Impact of celiac disease in Behçet's syndrome patients: a study based on the database of Türkiye.

Background/aim: Our primary aim was to investigate the effects of concomitant celiac disease (CD) on the clinical characteristics of Behçet's syndrome (BS) patients. Materials and method: The study was a retrospective, nationwide, multicenter study. Turkish Ministry of Health National Electronic Database (e-Nabiz) is used under Health Ministry's supervision to extract the subject's data. Statistical analysis: Statistical analyses were made by the Statistical Package for Social Sciences (SPSS) software version 20 (IBM Corp., Armonk, New York). Continuous variables were presented by mean ± standard derivation (SD) or median (min-max) according to normality and compared by student-t test. A binary logistic regression analysis was performed to further investigating the relation between having a concomitant CD with each BD manifestation and comorbidity, frequencies of which were detected to be significantly different in the student-test. Results: A total of 84,241 patients diagnosed with BS were analyzed, and CD was identified in 175 (0.21 %) patients. The group with CD had a mean age of 41.30 ± 13.69 which was significantly younger. the prevalence of females was significantly higher (71.4%). The mean age of first admission for BS was also significantly younger in the group with CD (36.64 ± 13.28). BS patients with CD had a significantly higher prevalence of inflammatory bowel disease (27.2% vs. 7.3%, p < 0.001). When comorbid conditions were investigated depression (35.4% vs. 23.3%, p < 0.001), migraine (7.4 % vs. 2.6%, p < 0.001), fibromyalgia (10.9% vs. 4.5%, p < 0.001) and osteoporosis (12.6% vs. 6.6%, p = 0.001) were significantly more frequent in BS patients with CD. Conclusion: Our results suggest coexistence of CD in BS patients is related to female dominance and probably to an earlier disease onset. Several CD-related comorbidities as well as inflammatory bowel disease were more frequent in the CD group which implied an increased overall disease burden.

Coronavirus Disease 2019 Outcomes in Amyloid A Protein Amyloidosis Secondary to Rheumatic Conditions and Signs of Post- Coronavirus Disease 2019 Proteinuria Progression.

BACKGROUND: We aimed to investigate coronavirus diease 2019 (COVID-19) outcomes in patients with amyloid A protein (AA) amyloidosis secondary to rheumatic diseases and discuss factors associated with disease course. METHODS: A retrospective cohort was formed from adult patients with a diagnosis of AA amyloidosis. In patients with a positive severe acute respiratory syndrome coronavirus 2 polymerase chain reaction (PCR) test, rates of hospitalization, intensive care unit admission and mortality due to COVID-19 were collected from medical records. Data regarding to demographics, comorbidities, laboratory tests, medical treatments, adherence to previous treatments during COVID-19 and treatment administered for COVID-19 were collected from hospital databases and patient reviews. RESULTS: In 96 patients with AA amyloidosis, 16 had COVID-19 with a positive PCR. Ten (62.5%) patients were hospitalized, 2 (12.5%) were admitted to ICU, 1 (6.25%) was died. Hospitalized patients tended to be older. Comorbidities seemed to be more frequent in hospitalized patients. None of the patients had rapid progression to end-stage renal disease post-COVID-19. Seven patients had pre-COVID-19 and post-COVID-19 proteinuria levels. Three had notable increase in proteinuria after COVID-19 in 2 of which amyloidosis treatment was revised accordingly. CONCLUSION: Despite high rates of hospitalization in AA amyloidosis patients, mortality was observed only in 1 patient. Progression of proteinuria requiring treatment adjustment may be an issue in these patients. Cite this article as: Güven SC, Erden A, Küçük H, et al. Coronavirus disease 2019 outcomes in amyloid A protein amyloidosis secondary to rheumatic conditions and signs of post-coronavirus disease 2019 proteinuria progression. Eur J Rheumatol. Published online April 4, 2024. DOI:10.5152/eurjrheum.2024.23050.

The Possible Relation of Demodex and Facial Erythema in Connective Tissue Diseases.

Background: We aimed to investigate the frequency of Demodex infestation and clinical implications in connective tissue disease patients with facial erythema. Methods: Patients diagnosed with a connective tissue disease and had facial erythema were consecutively enrolled in the study from 2019-2020. An age and gender matched control group was formed from healthy volunteers. Presence of Demodex was investigated by standardized skin surface biopsy. Number of Demodex mites over 5 per centimeter square was considered meaningful for infestation. Topical or systemic metronidazole treatment was given to the connective tissue disease patients with Demodex infestation. Facial erythema visual analog scale was questioned in patients at treatment onset and one month after. Results: A total of 31 connective tissue disease patients with facial erythema were enrolled. Control group included 31 healthy volunteers. Demographics and comorbidities were similar between groups. Demodex infestation was present in 58.1% of the disease group and in 25.8% of the control group (P=0.01). Pruritus was the most common symptom in patients with infestation. Median (IQR) facial erythema visual analog scale score was 6 (3) at treatment onset and was 2 (2.5) one month later (P<0.001). Conclusion: When evaluating facial cutaneous lesions, Demodex infestation should not be overlooked in a patient group like connective tissue diseases with dysfunctional immune system.

Rheumatology experience with FDG PET / CT in inflammation of unknown origin: a single - centre report for determining factors associated with diagnostic precision.

BACKGROUND / AIM: The use of PET / CT is becoming more common in the elucidation of inflammatory processes in which the underlying cause cannot be determined by conventional examinations. Although PET / CT is an effective method for detecting inflammatory foci, the precise diagnosis may not be obtained in all cases. In addition, considering factors such as radiation exposure and cost, it becomes important to identify patients who can get results with PET / CT. In this study, it was aimed to examine the factors that can predict the differential diagnostic value of PET / CT by retrospectively scanning patients who underwent PET / CT for inflammation of unknown origin (IUO) in rheumatology practice. METHODS: Demographic, clinical and laboratory information of the patients followed up in our clinic and who underwent PET / CT for differential diagnosis were enrolled. Whether they were diagnosed after PET / CT and during the follow - up period, and their diagnoses were examined. RESULTS: A total of 132 patients were included in the study. A previous diagnosis of rheumatic disease was present in 28.8 % of the patients, and a history of malignancy was present in 2.3 % . The patients were divided into three groups: group 1 patients with increased FDG uptake in PET / CT and diagnosis confirmed by PET / CT, group 2 patients with increased FDG uptake in PET / CT but diagnosis was not confirmed, and group 3 patients without increased FDG uptake in PET / CT. Increased FDG uptake in PET / CT was detected in 73 % of the patients. While PET / CT helped the diagnosis in 47 (35.6 %) patients (group 1), it did not help the diagnosis in 85 (64.4 %) (groups 2 and 3). Thirty - one (65.9 %) of the diagnosed patients were diagnosed with a rheumatologic disease. When the 3 groups were compared, male gender, advanced age, CRP levels, presence of constitutional symptoms, SUVmax values and number of different organs with increased FDG uptake were higher in Group 1. Sixty - six percent and 74 % of the patients in groups 2 and 3 were not diagnosed during the follow - up period. No patient in group 3 was diagnosed with malignancy during follow - up. CONCLUSION: PET / CT has high diagnostic value when combined with clinical and laboratory data in the diagnosis of IUO. Our study revealed that various factors can affect the diagnostic value of PET / CT. Similar to the literature, the statistically significant difference in CRP levels shows that patients with high CRP levels are more likely to be diagnosed with an aetiology in PET / CT. Although detection of involvement in PET / CT is not always diagnostic, there was an important finding that no malignancy was detected in the follow - up in any patient without PET / CT involvement. Key points • PET / CT is an effective method for detecting inflammatory foci. • PET / CT has proven to be effective in the diagnosis of rheumatological diseases, the extent of disease and the evaluation of response to treatment. • Indications for the use of PET / CT in the field of rheumatology and the associated factors and clinical features supporting the diagnosis with PET / CT are still to be fully clarified. • In routine practice, with PET / CT, both delays in diagnosis and examinations performed during diagnosis and the cost can be reduced.

Anti-tumor necrosis factor alpha treatment does not influence serum levels of the markers associated with radiographic progression in ankylosing spondylitis.

Objectives: The study aimed to determine the levels of change of the markers related to radiographic progression, such as Dickkopf-1 (DKK-1), sclerostin (SOST), bone morphogenetic protein (BMP)-2 and -4, and interleukin (IL)-17 and -23, in ankylosing spondyloarthritis (AS) during anti-tumor necrosis factor alpha (TNF-α) treatment. Patients and methods: Fifty-three anti-TNF-α naïve AS patients (34 males, 19 females; median: 38 years; range, 20 to 52 years) refractory to conventional treatments meeting the modified New York criteria or Assessment of SpondyloArthritis International Society classification criteria were enrolled to this cross-sectional, controlled study between October 2015 and January 2017. Fifty healthy volunteers (35 males, 15 females; median: 36 years; range, 18 to 55 years) with similar age and sex characteristics were recruited. Serum DKK-1, BMP-2, BMP-4, SOST, IL-17, and IL-23 levels were measured in both groups. The serum levels of the markers were measured again after about two years (mean follow-up duration of 21.7±6.4 months) in AS patients who started anti-TNF-α treatment. Demographic, clinical characteristics, and laboratory parameters were recorded. The disease activity at the time of inclusion was assessed through the Bath Ankylosing Spondylitis Disease Activity Index. Results: Serum DKK-1, SOST, IL-17, and IL-23 levels in the AS group before anti-TNF-a treatment were significantly higher compared to the control group (p<0.01 for DKK-1, p<0.001 for others). There was no difference regarding serum BMP-4 levels, whereas BMP-2 levels were significantly higher in the control group (p<0.01). Forty (75.47%) AS patients had serum marker levels measured after anti-TNF-α treatment. No significant change was observed in the serum levels of these 40 patients measured 21.7±6.4 months after the initiation of anti-TNF-α treatment (p>0.05 for all). Conclusion: In AS patients, there was no change in DKK-1/SOST, BMP, and IL-17/23 cascade with anti-TNF-α treatment. This finding may suggest that these pathways act independently of each other, and their local effects are not influenced by systemic inflammation.

Eosinophilic granulomatosis with polyangiitis as a rare cause of the syndrome of inappropriate antidiuretic hormone secretion.

Eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome) is a rare multisystem necrotizing vasculitis that involves small- to medium-sized blood vessels. We report a rare case of syndrome of the inappropriate antidiuretic hormone (ADH) secretion (SIADH) secondary to EGPA. A 53-year-old man applied with complaints of pain in the large joints and morning stiffness in knee for 2 months. The patient had the history of impaired fasting glucose, asthma, nasal polyps, and urticaria. Physical examination revealed intrinsic muscle atrophy and weakness in the right hand. Peripheral eosinophil count was 9.78 × 109/L (0.02-0.5), erythrocyte sedimentation rate 39 mm/h (0-20), and C-reactive protein 5.77 mg/dL (0-0.5). Migratory ground-glass pulmonary opacities had been reported in previous chest computed tomography scans. Echocardiography revealed findings compatible with eosinophilic involvement. Electroneuromyographic evaluation showed acute distal axonal neuropathy of right ulnar nerve. EGPA was considered. Oral methylprednisolone treatment was initiated. Intravenous immunoglobulin (IVIG) and cyclophosphamide treatment and gradual tapering of oral steroids were planned. In 24-h urine analysis, sodium was 387 mEq, creatinine was 1156 mg, and volume was 3000 mL. When his medical records were investigated, it was observed that hyponatremia was present for nearly 2 years. While serum osmolality was 270, urine osmolality was 604 mOsm/kg H2O. So, SIADH diagnosis was made. Fluid intake was restricted. Although the patient's sodium level did not return to normal, it rose up to 130 mEq/L. After second cycle of EGPA treatment (cyclophosphamide and IVIG), serum sodium was normal. There is only four other documented cases of SIADH associated with EGPA. We hypothesized that blood supply to the hypothalamus and/or posterior hypophysis might be affected from EGPA vasculitis. Here, in this case, with effective treatment of EGPA, SIADH was resolved which implies a causality between two conditions.

COVID-19 course in granulomatosis with polyangiitis: single center experience with review of the literature.

BACKGROUND: Coronavirus disease 2019 (COVID-19) shares some clinical features with new-onset granulomatosis with polyangiitis (GPA) or GPA flare that may lead to a challenge in differential diagnosis. To date, little is known whether GPA can be induced by COVID-19. Herein, we aimed to seek the frequency and mortality rates of COVID-19 in our GPA cohort, and along with the literature cases, to evaluate clinical features and treatments of GPA patients with COVID-19. We also tried to identify clinical features of COVID-19 induced GPA. METHODS: As of July 2021, we conducted a systematic literature review using different spelling combinations of "COVID-19 and GPA" in the PUBMED database. In total, 18 cases were found in the literature, 6 of them had COVID-19 induced GPA. The remaining 12 of literature cases and 6 cases in our GPA cohort (n = 81) had a COVID-19 infection while followed-up with GPA. We grouped these 18 patients as GPA+COVID-19. RESULTS: The frequency of COVID-19 was 7.4% in GPA cohort and mortality rate was 33% in GPA patients with COVID-19. The most common symptoms of GPA+COVID-19 patients were fever, cough, arthralgia/myalgia, and malaise. The most frequent treatments for GPA before the COVID-19 infection were steroids (72%) and rituximab (56%). Three patients who received rituximab also had COVID-19 reinfection. In the literature cases, mortality was observed in 4 (22%) of 18 patients with GPA+COVID-19. The most common symptoms of COVID-19 induced GPA were dyspnea, fever and cough. DISCUSSION: In our GPA cohort, we observed a higher mortality rate compared to global WHO data. In patients followed up with GPA, rituximab treatment may be precarious for both COVID-19 disease and reinfection. Our study also provided some clues about the diagnostic challenge of GPA induced by COVID-19.

The effectiveness of anti-interleukin-1 therapy on subclinical inflammation parameters during the attack-free period in familial Mediterranean fever patients: A case-control study.

BACKGROUND: : Anti IL-1 therapy is useful in suppressing attacks in FMF patients with colchicine resistance, however, it is not certain whether subclinical inflammation can sufficiently be inhibited with anti-IL-1 therapy in FMF patients with amyloidosis. METHODS: Forty-six FMF patients receiving anti-interleukin-1 therapy and 36 healthy control patients were compared in terms of laboratory parameters. Also, FMF patients were further divided into two groups; those with amyloidosis and those without it, and these subgroups were compared to each other in terms of clinical and laboratory findings. RESULTS: In comparison between the FMF and healthy control groups, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) level, and red cell distribution width (RDW) level were detected to be higher and hemoglobin level lower in the patient group. Within the FMF patient group, the ESR, CRP, fibrinogen, RDW, and NLR values were significantly higher in the subgroup with amyloidosis in comparison to the subgroup without amyloidosis. DISCUSSION: Anti-interleukin-1 therapy could not fully suppress the subclinical inflammatory parameters when compared to healthy individuals.

The effectiveness of anti-interleukin-1 therapy on subclinical inflammation parameters during the attack-free period in familial Mediterranean fever patients: A case-control study.

Background/aim: Anti IL-1 therapy is useful in suppressing attacks in FMF patients with colchicine resistance, however, it is not certain whether subclinical inflammation can sufficiently be inhibited with anti-IL-1 therapy in FMF patients with amyloidosis. Materials and methods: Forty-six FMF patients receiving anti-interleukin-1 therapy and 36 healthy control patients were compared in terms of laboratory parameters. Also, FMF patients were further divided into two groups; those with amyloidosis and those without it, and these subgroups were compared to each other in terms of clinical and laboratory findings. Results: In comparison between the FMF and healthy control groups, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) level, and red cell distribution width (RDW) level were detected to be higher and hemoglobin level lower in the patient group. Within the FMF patient group, the ESR, CRP, fibrinogen, RDW, and NLR values were significantly higher in the subgroup with amyloidosis in comparison to the subgroup without amyloidosis. Conclusion: Anti-interleukin-1 therapy could not fully suppress the subclinical inflammatory parameters when compared to healthy individuals.

COVID-19 and eosinophilic granulomatosis with polyangiitis or COVID-19 mimicking eosinophilic granulomatosis with polyangiitis?

Coronavirus disease 2019 (COVID-19) and eosinophilic granulomatosis with polyangiitis (EGPA) share similarities in clinical, imaging findings and may present with respiratory distress. Differentiating a new-onset EGPA from COVID-19 during the current pandemic is a diagnostic challenge, particularly if other EGPA symptoms are overlooked. Here in this study we reviewed the literature regarding EGPA patients with COVID-19 and patients who diagnosed with EGPA or suffered an EGPA flare mimicking COVID-19. We conducted a literature survey in PUBMED database using meshed keywords "COVID-19" and "EGPA", "COVID-19" and "eosinophilic granulomatosis with polyangiitis", "COVID-19" and "Churg Strauss Syndrome", to reveal previously reported cases involving EGPA patients who had COVID-19 infection, patients who suspected to have COVID-19 but eventually diagnosed with EGPA and patients with a known diagnosis of EGPA who suffered a flare but a COVID-19 infection was suspected initially. A total of 11 cases (6 literature cases, 5 cases from our clinic) were included in our study. Seven (63.6%) of the cases were defined as COVID-19 mimicker and 4 (36.4%) were EGPA with COVID-19. All of the cases in EGPA with COVID-19 group had a history of asthma. All of them had a positive PCR result and ground-glass opacities in thorax CT. In COVID-19 mimicker group, six (85.7%) patients had a history of asthma and other EGPA features that were observed were eosinophilia in 6 (85.7%). Our study provided clues regarding the EGPA/COVID-19 diagnostic challenge which may be useful in the current pandemic. Since none of the findings in COVID-19 are disease-specific, other conditions like EGPA should not be overlooked particularly in PCR negative patients and clinical, laboratory and imaging findings should be interpreted carefully. Furthermore, we did not observe poor outcomes in EGPA patients who had COVID-19.

Hand strenght and dexterity in interphalangeal hand osteoarthritis and effects of osteophyte formations.

BACKGROUND: Hand osteoarthritis (HOA) is a complex disorder with various subtypes characterized with predominance of different features. It is challenging to estimate the severity of hand disability in HOA, since contribution of different disease components to clinical burden is yet to be clarified. The aim of the study is to investigate hand functions in nonerosive interphalangeal hand osteoarthritis (HOA) without inflammatory features, and search for effects of osteophyte formations detected by radiography and ultrasound on functionality. METHODS: Thirty one HOA patients and 20 healthy subjects with similar age, gender, body mass index were included. Hand functions were evaluated by self-reported questionnaires and objective strength and dexterity measurements. A total of 459 interphalangeal joints were evaluated and scored by radiography and ultrasound for ostephyte formations. RESULTS: Strength and dexterity measurements were similar between groups. Self-reported functionality was hampered in HOA group but not statistically significant. Osteophyte scores obtained by ultrasound and radiography were significantly higher in HOA group. Osteophyte scores obtained by ultrasound were higher than the scores obtained by radiography. Ultrasound scores showed no correlation with any of the parameters while osteophytes scores obtained by radiography partially showed a significant negative correlation with assembly part of dexterity testing. CONCLUSIONS: No significant difference observed in hand strength and dexterity in nonerosive interphalangeal HOA patients withouth signs of inflammation when compared to healthy subjects. Osteophyte formations prominent enough to be deteceted by radiography may have a negative effect on hand dexterity.

Hand strenght and dexterity in interphalangeal hand osteoarthritis and effects of osteophyte formations

Abstract Background: Hand osteoarthritis (HOA) is a complex disorder with various subtypes characterized with predominance of different features. It is challenging to estimate the severity of hand disability in HOA, since contribution of different disease components to clinical burden is yet to be clarified. The aim of the study is to investigate hand functions in nonerosive interphalangeal hand osteoarthritis (HOA) without inflammatory features, and search for effects of osteophyte formations detected by radiography and ultrasound on functionality. Methods: Thirty one HOA patients and 20 healthy subjects with similar age, gender, body mass index were included. Hand functions were evaluated by self-reported questionnaires and objective strength and dexterity measurements. A total of 459 interphalangeal joints were evaluated and scored by radiography and ultrasound for ostephyte formations. Results: Strength and dexterity measurements were similar between groups. Self-reported functionality was hampered in HOA group but not statistically significant. Osteophyte scores obtained by ultrasound and radiography were significantly higher in HOA group. Osteophyte scores obtained by ultrasound were higher than the scores obtained by radiography. Ultrasound scores showed no correlation with any of the parameters while osteophytes scores obtained by radiography partially showed a significant negative correlation with assembly part of dexterity testing. Conclusions: No significant difference observed in hand strength and dexterity in nonerosive interphalangeal HOA patients withouth signs of inflammation when compared to healthy subjects. Osteophyte formations prominent enough to be deteceted by radiography may have a negative effect on hand dexterity.(AU)

Platelet/lymphocyte ratio and mean platelet volume in patients with granulomatosis with polyangiitis

Abstract Background: Granulomatosis with polyangiitis (GPA) is a granulomatous necrotizing vasculitis with high morbidity and mortality. Anti-neutrophil cytoplasmic antibody is a valuable diagnostic marker, however its titer lacks predictive value for the severity of organ involvement. Platelet to lymphocyte ratio (PLR) and mean platelet volume (MPV) has been regarded as a potential marker in assessing systemic inflammation. We aimed to explore the value of PLR and MPV in the assessment of disease activity and manifestations of disease in GPA. Methods: 56 newly diagnosed GPA patients and 53 age-sex matched healthy controls were included in this retrospective and cross-sectional study with comparative group. Complete blood count was performed with Backman Coulter automatic analyzer, erythrocyte sedimentation rate (ESR) with Westergen method and C-reactive protein (CRP) levels with nephelometry. The PLR was calculated as the ratio of platelet and lymphocyte counts. Result: Compared to control group, ESR, CRP and PLR were significantly higher and MPV significantly lower in GPA patients. In patients group, PLR was positively correlated with ESR and CRP (r = 0.39, p = 0.005 and r = 0.51, p < 0.001, respectively). MPV was negatively correlated with ESR and CRP (r = - 0.31, p = 0.028 and r = - 0.34 p = 0.014, respectively). Patients with renal involvement had significantly higher PLR than patients without renal involvement (median:265.98, IQR:208.79 vs median:180.34 IQR:129.37, p = 0.02). PLR was negatively correlated with glomerular filtration rate (r = - 0.27, p = 0.009). A cut-off level of 204 for PLR had 65.6% sensitivity and 62.5 specificity to predict renal involvement. Conclusion: PLR exhibit favorable diagnostic performance in predicting renal involvement in patients with GPA.(AU)

Efficacy and safety of interleukin-1 inhibitors in familial Mediterranean fever patients complicated with amyloidosis.

BACKGROUND: Colchicine is the mainstay of the treatment of familial Mediterranean fever (FMF). However, 10% of FMF patients do not respond well to colchicine. Efficacy of interleukin (IL)-1 inhibitors in reducing attacks have been demonstrated in colchicine-resistant FMF (crFMF) patients recently. Colchicine is still the only approved drug for the prevention of amyloidosis in FMF and utility of IL-1 inhibitors in crFMF cases who already has amyloidosis remain to be elucidated. Herein, we evaluated efficacy and safety of IL-1 inhibitors in patients with crFMF-associated AA amyloidosis in a relatively large single center study. METHODS: Medical records of FMF patients complicated with AA amyloidosis in our dedicated FMF center were retrospectively reviewed and those patients who ever treated with IL-1 inhibitors were enrolled into the study. Patient global, physician global assessments (on 0-10 cm visual analog scale), C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), serum creatinine and 24-h urinary protein excretion values for each visit were recruited from computer-based hospital records. Treatment response of patients were assessed with clinical symptoms, serum albumin, CRP and ESR values. Renal outcome parameters were analyzed on those not receiving renal replacement therapy. RESULTS: Seventeen patients were identified with crFMF-amyloidosis that ever treated with IL-1 inhibitors. Background colchicine therapy was continued in all patients in maximal-tolerated dose along with IL-1 inhibitors. All patients benefit from IL-1 antagonists assessed by patient and physician global assessments. Inflammatory markers, CRP and ESR, were significantly reduced in all and normalized in 12 out of 17 patients. More importantly, the amount of proteinuria was remarkably improved following IL-1 inhibitor therapy (1606 mg/day to 519 mg/day, p = .008). Both anakinra and canakinumab were well-tolerated without severe side effects. All patients were initially treated with anakinra but switched to canakinumab in seven patients (one leukopenia, four injection site reaction, two inefficacy). CONCLUSION: We evaluated the clinical and laboratory responses to IL-1 inhibitors in crFMF-associated amyloidosis patients. We found significant decreases in CRP, ESR and proteinuria after IL-1 inhibitor therapy. This study confirmed that IL-1 inhibitors are effective for controlling attacks and inflammatory activity in FMF patients complicated with AA amyloidosis. Moreover, they reduce or stabilize amount of proteinuria and preserve renal function in short-term follow-up. Prolonged prospective clinical trials are warranted to assess their long-term efficacy in this particular patient group.

Platelet/lymphocyte ratio and mean platelet volume in patients with granulomatosis with polyangiitis.

BACKGROUND: Granulomatosis with polyangiitis (GPA) is a granulomatous necrotizing vasculitis with high morbidity and mortality. Anti-neutrophil cytoplasmic antibody is a valuable diagnostic marker, however its titer lacks predictive value for the severity of organ involvement. Platelet to lymphocyte ratio (PLR) and mean platelet volume (MPV) has been regarded as a potential marker in assessing systemic inflammation. We aimed to explore the value of PLR and MPV in the assessment of disease activity and manifestations of disease in GPA. METHODS: 56 newly diagnosed GPA patients and 53 age-sex matched healthy controls were included in this retrospective and cross-sectional study with comparative group. Complete blood count was performed with Backman Coulter automatic analyzer, erythrocyte sedimentation rate (ESR) with Westergen method and C-reactive protein (CRP) levels with nephelometry. The PLR was calculated as the ratio of platelet and lymphocyte counts. RESULT: Compared to control group, ESR, CRP and PLR were significantly higher and MPV significantly lower in GPA patients. In patients group, PLR was positively correlated with ESR and CRP (r = 0.39, p = 0.005 and r = 0.51, p < 0.001, respectively). MPV was negatively correlated with ESR and CRP (r = - 0.31, p = 0.028 and r = - 0.34 p = 0.014, respectively). Patients with renal involvement had significantly higher PLR than patients without renal involvement (median:265.98, IQR:208.79 vs median:180.34 IQR:129.37, p = 0.02). PLR was negatively correlated with glomerular filtration rate (r = - 0.27, p = 0.009). A cut-off level of 204 for PLR had 65.6% sensitivity and 62.5 specificity to predict renal involvement. CONCLUSION: PLR exhibit favorable diagnostic performance in predicting renal involvement in patients with GPA.