Svetlana Agachi

Introduction

Systemic rheumatoid vasculitis accounts for 1 to 5% of complications seen in rheumatoid arthritis, while autopsy studies report an average of 23% incidence. This enormous difference in numbers emphasizes the rate of misdiagnosis or underdiagnosis of systemic rheumatoid vasculitis. It mainly affects people with a median age of 65 years. It is particularly noteworthy, as systemic rheumatoid vasculitis has a high mortality and relapse rate. Also, the multifactorial aetiology: cytokines/immune cells and other particles determines clinical complexity of this type of angiitis.

Materials and methods

A comprehensive literature search of articles published since 1996 was conducted using MEDLINE via PubMed and HINARI. The search included terms such as "rheumatoid vasculitis", "rheumatoid arthritis", and "endothelial dysfunction", focusing on mechanisms driving vascular damage. A total of 217 relevant sources were identified, including original studies, reviews, and book chapters. The study evaluated pathogenic factors like cytokines, immune complexes, and systemic inflammation, highlighting their roles in endothelial dysfunction and hypercoagulability.

Results

The main pathogenetic factors in systemic rheumatoid vasculitis were immune complexes, cytokines (IL-6/TNF-α and IL-17), immune and blood cells (CD20/TH17/Platelets) and others (microparticles, blood rheology modifications). Even though, taken separately, those factors appear to have little to no impact on vascular endothelium, their synergistic effect lead to significant endothelial damage.

Conclusions

To conclude, each disease has its own pathogenetic factors which determine the natural course of this pathology. Understanding these mechanisms plays an important role for clinicians helping them to diagnose and effective treatment. Taking into consideration the relationships between specific factors in rheumatoid angiitis, we can make more specific decisions for its diagnosis and treatment. We can also use the pathophysiology of systemic rheumatoid vasculitis as a foundation for developing prevention measures.

Abstract

Introduction

Scleroderma Renal Crisis (SRC) is a life-threatening complication of systemic sclerosis (SSc), traditionally associated with anti-RNA polymerase III antibodies, corticosteroid use, and diffuse skin involvement. However, the role of COVID-19 as a potential trigger for SRC remains poorly understood. This study explores the occurrence of COVID-19-associated SRC, focusing on its clinical presentation, underlying risk factors, and outcomes.

Case presentation

We present a case series of two unvaccinated patients with systemic sclerosis who developed SRC following COVID-19 infection, despite the absence of traditional risk factors. Clinical features, laboratory findings, renal histopathology, and disease progression were analyzed to assess potential mechanisms linking SARS-CoV-2 infection to SRC onset. Both patients developed abrupt-onset malignant hypertension and acute kidney injury after supporting the COVID-19. Neither patient had a history of corticosteroid use or known anti-RNA polymerase III positivity, suggesting an alternative mechanism of SRC activation. Notably, both cases had pre-existing renal anomalies (renal developmental abnormality and prior nephrectomy), which may have contributed to increased susceptibility. Despite aggressive management, both patients developed dialysis-dependent renal failure and succumbed to SRC-related complications.

Conclusions

Our findings highlight COVID-19 as a potential trigger for SRC, possibly through endothelial dysfunction, inflammatory cytokine storms, and renal microangiopathy. The presence of pre-existing kidney conditions may further predispose SSc patients to SRC following SARS-CoV-2 infection. Additionally, the lack of vaccination in these cases raises the question of whether COVID-19 immunization could reduce SRC risk. Further research is needed to elucidate the pathophysiology, risk stratification, and long-term outcomes of COVID-19-associated SRC, as well as the role of vaccination in prevention.

Introduction

Autoimmune hemolytic anemia occurs due to the accelerated destruction of erythrocytes as a result of the dysfunction of immune system cells, which produce antibodies against the normal antigens of the membrane of hematopoietic cells. One of its causes is systemic lupus erythematosus.

Materials and methods

We present a case of a 20-year-old patient who was hospitalized with acute autoimmune hemolytic anemia, having been diagnosed with SLE at the age of 18 years. At the onset of the disease, hemolytic anemia was a differential diagnostic challenge.

Results

The differential diagnosis between primary and secondary autoimmune hemolytic anemia (AIHA) was an important step. The presence of antinuclear antibodies (ANA Hep2, Anti-dsDNA, Anti-cardiolipin, Anti-phospholipids, anti-Ro, Anti-Sm B) were important arguments in making the diagnosis. The relapse of AIHA was caused by inadequate treatment, due to a lack of compliance. Pulse therapy combined with methylprednisolone and cyclophosphamide successfully resolved the AIHA.

Conclusions

Hematological abnormalities are commonly seen in SLE patients, but hemolytic autoimmune anemia is a rare condition. A timely diagnosis of the cause of hemolytic anemia and proper treatment of lupus by correcting autoimmune disorders are crucial in disease management. Pulse therapy combined with corticosteroids and immunosuppressants is effective in acute relapses of hemolytic anemia.

Background

Pachydermoperiostosis (or primary hypertrophic osteoarthropathy) is a rare genetic disease that usually begins in childhood or adolescence, is characterized by certain clinical signs (pachydermia, periostosis, drum sticks) that gradually progress over many years until the disease stabilizes. Currently, there are 2 genes in which mutations are associated with the development of pachydermoperiostosis - HPGD and SLCO2A1. The functions of these genes are not fully understood, but their influence on the metabolism of prostaglandin E2 is known.

Case presentation

We present a case of a 20-year-old patient mistakenly diagnosed as juvenile idiopathic arthritis. Symptoms on admission to the hospital: pain accompanied by swelling in the hands and feet, arthralgias in the talocrural joints, knees with slight swelling, paresthesia in the extremities, hyperhidrosis, fatigue. Clinical and paraclinical examinations confirmed the diagnosis of pachydermoperiostosis.

Conclusions

Pachydermoperiostosis should be considered as a differential diagnosis when a patient presents with hypertrophic osteoarthropathy and acromegalic features.

Introduction

Due to the heterogeneous nature of systemic sclerosis, it is difficult to predict disease progression and complications. Despite the discovery of novel autoantibodies associated with systemic sclerosis (SSc), there is an unmet need for biomarkers for diagnosis, disease progression, and response to treatment.

Materials and methods

An analytical, qualitative study was performed with a narrative review of literature in the form of a synthesis article. Relevant primary sources published in 2020-2022 were identified and selected, using data extraction and analysis.

Results

Anti-citrullinated protein/peptide antibody could be useful in identifying patients with a more prominent joint disease. Of most interest, the anti-carbamylated protein antibodies (anti-CarP) could be a relevant biomarker related to fibrotic skin and lung disease. Positive anti-RNA (Ribonucleic acid) polymerase III antibody and antinuclear antibodies (ANA) negativity were significantly associated with GAVE (gastral antral vascular ectasia). Autoantibodies against telomeres may help identify systemic sclerosis with lung disease. Osteopontin links myeloid activation and disease progression in systemic sclerosis. CTRP (C1q tumor necrosis factor-related proteins) 9 protein levels may be biomarker of lung disease severity. CD (cluster differentiation) 21-low B cells are linked to vascular damage. L-tyrosine, L-tryptophan, and 1-methyl-adenosine distinguished healthy controls from SSc patients. L-leucine, L-isoleucine, xanthosine, and adenosine monophosphate differentiated between progressing and stable SSc-ILD. CECs (circulating endothelial cells) are a direct indicator of systemic vascular damage. Levels of the protein, galectin-3, are associated with heart involvement in people with systemic sclerosis. Low levels of the galectin-10 protein (Gal-10) in scleroderma associate with inflammation and vascular changes in the lungs, leading to pulmonary arterial hypertension (PAH). High levels of the CD146 protein may be a potential biomarker in identifying people with systemic sclerosis. Blood levels of the protein endocan increased in scleroderma patients who are at risk for pulmonary arterial hypertension . FLCs (free light chain) could be employed as useful potential biomarker of early diagnosis and to follow disease activity.

Conclusions

Novel discovered biomarkers could predict disease development, activity, and severity of diverse organ involvement, predict risk of complications of systemic sclerosis.

Introduction

Despite numerous fundamental clinical studies on the frequency, pathogenesis, clinical features of spondyloarthritis (SpA) in intestinal infectious diseases (IID) and intestinal damage in SpA, there are currently a number of unresolved problems, one of which is the problem of early diagnosis of arthropathies, especially associated with IID, which makes it necessary to continue the research in the scientific problem.

Purpose of the study

Appreciation of the features of early manifestations of axial arthropathies in intestinal infectious diseases in order to improve their early diagnosis.

Objectives of the study

Identification of clinical variants of axial arthropathies in patients with infectious intestinal diseases and instrumental description of axial lesion by conducting comparative analysis of clinical and laboratory parameters in these patients depending on the presence of axial arthropathies. Development of an algorithm for the early detection of axial spondyloarthritis in intestinal infectious diseases.

Material and methods

During 2015-2021, 141 patients were analyzed retrospectively, out of which 50 patients with SpA from the Republican Clinical Hospital „Timofei Moşneaga” and 91 patients with infectious intestinal diseases from the gastro-enterology and hepatology departments of the Republican Clinical Hospital „Timofei Moşneaga”. Depending on the etiology of IID, subjects were divided into 2 groups: the first group included patients with Yersinia enterocolitica or Campylobacter jejuni (Y±C), the second with Salmonella enteritidis or Shigella flexneri (S±Sh).

Results

In patients with IID, the following clinical variants of arthropathies have been identified: axial lesion (spinal inflammatory pain according to ASAS criteria (2009) in 42.9%, axial spondyloarthritis (axSpA) in 28.6%, AS in 15.4%), arthralgia - 38.5%, arthritis - 13.2%. Conventional radiography imaging and magnetic resonance imaging (MRI) of the sacroiliac joints (SI) increased the incidence of SpA from 6.6% (n = 6) to 28.6% (n = 26).

Conclusions

In patients with IID, the following clinical variants of arthropathies have been identified: axial lesion (spinal inflammatory pain according to ASAS criteria (2009) in 42.9%, axSpA in 28.6%, ankylosing spondylitis in 15.4%), arthralgia – 38.5%, arthritis – 13.2%. Imaging in the form of conventional radiography and MRI of SI joints increased the incidence of SpA from 6.6% (n = 6) to 28.6% (n = 26). Axial arthropathies, arthralgia, arthritis, and uveitis are encountered more frequently. At the same time, the possibility of detecting axSpA is greater if arthritis, arthralgia, inflammatory back pain, uveitis is present. Patients with S±Sh have a higher chance of developing axSpA compared to patients with Y±C.

Materials and methods

Description of two clinical cases with gout and renal involvement, one male and one female, which were hospitalized in the „Timofei Mosneaga” Republican Clinical Hospital and Municipal Clinical Hospital „Saint Trinity” in 2021.

Results

The male patient was hospitalized in a serious condition, being drowsy, disoriented and with bradyphrenia. Patient complained of dyspnea at mild exertion, severe fatigue, 5-day anuria and generalized edema. From the history collected from relatives, it was discovered that the patient has been diagnosed with gout a few years ago. The female patient has been hospitalized in the Nephrology department of the Republican Clinical Hospital „Timofei Moşneaga” for comprehensive evaluation. Her complaints: low back pain, dysuria, nocturia, moderate ankle edema, oliguria, marked asthenia, paresthesia.

Conclusions

In women, primary renal damage is more common until the onset of menopause. After the onset of menopause, there is primarily joint damage, then kidney damage, or this process develops simultaneously (kidney and joint damage). In males due to hyperuricemia, there is joint damage, which later is associated with gouty nephropathy or kidney and joint involvement evolve paralleled.