Management of granulomatosis with polyangiitis complicated by intestinal perforation and pancytopenia: a case report and literature review.

Granulomatosis with polyangiitis is a systemic vasculitis. While the classic triad typically comprises otorhinolaryngologic, pulmonary, and renal manifestations, it is essential to recognize that granulomatosis with polyangiitis can affect any organ. Furthermore, reports have documented less common sites of involvement, such as the gastrointestinal tract. In this case-based review, we focus on a case of granulomatosis with polyangiitis presenting with intestinal perforation and the added challenge of concurrent pancytopenia.A 25-year-old female was diagnosed with granulomatosis with polyangiitis, with her clinical course progressing from joint pain to severe multi-organ involvement, including gastrointestinal complications. Treatment challenges emerged with the development of pancytopenia. While this may not directly result from granulomatosis with polyangiitis, it introduced an additional layer of complexity and delayed the induction of remission with immunosuppressants. Despite initial stabilization, an unexpected jejunal perforation occurred, requiring surgical intervention and subsequent postoperative care. The case underscores the complex nature of granulomatosis with polyangiitis and its potential complications. A literature search yielded discrete relevant cases in the context of our patient's intricate presentation, which has been summarized.We highlight the complexities in diagnosing and managing granulomatosis with polyangiitis-related complications, especially in uncommon presentations, and emphasize the importance of a personalized approach to patient care in these circumstances.

Peculiarities of clinical signs, course and treatment of musculoskeletal system lesions in post-COVID syndrome.

Introduction: Post-COVID syndrome (PCS) is a frequent phenomenon of patients who have suffered from an acute attack of COVID-19 infection, and it is characterized by a wide range of symptoms from different organs and systems including the musculoskeletal system (MSS). However, peculiarities of MSS lesions have not been sufficiently studied to date, in particular, in the aspect of the therapeutic process. We aimed to investigate peculiarities of MSS lesions in patients with PCS. Material and methods: Observations were carried out in 142 patients with PCS and MSS lesions. The age of patients was 36-67 years. Up-to-date methods of disease verification were used. An acute period of COVID-19 in all the patients was of moderate severity without oxygen support. Results: Musculoskeletal system lesions in patients with PCS were found to appear 1-4 weeks after the experienced acute period of COVID-19 infection. Against the background of significant arthralgia (100%) in 93 (65.5%) patients manifestations of acute arthritis were detected, the frequency of which increased with age. Musculoskeletal system lesions were found against the background of dominating PCS manifestations from the cardiovascular and digestive systems. Deterioration of the course and results of treatment of diseases caused by an age-related polymorbid background was determined. Certain difficulties in the treatment of MSS lesions by means of non-steroidal anti-inflammatory drugs and limitation in the use of glucocorticosteroids are caused by severe gastroduodenopathy and arterial hypertension. Long-term, up to 6 months, administration of L-arginine, L-carnitine and quercetin in the rehabilitation complex improved the overall results of treatment of PCS manifestations including arthropathy. Conclusions: Musculoskeletal system lesions in patients with PCS are not the main constituent of this syndrome. Difficulties in the treatment of arthropathy are due to the signs of gastroduodenopathy and arterial hypertension. Additional administration of L-arginine, L-carnitine and quercetin is reasonable.

Diagnostic challenge of tuberculosis in systemic lupus erythematosus: a case report and literature review.

Patients with systemic lupus erythematosus (SLE) are at increased risk of tuberculosis (TB) infection due to immune dysfunction and immunosuppressive therapy. We present a case study of a 40-year-old woman with systemic lupus erythematosus (SLE). Initially, she was diagnosed with a lupus flare based on her clinical symptoms and laboratory results. However, upon further investigation, positive polymerase-chain reaction results for M. tuberculosis in the cerebrospinal fluid and lung parenchymal changes on chest computed tomography scan were indicative of TB infection. There was initial uncertainty regarding whether TB had triggered a flare-up of SLE or if TB was merely mimicking the symptoms of a SLE flare-up. However, as increasing the prednisolone dose did not have a positive effect and the patient's condition improved significantly with anti-TB treatment alone, it became clearer that the deterioration observed upon admission was primarily due to TB progression rather than an SLE flare-up. Additionally, we review the current literature on TB and SLE, including risk factors, diagnostic challenges, and treatment considerations, highlighting the importance of considering TB infection in patients with SLE who present with overlapping manifestations. Prompt diagnosis and treatment are essential for improving outcomes in these patients.

Assessment of quality of life in patients with Lyme arthritis and rheumatoid arthritis.

Objectives: Patients' quality of life is one of the key concepts of modern medicine, which characterizes the role of physical and mental functioning in the course of the disease. This article presents a stratification of factors influencing the quality of life in patients with rheumatoid arthritis (RA) and Lyme arthritis (LA). Material and methods: Ninety patients with RA were included in this study among them n = 44 (48.89%) also with LA diagnosis and n = 46 (51.11%) with RA. All studied patients were examined and questioned according to the Health Assessment Questionnaire-Disability Index and the 36-Item Short Form Survey (SF-36) to assess their quality of life. The disease activity score with examination of 28 joints was used to assess the activity of the disease. Multiple regression analysis was used to determine the most significant factors influencing patients' quality of life. Results: Patients who agreed to participate in the study had high and moderate disease activity; however, patients with LA showed significantly higher baseline data on the intensity of the inflammatory process. According to the analysis of the questionnaire responses, a significantly reduced physical activity of both groups was revealed, but patients with LA had significantly worse BP (p = 0.002), GH (p = 0.006), and Mental Component Summary scales (p = 0.001). The greatest relationship between disease activity and quality of life by Physical Component Summary according to SF-36 (r = -0.80) was found in patients with LA and (r = -0.72) - with RA. Conclusions: The presence of Borrelia burgdorferi in patients with arthritis not only significantly reduced the motor activity of patients, but also complicated the mental adaptation to their own disease. Factors that affect the quality of life - the activity and duration of the disease in patients of both cohorts, and age and total number of affected joints - for patients with RA.

POSSIBILITIES OF RENOPROTECTION IN PATIENTS WITH CHRONIC KIDNEY DISEASE AND HYPERURICEMIA.

OBJECTIVE: The aim: To determine efficacy and safety of allopurinol and febuxostat in treatment of patients with CKD to reduce the sUA level and analyze its influence on glomerular filtration rate (GFR). PATIENTS AND METHODS: Materials and methods: The study included 45 CKD patients (stages 3b-5) without other severe/decompensated diseases and contraindications to the allopurinol/febuxostat. All patients underwent a comprehensive clinical and laboratory examination, and were divided into the study groups: Group I (28 patients, 61,3±3,2 y.o., CKD3b-12, CKD4-10, on hemodialysis-6 patients) received febuxostat, Group II (24 patients, 60,7±4,1y.o., CKD3b-9, CKD4-10, on hemodialysis -5 patients) took allopurinol. RESULTS: Results: Achievement of the target level of sUA was significantly often registered in Group I: after 1 month - in 45.5% (in group II - in 15.9%, p<0.001); after 3 months - in 67.5% (in group II - 21.2% p<0.01); after 6 months, these figures were 90% and 37.1%, respectively (p<0.01). sUA level <300 µmol/l was accompanied by significant positive GFR changes in group I patients; in group II there was a gradual progression of GFR deterioration in 31.8% of patients. CONCLUSION: Conclusions: In patients with pre-dialysis stages of CKD febuxostat demonstrates renoprotective abilities. Use of febuxostat in patients with CKD stage 3b-4 and in patients on hemodialysis is safe and more effective for target sUA level achievement than the use of allopurinol.

Pulmonary pattern in systemic vasculitis: granulomatosis, lung cancer or both?

As clinical manifestations of systemic vasculitides share many common features with other conditions, the rate of diagnostic errors and delayed diagnoses is high. Hence we performed an analysis of the available data regarding misdiagnosis of vasculitis as lung cancer and vice versa, as well as coexistence of vasculitis and lung cancer. The present case-based review highlights the diagnostic challenges encountered when granulomatosis with polyangiitis (GPA) mimics lung cancer. The importance of a multidisciplinary team approach to patients with pulmonary involvement and multisystem manifestations is essential for appropriate planning of further diagnostic steps and for minimizing the delay in correct diagnosis and treatment. In the present case, although computed tomography raised suspicion for lung cancer, further biopsies and laboratory screening for systemic vasculitides rejected malignancy and confirmed the diagnosis of GPA.

"All that glitters is not gold": sialomegaly, xerostomia, and dry eye is not always Sjögren's syndrome.

Although dry mouth, dry eye, and swollen salivary glands are the hallmark manifestations of Sjögren's syndrome (pSS), a wide spectrum of other conditions should be considered for differential diagnosis. The diagnostic challenge is mainly encountered in patients presenting with dry eyes and/or dry mouth, who do not meet the full established classification criteria for pSS. Presented case-based review highlights the relationship between dry eye, parotid swelling, and psychiatric disorders. The obsessive-compulsive disorder may be separately be a cause of dryness symptoms even were not using any psychiatric drugs. The presented review widely discussed this problem and the aim is to shed new light on the interpretation of the dryness symptom and the necessity of individual patient assessment, excluding causes other than pSS before making a final diagnosis and making a decision on the treatment method.

Crystallographic investigation of urine in newborn with renal disturbance due to asphyxia.

OBJECTIVE: The aim is to increase the efficiency of diagnosis of renal injury in neonates with asphyxia by identifying of structural markers according to research facies of urine in newborns of different gestational ages. PATIENTS AND METHODS: Materials and methods: The study involved 150 full-term with signs of kidney damage due to asphyxia: 75 babies with severe asphyxia, and 75 children with moderate asphyxia and 100 preterm infants: 50 children with severe asphyxia and 50 children with moderate. Comparison groups: group 1 consisted of 20 full-term infants, group 2 which included 20 preterm neonates. Material for the study - morning portion of urine, which was collected at 8-10 a.m. on 1-2 and 7-8 days of life. RESULTS: Results: morphological picture of facies of newborns with asphyxia depends on the severity of pathological changes in the urine (proteinuria) and urine output. Structure of facies in babies with renal distorbance due to severe asphyxia indicates a significant loss of organic and mineral substances in the urine. The width of the peripheral zone facies, the amount of solid particles transferred depends on the severity of asphyxia, the difference in morphology facies is maintained even at the end of the early neonatal period. CONCLUSION: Conclusions: Analysis of dried drops of urine in infants with renal impairment on the background of asphyxia can be used as one of the criteria for assessing kidney function and have prognostic value.

Acute coronary syndrome leading to revision of a co-morbid condition in a young man with arthritis.

Although patients with rheumatoid arthritis (RA) may have an increased incidence of cardiovascular events, the development of coronary artery disease and of myocardial infarction at young age is rather uncommon. Herein, we report a case of a 26-year-old man without classical cardiovascular risk factors, but with a 2-year history of RA, who experienced recurrent episodes of angina-like chest pain. His electrocardiogram showed ST-elevation and T-wave inversion in anterior chest leads, and the patient was sent for coronary angiography, which revealed multivessel coronary artery disease. Subsequently, the patient underwent coronary artery bypass grafting. Closer analysis of the patient's history and of the laboratory findings led to revision of the diagnosis of RA: the patient was found to meet the classification criteria for systemic lupus erythematosus. Pitfalls of the classification criteria and the impact of the revised diagnosis on the patient's care are discussed.

Sjögren's syndrome and lymphadenopathy unraveling the diagnosis of Lyme disease.

Lyme disease is a multisystem tick-borne transmissive disease with heterogeneous manifestations, frequently making the diagnosis difficult in clinical practice. Herein, we report a case of a 43-year-old female patient who presented with generalized lymphadenopathy requiring a further diagnostic evaluation towards lymphoma. The patient also had clinical signs of Sjögren's syndrome. Lymph node excision with subsequent histological and immunohistochemical studies were performed. While light microscopy findings were suspicious for lymphoma, immunohistochemistry results were consistent with cortical and paracortical lymph node hyperplasia with no evidence of lymphoproliferative disorder. Further laboratory testing confirmed the presence of Lyme borreliosis. Effectiveness of the administered antibiotic therapy confirmed Borrelia burgdorferi infection. Interrelationships between Sjögren's syndrome, lymphadenopathy and Lyme disease are discussed.

Efficacy and safety of switching from reference infliximab to CT-P13 compared with maintenance of CT-P13 in ankylosing spondylitis: 102-week data from the PLANETAS extension study.

OBJECTIVES: To investigate the efficacy and safety of switching from infliximab reference product (RP) to its biosimilar or maintaining biosimilar treatment in patients with ankylosing spondylitis (AS). METHODS: This open-label extension study recruited patients with AS who completed a 54-week, randomised controlled study comparing CT-P13 with RP (PLANETAS). CT-P13 (5 mg/kg) was administered intravenously every 8 weeks from week 62 to week 102. Efficacy end points included the proportion of patients achieving Assessment of SpondyloArthritis international Society (ASAS)20. Antidrug antibodies (ADAs) were measured using an electrochemiluminescent method. Data were analysed for patients treated with CT-P13 in the main PLANETAS study and the extension (maintenance group) and those who were switched to CT-P13 during the extension study (switch group). RESULTS: Overall, 174 (82.9%) of 210 patients who completed the first 54 weeks of PLANETAS and agreed to participate in the extension were enrolled. Among these, 88 were maintained on CT-P13 and 86 were switched to CT-P13 from RP. In these maintenance and switch groups, respectively, ASAS20 response rates at week 102 were 80.7% and 76.9%. ASAS40 and ASAS partial remission were also similar between groups. ADA positivity rates were comparable (week 102: 23.3% vs 27.4%). Adverse events led to treatment discontinuation during the extension study in 3 (3.3%) and 4 (4.8%) patients, respectively. CONCLUSIONS: This is the first study to show that switching from RP to its biosimilar CT-P13 is possible without negative effects on safety or efficacy in patients with AS. In the maintenance group, CT-P13 was effective and well tolerated over 2 years of treatment. TRIAL REGISTRATION NUMBER: NCT01571206; Results.

Efficacy and safety of CT-P13 (biosimilar infliximab) in patients with rheumatoid arthritis: comparison between switching from reference infliximab to CT-P13 and continuing CT-P13 in the PLANETRA extension study.

OBJECTIVES: To assess the efficacy and safety of switching from the infliximab reference product (RP; Remicade) to its biosimilar CT-P13 (Remsima, Inflectra) or continuing CT-P13 in patients with rheumatoid arthritis (RA) for an additional six infusions. METHODS: This open-label extension study recruited patients with RA who had completed the 54-week, randomised, parallel-group study comparing CT-P13 with RP (PLANETRA; NCT01217086). CT-P13 (3 mg/kg) was administered intravenously every 8 weeks from weeks 62 to 102. All patients received concomitant methotrexate. Endpoints included American College of Rheumatology 20% (ACR20) response, ACR50, ACR70, immunogenicity and safety. Data were analysed for patients who received CT-P13 for 102 weeks (maintenance group) and for those who received RP for 54 weeks and then switched to CT-P13 (switch group). RESULTS: Overall, 302 of 455 patients who completed the PLANETRA study enrolled into the extension. Of these, 158 had received CT-P13 (maintenance group) and 144 RP (switch group). Response rates at week 102 for maintenance versus switch groups, respectively, were 71.7% vs 71.8% for ACR20, 48.0% vs 51.4% for ACR50 and 24.3% vs 26.1% for ACR70. The proportion of patients with antidrug antibodies was comparable between groups (week 102: 40.3% vs 44.8%, respectively). Treatment-emergent adverse events occurred in similar proportions of patients in the two groups during the extension study (53.5% and 53.8%, respectively). CONCLUSIONS: Comparable efficacy and tolerability were observed in patients who switched from RP to its biosimilar CT-P13 for an additional year and in those who had long-term CT-P13 treatment for 2 years. TRIAL REGISTRATION NUMBER: NCT01571219; Results.