Rıdvan Mercan

This study aimed to evaluate treatment patterns, adverse events, and drug discontinuations associated with conventional synthetic (csDMARDs), biologic, and targeted synthetic disease-modifying antirheumatic drugs in patients with rheumatoid arthritis (RA) and chronic kidney disease (CKD). A retrospective cohort study was conducted on 264 RA patients with CKD, followed between 2015 and 2025. Renal function was assessed using estimated glomerular filtration rate (CKD-epidemiology collaboration) and categorized according to Kidney Disease Improving Global Outcomes guidelines into stages 3 to 5. Demographic data, laboratory parameters, comorbidities, medication history, and all drug-related adverse events were collected. Severe adverse events were defined as those requiring hospitalization, causing drug discontinuation, or resulting in death. Comparisons among CKD stages were performed using chi-square, Fisher exact test, analysis of variance, or Kruskal–Wallis tests as appropriate. Of 264 patients, the median age was 65 years, and 73.9% were female. Hypertension and diabetes were the leading comorbidities and major contributors to CKD etiology. Use of nephrotoxic csDMARDs (methotrexate, leflunomide) and nonsteroidal anti-inflammatory drugs (NSAIDs) decreased with advancing CKD, whereas glucocorticoid use increased. Toxicity from methotrexate, leflunomide, and NSAIDs rose significantly across renal stages ( P < .05 for all). In contrast, adverse event rates for biologic disease-modifying antirheumatic drugs and targeted synthetic disease-modifying antirheumatic drugs did not differ significantly between CKD stages (all P > .05). No renal function-dependent increase in severe adverse events was observed for biologic or targeted therapies. In multivariable analysis, younger age, longer RA disease duration, higher baseline estimated glomerular filtration rate, and higher baseline Disease Activity Score 28-erythrocyte sedimentation rate were independently associated with the occurrence of any adverse event. While csDMARD and NSAID toxicity increase progressively with renal impairment, biologic and targeted synthetic agents maintain a stable safety profile across CKD stages.

Background: Systemic lupus erythematosus (SLE) is a chronic, multisystem autoimmune disorder characterized by significant heterogeneity in clinical manifestations. Although SLE can involve almost any organ system, hepatic involvement is notably absent from formal classification criteria, leading to frequent underrecognition. While liver function test (LFT) abnormalities occur in up to 60% of patients, they are typically mild, transient, and often attributed to drug-induced liver injury (DILI). Severe transaminase elevation resulting from "lupus transaminitis" is rare and presents a diagnostic challenge, requiring the exclusion of viral, metabolic, and primary autoimmune liver diseases, such as autoimmune hepatitis (AIH) or AIH–SLE overlap syndrome. Case Presentation: A 28-year-old woman presented with a three-day history of abdominal pain, nausea, inflammatory arthralgia, and a facial rash. Physical examination revealed a malar rash and diffuse alopecia. Laboratory investigations showed marked hepatocellular enzyme elevation (AST 948 IU/L, ALT 618 IU/L), cytopenias, and preserved renal function. Serological evaluation demonstrated high-titer ANA (1:1000, homogeneous), markedly elevated anti-dsDNA, hypocomplementemia, and a positive direct Coombs test. Extensive screening excluded viral hepatitis, EBV, DILI, AIH, and primary biliary cholangitis. Imaging confirmed hepatosplenomegaly and diffuse lymphadenopathy without portal vein thrombosis. Based on the 2019 EULAR/ACR criteria, a diagnosis of SLE with hepatic involvement was established. Treatment with high-dose intravenous methylprednisolone, followed by oral corticosteroids and hydroxychloroquine, resulted in rapid normalization of liver enzymes. Mycophenolate mofetil was initiated for maintenance. The patient achieved sustained biochemical remission over six months with complete resolution of hepatosplenomegaly. Conclusions: Severe transaminase elevation due to lupus transaminitis is an uncommon but significant manifestation of SLE. While AIH–SLE overlap syndrome is a key differential, it is typically distinguished by AIH-specific autoantibodies and elevated IgG levels. After excluding infectious and toxic etiologies, lupus-related hepatic involvement should be considered in patients with marked LFT abnormalities and systemic inflammatory signs. Prompt initiation of immunosuppressive therapy facilitates rapid reversibility and optimizes long-term outcomes.

Background/Objectives: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a rare autoimmune disease marked by small-vessel inflammation. Pulmonary and renal manifestations are believed to critically influence prognosis, but detailed regional data are lacking. This study aimed to determine the prevalence and prognostic impact of pulmonary and renal involvement in AAV patients in the Thrace region of Türkiye. Methods: A retrospective cohort study was conducted on 78 biopsy-proven AAV patients followed between 2018 and 2025. Demographic, clinical, laboratory, and outcome data were analysed. Logistic regression identified predictors of relapse and mortality. Results: The cohort included 44 granulomatosis with polyangiitis, 30 microscopic polyangiitis, and 4 eosinophilic granulomatosis with polyangiitis patients; 40 were pr3-ANCA positive and 33 MPO-ANCA positive. Pulmonary involvement was observed in 71.8% and renal involvement in 74.4%, and overall mortality was 20.5%. All deaths occurred in patients with pulmonary involvement (28.6% vs. 0%, p = 0.048). Relapse was higher in those with pulmonary (17.9% vs. 4.5%, p = 0.048) and renal (15.5% vs. 5%, p = 0.056) involvement. Multivariate analysis showed that pulmonary involvement (OR 3.82, p = 0.002), renal involvement (OR 4.73, p = 0.013), and rituximab treatment (OR 10.79, p = 0.049) predicted relapse; elevated CRP (OR 1.01, p = 0.003), creatinine (OR 1.42, p = 0.028), hypoalbuminaemia (OR 0.24, p = 0.046), renal (OR 2.86, p = 0.031), and pulmonary (OR 3.21, p = 0.003) involvement predicted mortality. Conclusions: Pulmonary and renal involvement are highly prevalent and represent the strongest predictors of relapse and mortality in AAV patients in this regional cohort. Recognising these risks is essential to guide early interventions and improve patient outcomes.

Objectives: This study aimed to examine the frequency and associated factors of extra-musculoskeletal manifestations (EMMs) and peripheral manifestations in an axial spondyloarthritis (axSpA) cohort and their impact on the choice of first biologic treatment. Patients and methods: A total of 1,687 patients with axSpA (978 males, 709 females; mean age: 38.5±11 years) who started their first biologic disease modifying antirheumatic drug (bDMARD) were included from a national prospective database of TReasure between its inception and 2018-2021. Demographic and clinical characteristics, disease-related features, and treatment patterns were compared between patients with and without EMMs or peripheral involvement. Results: Of the patients, 1,283 had radiographic axSpA (r-axSpA), while 404 had nonradiographic axSpA (nr-axSpA). Acute anterior uveitis (AAU) was the most common (11.4%) EMM, and older age, female sex, human leukocyte antigen B27 (HLA-B27) positivity, and a lower Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) score were associated with AAU. Female sex, methotrexate use, dactylitis, and higher Ankylosing Spondylitis Disease Activity Score (ASDAS)-serum C-reactive protein (CRP) scores were related to psoriasis (PsO). Inflammatory bowel disease (IBD) and PsO were negatively associated with HLA-B27 positivity. Enthesitis was the most frequent (28.2%) peripheral manifestations, and peripheral arthritis, dactylitis, and enthesitis were independent predictor of each other. In addition, dactylitis and peripheral arthritis were related to more frequent use of conventional disease modifying antirheumatic drugs. In addition, IBD history was associated with less frequent use of etanercept. Older age, less use of sulfasalazine, the absence of enthesitis, and lower Bath Ankylosing Spondylitis Functional Disease Index (BASFI) scores were associated with secukinumab use. Conclusion: Acute anterior uveitis was associated with HLA-B27 positivity, while PsO or IBD were negatively associated with HLA-B27 in patients with axSpA. Peripheral manifestations appeared to be related to each other. Among EMMs, we found that only IBD had an effect on the bDMARD preference.

Thrombocytosis is a condition that is often detected incidentally and can be seen both in the course of myeloproliferative diseases (MPD) and as a reactive condition. Ankylosing spondylitis (AS) is a chronic multisystemic inflammatory disease that mainly affects the spine. Mild to moderate thrombocytosis may occur secondary to the course of AS. In the treatment of AS, tumor necrosis factor inhibitor (anti-TNF) treatments are actively used as first-line therapy. The number of cases of MPDs occurring in the course of AS reported in the literature is limited. Although the exact effect of anti-TNF treatment on the MPD process is not fully known, there are publications stating that caution should be exercised in cases of MPD. By this case, we wanted to share our experience of using anti-TNF therapy in a patient diagnosed with ET in the course of AS. The 35-year-old male patient had been diagnosed with AS for 11 years and had been followed up by hematology with the diagnosis of essential thrombocytosis (ET) since 2010. When there was no response to indomethacin and sulfasalazine treatments, the patient was first given etanercept, and after secondary unresponsiveness, infliximab and adalimumab treatments were given. Despite the use of multiple anti-TNFs, no hematological deterioration was detected in terms of ET. Clinical and laboratory responses were also obtained in terms of AS. The patient has been stable and in remission in terms of both AS and ET since 2016.

BACKGROUND This study aimed to evaluate the frequency of human leukocyte antigen (HLA)-B27 in 488 patients with ankylosing spondylitis (AS) from the Thrace region of Turkey and to assess its association with clinical and radiological features. MATERIAL AND METHODS A retrospective analysis was conducted on patients diagnosed with AS according to the modified New York criteria and/or Assessment of Spondyloarthritis International Society (ASAS) classification criteria. HLA-B27 status was determined using real-time polymerase chain reaction (PCR). Clinical data, including disease activity indices such as Bath AS disease activity index (BASDAI), Bath AS functional index (BASFI), C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), and radiological findings were analyzed. Statistical comparisons were performed using chi-square, Mann-Whitney U, Spearman's correlation, and logistic regression models. RESULTS Among 488 AS patients, 59.43% were HLA-B27 positive. HLA-B27 positivity was significantly associated with male sex (p=0.020), earlier disease onset (p=0.021), higher CRP (p=0.020) and ESR (p=0.04) levels, and the initial BASDAI and initial BASFI scores (p=0.042 and p=0.044). A strong correlation was found between HLA-B27 positivity and sacroiliitis severity on X-ray (p=0.016, OR: 1.518) and magnetic resonance imaging (MRI) (p=0.001, OR: 1.199). No significant associations were observed between HLA-B27 status and extra-articular manifestations. CONCLUSIONS The HLA-B27 frequency in Thracian AS patients (59.43%) was lower than reported in Western populations but consistent with some Turkish studies. HLA-B27 positivity was associated with earlier disease onset, male predominance, and more severe sacroiliitis. These findings highlight regional variations in AS presentation and the role of HLA-B27 in disease severity.

Systemic lupus erythematosus (SLE) is a systemic autoimmune disease that can present with a variety of clinical manifestations, ranging from mild skin involvement to multisystemic and severe central nervous system issues. While peripheral blood cytopenia is common in patients diagnosed with SLE, bone marrow involvement is very rare. Determining the cause of cytopenia in SLE is crucial for accurate diagnosis, effective treatment, and prognosis. SLE is characterized by immunological disorders, including T lymphocyte signal abnormalities and the pathological production of autoantibodies, influenced by various genetic and environmental factors. In SLE, the body loses immune tolerance to self-antigens. Antibodies against circulating T cells bind to the cell surface membranes of erythrocytes, leukocytes, lymphocytes, and platelets, resulting in tissue damage. This occurs through autoantibodies developed against cells, the complement system, or antibody-dependent cellular cytotoxicity. As a result, it induces cell death, cytopenia, and myelosuppression.[1] Most cytopenias in the SLE period resolve with disease control. However, in cases of persistent and long-term cytopenias, bone marrow assessment should not be overlooked.[2] The most common bone marrow abnormalities include hypocellularity, plasmacytosis, hemophagocytosis, dyserythropoiesis, and aplastic bone marrow. Myelofibrosis accounts for only 5%.[3] In a patient with myelofibrosis, distinguishing between primary and secondary myelofibrosis is crucial as the treatment approaches differ. Unlike primary myelofibrosis, the detection of autoantibodies is crucial in autoimmune myelofibrosis, where no atypical cells are present in the bone marrow. The primary pathological distinction from classical myelofibrosis is the characteristic bone marrow reticulin fibrosis.[4] SLE is the most common autoimmune disease associated with autoimmune myelofibrosis.[5] In this regard, instances of similar cases are relatively rare in the literature. A recent article reported the detection of SLE-related myelofibrosis after observing pancytopenia in a 40-year-old female patient who presented with bleeding diathesis.[6] According to an article published in 2018, a total of 45 cases of SLE-related myelofibrosis were documented in the literature.[7] The initial treatment for SLE-associated myelofibrosis typically involves steroids, often resulting in a positive response.[8] However, in cases where favorable results are not achieved or relapse occurs on dose reduction, alternative treatments become prominent. Second-line treatment with intravenous immunoglobulin has demonstrated clinically meaningful results.[9] Other potential agents for SLE-associated myelofibrosis include azathioprine, mycophenolate mofetil, and cyclosporine. In the cases documented in the literature, a treatment response is mostly achieved with these agents. However, there are just a few rare instances in which rituximab has been employed for the treatment of SLE-associated myelofibrosis due to its refractoriness to existing therapies. These cases hold promise for patients refractory to current treatments. From this perspective, it is noteworthy that rituximab, despite its potential for bone marrow depression, was successfully employed in the treatment of a patient with SLE-related myelofibrosis in 2019.[10] Few cases of rituximab use for this purpose have been documented in the literature.[11] Herein, a case of rituximab treatment for bone marrow fibrosis that developed in a patient diagnosed with SLE was discussed. A 31-year-old female patient was referred from an external medical center in 2014 for the sudden onset of pleural effusion, refractory fever, and proteinuria. The family history revealed that the patient’s aunt was diagnosed with lupus. Initial laboratory tests at that time revealed the following: hemoglobin, 7.6 g/dL (normal range: 11.7-15.5 g/dL); leukocyte count, 5.9×103/uL (normal range: 4.4-11.3×103/uL); neutrophil count, 4.49×103/uL (normal range: 1.5-7×103/uL); platelet count, 41×103/uL (normal range: 150-400×103/uL). Antinuclear antibodies were positive with a homogeneous pattern, and anti-double-stranded deoxyribonucleic acid (anti-dsDNA) was also positive. Consequently, the patient was diagnosed with SLE, and treatment was started. In 2015, mycophenolic acid was started due to persistent anemia and thrombocytopenia. However, the patient developed an allergy to mycophenolic acid; therefore, treatment was changed to azathioprine, methylprednisolone, dapsone, prednisolone, and hydroxychloroquine. The symptoms improved, and blood counts normalized. After a period of remission, the patient was reassessed five years later due to persistent cytopenia, with laboratory results showing a hemoglobin of 6.6 g/dL, a white blood cell count of 1.3×103/uL, a neutrophil count of 0.3×103/uL, and a platelet count of 42×103/uL. Antinuclear antibody was positive with a fine nucleolar pattern, while anti-dsDNA was negative, and anti-SSA was positive. The patient presented with pale conjunctivae and marked malaise. A bone marrow biopsy was performed due to the profound cytopenia, which showed a hypercellular bone marrow with evidence of fibrosis and a Grade 2-3 increase in reticular fibers. This was interpreted as bone marrow involvement in SLE. Intravenous immunoglobulin was started, along with hydroxychloroquine and methylprednisolone. However, there was no clinical improvement and cytopenia persisted. Consequently, we initiated off-label treatment with rituximab. Notably, the patient demonstrated significant improvement in pancytopenia six months after rituximab therapy. Subsequent follow-up examinations after rituximab treatment showed a hemoglobin level of 12.2 g/dL, a white blood cell count of 5.18×103/uL, a neutrophil count of 2.96×103/uL, and a platelet count of 187×103/uL. These values were measured at a 2-g dose...

Background Chronic itch is often associated with pain due to its complex nature. Neuropathic pruritus is defined as a debilitating form of chronic itching, about which not much is known to date. This condition may develop as a result of various neurological events and is associated with damage to the somatosensory nervous system, accounting for approximately 10% of chronic itching cases. Syringomyelia, transverse myelitis, radiculopathies, thoracic spine masses, brain tumors, strokes, and abscesses are among the conditions associated with neuropathic itching to date. Case presentation A 65-year-old female patient, with no known diseases and no regular medication usage, presented with a complaint of itching that had been ongoing for the past two years. Itching initially started in approximately five cm areas on the bilateral below-knee flexor surfaces. The patient, whose complaints persisted, was referred to the internal medicine outpatient clinic by dermatology. On physical examination, erythematous excoriated papules were observed in the areas affected by itching. The patient's blood sugar, liver and kidney function tests, complete blood count, erythrocyte sedimentation rate, thyroid function tests, urinalysis, stool parasitology were all normal or negative. The patient reported a sensation of coldness in the same area, was referred to neurosurgery to investigate the etiology of possible neuropathic itching. The patient's spinal imaging, revealed a spinal mass. She underwent surgery performed by a neurosurgeon, during which the spinal mass was completely removed. She reported that her itching had completely disappeared post-operatively. Conclusions The concept of itching as a variant of pain is not very new. Any damage occurring in the central nervous system or peripheral nervous system that affects the neurons responsible for transmitting and processing itch can lead to neuropathic itching. Focusing on spinal cord pathologies, any condition that damages the spinal cord may cause itching, depending on the level of damage. In cases of itching with dermatomal localization, where pain, hot or cold sensations, and paroxysmal itching are present, additional imaging methods or investigations for etiology should be performed.

Moyamoya syndrome (MMS) is a rare, chronic, progressive cerebrovascular disorder characterized by stenosis at the apices of the intracranial internal carotid arteries, including the proximal anterior cerebral arteries and middle cerebral arteries. Cerebral angiography images are used for detection through measurement. Systemic lupus erythematosus (SLE) is an autoimmune disease that can cause multisystemic involvement. The coexistence of SLE and MMS has been rarely reported in the literature. A 46-year-old male patient with malar rash, Raynaud phenomenon presented to the hospital with a complaint of weakness in the left lower extremity, which began 3 days before the date of the visit. In the diffusion magnetic resonance imaging, multiple diffusion restrictions were observed in the right frontal region. The patient underwent MR angiography, revealing stenosis in the terminal and supraclinoid segments of the right internal carotid artery, which made us consider moyamoya disease. This patient, with a malar rash and Raynaud’s, a positive antibody profile, was diagnosed as a male with SLE accompanied by MMS.