambs. When the change in body weight was investigated, the results indicated that the differences between caged creep and control feeding groups (P = 0.343) and among the groupings concerning gender (P = 0.735) were insignificant. The importance levels of change differences in body measurements based on feeding methods and gender are withers height: P = 0.003, P = 0.198; body length: P < 0.001, P = 0.394; pectoral chest width: P = 0.030, P = 0.906; chest depth: P < 0.001, P = 0.741; chest circumference: P = 0.093, P = 0.529; back length: P = 0.221, P = 0.935; head length: P = 0.004, P = 0.072 and head width: P = 0.112, P = 0.617 calculated. The study's saliva samples, the effects were examined, and significant differences in Malondialdehyde (P = 0.014), superoxide dismutase (P = 0.029), catalase (P < 0.001), and glutathione s-transferase (P = 0.001) were discovered between control feeding and caged creep feeding methods. In ischemia-modified albumin (P > 0.05), the difference between the groups was insignificant. According to these findings, caged creep feeding systems are preferable for achieving faster growth rates in Akkaraman lambs; however, caged creep feeding techniques in bigger areas with longer periods are considered better for welfare conditions.

Lupus nephritis presenting with massive ascites and pleural effusion (pseudo-pseudo Meigs' syndrome).

The triad of ascites, pleural effusion, and elevated cancer antigen-125 (CA-125) levels in the absence of ovarian malignancy in systemic lupus erythematosus patients is specifically named pseudo-pseudo Meigs' syndrome (PPMS) or Tjalma syndrome. In this case we reported a 33 years female patient with pleural effusion lasting for 3 years and new onset progressive massive ascites and increased level of CA-125. After she was evaluated for an underlying benign and malign ovarian tumor or any other malignancies, serologic tests were requested with respect to progressive renal dysfunction, proteinuria, lymphopenia, anemia, and effusion. She was diagnosed with systemic lupus erythamatosus (SLE) and renal biopsy showed class-V lupus nephritis. Immunosuppressive treatment led to improvement in both SLE activity and components of PPMS, including massive ascites and pleural effusion and without the need of diuretics. Co-existence of unexplained CA-125 increase, pleural effusion, and ascites might be related to PPMS and detailed examination to exclude malignancy and early and effective treatment of SLE are the mainstay of management.

Successful treatment of a rheumatoid arthritis patient with severe synovial hypertrophy and impaired shoulder function with microwave ablation technique.

Rheumatoid arthritis (RA) is a chronic, inflammatory systemic disorder of synovial joints and results in polyarthritis, chronical degeneration, and finally deformities and ankylosis in severe cases. Synovitis and pannus formation are results of inflammatory changes and lead into restriction in joint movement. Shoulders are among the later affected and larger joints and formation of synovitis in early active stages and pannus in later stages might be concluded with frozen shoulder and severe impairment in functionality. These late-term changes cannot be controlled with systemic or local anti-inflammatory agents and synovectomy is chosen in some cases. However, the results are not satisfactory and recurrence is common. In this case report, we presented a case of RA with severe shoulder pain, restricted movement due to synovial hypertrophy, and pannus formation which are resistant to local and systemic interventions and not suitable for surgical or chemical synovectomy. Microwave ablation (MWA) was performed successfully without any complication and she well responded in terms of DAS-28, functional, and pain scores. Range of motion and funcitonal restriction were recovered. This case report describes the use and promising results of MWA in RA with severe synovial hypertrophy and pannus formation even in the absence of active arthritis and effusion. MWA is a safe and minimally invasive technique that can be easily performed in coordinance of rheumatologists and interventional radiologists in proper cases.

A case of Behçet syndrome presenting with acute coronary syndrome.

Cardiac involvement (CI) is rare in Behçet syndrome (BS), but the important point is that CI may be the first manifestation of the disease. The presence of CI worsens the prognosis of BS, so early diagnosis and early initiation of immunosuppressive treatment (IST) are vital. Coronary aneurysm may develop spontaneously in these patients, or any vascular intervention may cause aneurysm with a pathergy-like reaction. The risk of restenosis is high after percutaneous coronary intervention or coronary artery bypass surgery applied without IST. Therefore, it should be kept in mind that IST constitutes the main step of treatment. Herein, we present a young male diagnosed with BS after acute coronary syndrome caused by coronary artery aneurysms and thrombosis.

Behcet's Syndrome Resembling Takayasu's Arteritis with the Distribution of Arterial Involvement: A Case Report and Literature Review.

Behcet's syndrome is a variable vessel vasculitis characterized by recurrent oral and genital ulcers with concomitant skin, ocular, neurologic, gastrointestinal, and joint involvement. Herein, we present a patient who was diagnosed with Behcet's syndrome, which with magnetic resonance angiography showed occlusion of the right subclavian artery at the level of the thoracic outlet and reverse flow in the right vertebral artery consistent with subclavian steal syndrome. In addition, partial narrowing was noted in the left renal artery. The distribution of arterial involvement resembled Takayasu's arteritis, although the presence of mucocutaneous lesions, male gender, history of deep vein thrombosis, and HLA-B51 positivity favored a diagnosis of vasculo-Behçet's syndrome. We treated the patient with methylprednisolone and cyclophosphamide. After the regression of vascular inflammation with immunosuppressive therapy, stenting was performed in the left renal artery.

Pulmonary involvement in antineutrophil cytoplasmic antibody-associated vasculitides: A single center experience from Turkey.

OBJECTIVE: Pulmonary involvement is an important cause of mortality and morbidity in antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV). In this study, we aimed to evaluate the type and frequency of pulmonary involvement and investigate the potential relationship between thorax computed tomography (CT) signs and other systemic clinical findings in AAV. METHODS: A total of 63 patients over the age of 18 and diagnosed with AAV were included in this study. Thoracic CT imaging findings and clinical features of the patients at the time of diagnosis were retrospectively evaluated. The frequency and distribution of detected pathological findings on imaging according to disease types, in addition to the relationship of these findings with other systemic findings and disease severity, were analyzed. RESULTS: Of 63 patients, 50 (79.4%) had pulmonary symptoms at the time of presentation. Most frequently observed pulmonary finding of thorax CT was nodular opacity. Consolidation, cavitary nodules, bronchiectasis, emphysema and fibrotic sequelae change were more common in the patients with diagnosis of granulomatosis with polyangiitis. Honeycomb, atelectasis, interstitial pneumonia, pulmonary venous congestion and pleural effusion were more common in the patients with diagnosis of microscopic polyangiitis. Ground-glass appearance, central airway disease, peribronchovascular nodules, pericardial effusion and lymphatic adenomegaly (>10 mm) were more common in the patients with diagnosis of eosinophilic granulomatosis with polyangiitis. Interstitial lung disease, pulmonary hemorrhage and severe lung involvement were found significantly increased in patients with myeloperoxidase antibody (MPO)-ANCA positivity (P < 0.05). CONCLUSION: Lung involvement was detected in almost all of the patients with AAV. Both interstitial lung disease and severe lung involvement were more frequently found in patients with MPO-ANCA positivity compared to other patients. Pulmonary examination by an imaging technique in all patients with AAV may be useful to determine the vasculitis subtype and to determine the extent of the disease. PRACTITIONER POINTS: Pulmonary involvement is quite common in AAV. Every patient with suspected AAV should be examined with imaging for lung involvement, even in the absence of respiratory symptoms. Severe pulmonary involvement is associated with the presence of severe disease and MPO-ANCA positivity.

Isolated inferior thyroidal artery vasculitis: A rare cause of neck pain.

Vasculitis is the inflammatory changes in vessels of any size that usually have a systemic involvement with a quite variable clinical presentation affecting various organs. Although systemic presentation is more common, in some cases localized inflammation of vasculature of a single organ or limited branches of aorta are reported. Here we present, an isolated vasculitis of bilateral inferior thyroidal arteries in a female patient aged 49 years, who presented with neck pain and was diagnosed with ultrasonography and computed tomographic angiography. The clinical and imaging findings were managed successfully with glucocorticoid induction and addition of methotrexate to the treatment. Localized forms of vasculitis are rarer and the limited size of the affected area makes diagnostic investigations and management more complicated. Non-invasive imaging modalities rather than conventional angiography provide useful information in a safer and easier way. Isolated vasculitis of thyroidal arteries is an extremely uncommon site and should be excluded in case of unexplained neck pain, even in the presence of normal laboratory examinations, probably because of the size of the involved vessels.

Red Cell Distribution Width to Platelet Count Ratio Reference Intervals in Premature Infants Beyond the First Week of Life.

Objective: Red cell distribution width (RDW) is a parameter of complete blood count (CBC). The RDW to platelet count ratio (RPR) is a new index that has been shown to reflect the severity of inflammation. We aim to determine the reference interval (RI) of RPR for premature newborns. Study design: The medical records of preterm infants who were followed up between January 2016 and December 2018 were reviewed. CBC levels were measured in 144 infants at <72 hours of age. Results: CBCs of infants (gestational age from 28 to 35weeks) had a RI of 0.038-0.126 for the RPR. The RI for RPR in infants with a gestational age of 32-35weeks was 0.042-0.129; and the RI for infants at 28-31weeks was 0.022-0.121. Conclusion: Establishment of RI for RPR in premature infants will allow clinical correlation of RPR alterations in this population.

The effect of being housed with a goat on abnormal behavior in horses.

Horses in stalls generally remain in single-stall housing, so abnormal behavior can be observed in this management condition. Abnormal behavior can harm the horse's keeper, rider, or even the animal itself. The present study aims to examine the effect of goats on abnormal behaviors in horses. For this purpose, it placed six horses and six goats in the single-stall housing of the horses, one horse and one goat, and monitored them for 45 d. The research was divided into three observation phases during this time: in the first observation, the horses were left alone (0-15 d), in the second observation, the horses were left together with the goats (16-30 d), and in the third observation, the horses were left alone again (31-45 d). In the first of these three stages, 12 different abnormal behaviors were detected in horses. The determined hours (00:00-01:00, 06:00-07:00, 07:00-08:00, 19:00-20:00, 20:00-21:00, 23:00-24:00 LT) of the day for abnormal behaviors identified in six different single-stall houses were examined. The frequency of the total abnormal behavior occurring at these hours was evaluated with nonparametric statistical tests. At the end of the research, front feet playing ( p = 0.002 ), crib-biting objects ( p < 0.001 ), and box walking ( p < 0.001 ) behaviors were significantly affected positively by the goats, while there was no statistical difference in other abnormal behaviors ( p > 0.05 ). In conclusion, it can be said that goats positively affect some abnormal behaviors in horses but not others.

VEXAS syndrome with severe multisystem involvement: Rapid recovery after splenectomy.

VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a newly defined disorder in which treatment is still unclear. Herein, a patient with VEXAS syndrome who had atypical findings and an interesting treatment course is presented as a case report. He had fatigue, recurrent fever, pulmonary infiltrates, proteinuria, anemia, leucopenia, transient skin rush and increased acute phase reactants. The patient, who could not tolerate corticosteroid tapering, recovered rapidly after diagnostic splenectomy and the pathological examination of the spleen revealed significant findings.

Visceral leishmaniasis in a patient with systemic lupus erythematosus: Dilemma in diagnosis and management.

Patients with systemic lupus erythemasus (SLE) have an increased risk of bacterial, viral, fungal or parasitic infections, especially if they are receiving immunosuppressive therapy. Leishmaniasis is a group of diseases caused by intracellular flagellate protozoan parasites belonging to the genus Leishmania. We present a 48-year-old female patient, diagnosed with SLE many years ago, who presented with high fever and pancytopenia. We thought that the patient's hematologic findings were related to SLE hematologic involvement. However, we investigated other possible causes when there was no response to drugs for the treatment of SLE. A second bone marrow biopsy showed Leishmania amastigotes and the patient was diagnosed with leishmaniasis. The patient was treated with liposomal amphotericin-B (treatment completed at 40 days). She showed rapid clinical improvement and showed no signs of disease after 4 months.

A Case of Protracted Febrile Myalgia Syndrome with Atypical Course and Severe Asymmetric Loss of Muscle Strength.

Protracted febrile myalgia syndrome (PFMS) is a rare form of familial Mediterranean fever (FMF) characterised by prolonged myalgia. The duration of PFMS is much longer than a typical 2-5-day attack familial Mediterranean fever and lasts for 2-6 weeks until they treated with corticosteroids. Colchicine is not effective for control of PFMS's attacks. The attacks typically resolve with corticosteroid and/or IL-1 receptor blockers. Herein, we present a young adult without typical familial Mediterranean fever clinical features but presenting with atypical course and severe asymmetric muscle strength loss. Thigh magnetic resonance imaging confirmed inflammation and oedema and muscle biopsy showed no pathological findings. Electromyography revealed myopathic findings during attack-period, despite normal results in attack-free study. The patient was treated successfully with anakinra and remarkable rapid recovery in both muscular findings and acute phase reactants were observed. PFMS should be considered even in the absence of apparent FMF attack pattern and in the presence of unexpected severe muscle weakness, especially in areas endemic for FMF and long-lasting myalgia attacks.

A case of palmoplantar pustular psoriasis induced by hydroxychloroquine in a patient with systemic lupus erythematosus.

Palmoplantar pustular psoriasis (PPP) is a rare clinical form of psoriasis. It is usually seen on the palms and soles, and affects patients' quality of life. In most cases, topical or systemic treatments are not sufficiently effective, so management of PPP is generally difficult. Hydroxychloroquine (HQ) is an antimalarial drug that is widely used in many autoimmune rheumatic diseases, mainly in systemic lupus erythematosus (SLE). Several reports describe the induction and exacerbation of psoriasis by HQ. Within this report, we aimed to put emphasis on considering possible drug effects by presenting a case of PPP, induced by HQ.

A retrospective study of age-defined hematologic inflammatory markers related to pediatric COVID-19 diagnosis.

BACKGROUND: The aim of this study was to examine age-related differences in hemogram parameters and hematologic inflammatory markers in pediatric patients with COVID-19. METHODS: This retrospective study included children aged 2 months to 18 years (n = 208) who have a confirmed diagnosis of COVID-19 and a control group comprising 117 healthy children between February 2021 and July 2021. The analysis of subgroup hematological values were performed according to the children's age cutoffs. RESULTS: The most significant difference between pediatric patients with COVID-19 and controls were peripheral blood eosinophil counts and eosinophil-to-monocyte ratio (EMR) levels on admission. The levels of monocyte-to-lymphocyte ratio, aggeregate index of systemic inflammation (neutrophil × platelet × monocyte/lymphocyte), neutrophil-to- lymphocyte × platelet ratio, and systemic inflammation response index (neutrophil × monocyte/ lymphocyte) were higher in patients than in controls. EMR had the highest area under the curve (AUC) value of 0.777, with a cutoff value of 0.26. The sensitivity for EMR was 75% under 2 years of age, and between 78.6-87.5% in the other age groups. CONCLUSION: In children younger than 6 months, the discriminative power of hematological indices is low, while the discriminative power of EMR is high at all ages when age appropriate cutoffs are used. Hematological inflammatory parameters may be particularly practical in pediatric clinics to help identify COVID-19 infection.

Antifibrotic therapies in rheumatoid arthritis associated interstitial lung disease.

Interstitial lung disease (ILD) is one of the common extra-articular manifestations of rheumatoid arthritis (RA) and it is associated with high mortality rate. The usual interstitial pneumonia (UIP) pattern of RA associated ILD (RA-ILD) shows some similarities to idiopathic pulmonary fibrosis, suggesting that antifibrotic therapies may have potential positive affects. In this review, we discuss the effectiveness of antifibrotic therapy for RA-ILD.

Palpable purpura: is it associated with vasculitis or not? A single-center experience.

BACKGROUND AND AIM: The most common cutaneous manifestation of small vessel vasculitis is palpable purpura. Etiology includes various causes such as infections, malignancies, drugs, and systemic vasculitides. The number of studies that evaluated the etiology of patients presenting with palpable purpura in the adult age group is minimal. This study aimed to determine the etiology in patients presenting with palpable purpura and analyze the clinical features associated with this pathology. MATERIALS AND METHODS: We included 85 patients over 18 years old who presented with palpable purpura in the study. The presenting demographic characteristics, medical history, systemic examination findings, laboratory, imaging, and histopathological results, and initial treatment of the patients were recorded. At the end of data collection, statistical analyses were performed to determine the patients' final diagnoses and organ involvement. RESULTS: Etiological evaluation revealed Ig A vasculitis (IgAV) in 58.8% (n = 50) of the cases, cutaneous leukocytoclastic vasculitis (CLV) in 23.5% (n = 20), and ANCA-associated vasculitis (AAV) in 3.5% (n = 3). Rheumatologic disease-associated vasculitis (RDaV) was detected in 7.1% (n = 6) of the patients. In 7.1% (n = 6) of the patients, the biopsy results were not compatible with vasculitis (NVH). DISCUSSION: Palpable purpura can occur due to many reasons. Ig A vasculitis was the most common cause of palpable purpura in our study. KEY POINTS: • All clinicians should recognize and know palpable purpura and its differential diagnosis. • IgA vasculitis is the most common cause of palpable purpura in adult patients. • In our study, Anca-associated vasculitis (AAV) was found in 3 (3%) of 85 adult patients with palpable purpura.

The relationship of serum testosterone levels with the clinical course and prognosis of COVID-19 disease in male patients: A prospective study.

BACKGROUND: A potential role of testosterone among sex hormones has been hypothesized in identifying sex-related differences in the clinical consequences of severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection. Due to the high global prevalence of hypogonadism, the relationship between hypogonadism and SARS-CoV-2 infection outcomes deserves an in-depth study. OBJECTIVE: The present study aimed to investigate the relationship of serum testosterone with other laboratory parameters on the prognosis of coronavirus disease-19 (COVID-19) in male patients with COVID-19 diagnosis. MATERIALS AND METHODS: This prospective cohort study included 358 male patients diagnosed with COVID-19 and 92 COVID-19 negative patients admitted to the urology outpatient clinics as a control group. The COVID-19 patients were divided into groups according to prognosis (mild-moderate and severe group), lung involvement in chest computed tomography (<50% and >50%), intensive care unit needs, and survival. RESULTS: The measured serum total testosterone level of the COVID-19 patients group was found to be significantly lower than that of the control group (median, 140 ng/dl; range, 0.21-328, 322 ng/dl; range, median, 125-674, p < 0.001, respectively). The serum TT levels were statistically significantly lower in severe COVID-19 patients compared to mild-moderate COVID-19 patients (median, 85.1 ng/dl; range, 0.21-532, median, 315 ng/dl; range, 0.88-486, p < 0.001, respectively), in COVID-19 patients in need of intensive care compared to COVID-19 patients who did not need intensive care (median, 64.0 ng/dl; range, 0.21-337, median, 286 ng/dl; range, 0.88-532 p < 0.001, respectively), and in COVID-19 patients who died compared to survivors (median, 82.9 ng/dl; range, 2.63-165, median, 166 ng/dl; range, 0.21-532, p < 0.001, respectively). DISCUSSION AND CONCLUSION: Our data are compatible with low TT levels playing a role on the pathogenesis of the disease in Covid-19 patients with poor prognosis and a mortal course and may guide clinicians in determining the clinical course of the disease.