Manifestations Of Vasculitis Research Articles

Manifestations of Hydralazine-Induced Vasculitis: A Case Series

Manifestations of Hydralazine-Induced Vasculitis: A Case Series

Dupilumab for refractory chronic rhinosinusitis in eosinophilic granulomatosis with polyangiitis.

Eosinophilic granulomatosis with polyangiitis (EGPA) is frequently associated with refractory chronic rhinosinusitis with nasal polyps (CRSwNP), despite current treatments. Dupilumab demonstrated efficacy in the treatment of severe and uncontrolled CRSwNP, with improvements in patient-reported outcome measures and in objective measurements. This study aims to evaluate efficacy and safety of dupilumab in refractory CRSwNP in EGPA patients. A prospective observational study was conducted on EGPA patients treated with dupilumab between 2021 and 2023. Patients in a phase of prolonged remission of vasculitis manifestations but still experiencing active CRSwNP were included. Clinical, biological, and rhinologic evaluations were performed, alongside with patient-reported outcomes measures (PROMs) and nasal cytology. Complete response was defined by Birmingham Vasculitis Activity Score (BVAS)=0 and prednisone dose ≤ 4 mg/day, while partial response by BVAS = 0 and prednisone dose >4 mg/day. Nine EGPA patients were included. After 3 months, 55.6% achieved complete response, increasing to 83.3% at 12 months. Nasal symptoms and patient-reported outcomes improved significantly, with sustained efficacy over 12 months. An improvement in quality of life was also observed, with a significant reduction of the AAV-PRO score. Nasal cytology revealed reductions in eosinophils and neutrophils counts. Adverse events occurred in 44.4%, including hypereosinophilia in 2 cases, which led to dupilumab discontinuation. Dupilumab is an effective treatment option for severe and refractory ENT manifestations in EGPA, as it improves symptoms, reduces inflammation, and leads to better a quality of life. However, careful patient selection and monitoring are necessary to minimize adverse events and optimize outcomes.

Vasculitic involvement of the skeletal muscle and the peripheral nervous system: clinical and neuropathologic perspective

The peripheral nervous system is a classic target organ in systemic vasculitis. In addition, the skeletal muscle can also be affected. Myalgia, muscle weakness and sensory deficits are typical signs, which can lead to severe functional limitations and impaired of quality of life. Vasculitic involvement of the skeletal muscle (vasculitic myopathy [VM]) and peripheral nerves (vasculitic neuropathy [VN]) occurs predominantly in polyarteritis nodosa and small-vessel vasculitis. VM presents with elevated markers of inflammation and is typically characterized by immobilizing myalgia with normal creatine kinase activity and diffuse or patchy areas of hyperintensity on T2-weighted MRI ("MRI myositis without myositis"). In VN, sensor motor deficits predominantly affect the lower extremity in the area supplied by several peripheral nerves (e.g., mononeuritis multiplex) with acute to subacute history. The histopathological examination of nerve and muscle biopsies is the gold standard for the diagnosis of vasculitic manifestations and has a significant impact on the therapeutic approach.

Damage to the organ of hearing and upper respiratory tract in vasculitis associated with antineutrophil cytoplasmic antibodies

Antineutrophil cytoplasmic antibodies associated vasculitis (ANCA-AV) is a systemic necrotizing granulomatous vasculitis affecting mainly small-caliber vessels. ANCA-AV occupy a special place among systemic vasculitis, which is characterized by a highly active life-threatening course of the disease, requiring rapid differential diagnosis and aggressive immunosuppressive therapy. The ANCA-AV group consists of 3 nosologies: granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis and microscopic polyangiitis. The “calling card” of ANCA-AV is the lesion of the upper respiratory tract, especially the ENT organs (70–100 % of patients). The nasal cavity and paranasal sinuses are the most common areas of lesion in the head and neck (85–100 %), whereas ear damage occurs in about 35% (range, 19–61 %) of cases. Lesion of the ENT organs is typical for the debut of ANCA-AV, which makes early diagnosis difficult, since diseases of the upper respiratory tract are extremely common in all age groups. Diagnosis verification occurs mainly at the stage of generalized involvement of many organs and systems, causing severe course with the development of pulmonary-cardiac and renal insufficiency, which lead to the death of the patient. The main ENT manifestations of ANCA-AV can be grouped into several groups: sinonasal, otological, tracheobronchial, oral cavity lesions and others. Pseudotumors are often found in ANCA-AV. They are characterized by the appearance of parapharyngeal, parotid, submandibular, paratracheal volumetric formations. As a rule, the appearance of tumor-like formations is observed at an early stage of the disease and is associated with the presence of antibodies to proteinase 3, systemic manifestations of vasculitis. Pseudotumor in the ENT region may be accompanied by secondary neuropathies of cranial nerves, destruction of bone tissue, which requires histological verification of the disease.

Deficiency of Adenosine Deaminase 2

Adenosine deaminase 2 (ADA2) deficiency is an autosomal recessively inherited autoinflammatory disorder caused by loss-of-function mutations in the ADA2 gene. Although the pathogenesis involves the triggering of a proinflammatory cascade due to increased production of inflammatory cytokines such as tumor necrosis factor (TNF)-α and dysregulation of neutrophil extracellular trap formation resulting from an excess accumulation of extracellular adenosine, the pathogenetic mechanism still needs further clarification due to the broad clinical spectrum. In addition to the initially described vasculitis-related symptoms, hematological, immunological, and autoinflammatory symptoms are now well recognized. The diagnosis is made by demonstration of pathogenic variants of ADA2 with biallelic loss of function and identification of low plasma ADA2 catalytic activity. Currently, TNF-α inhibitors are the treatment of choice for controlling vasculitis manifestations and preventing strokes. However, in patients presenting with severe hematologic findings, TNF-α inhibitors are not the treatment of choice and hematopoietic stem cell transplantation has been shown to be successful in selected cases. Recombinant ADA2 protein and gene therapy are promising treatment modalities for the future. In conclusion, ADA2 deficiency has a broad phenotype and should be considered in the differential diagnosis of different clinical situations. In this review, we summarize the disease manifestations of ADA2 deficiency and available treatment options.

Magnetic resonance imaging to monitor disease activity in giant cell arteritis treated with ultra-short glucocorticoids and tocilizumab.

MRI is well established for diagnosing GCA. Its role in monitoring disease activity has yet to be determined. We investigated vascular and musculoskeletal inflammation using MRI in the patients of the GUSTO trial to assess the utility of MRI in monitoring disease activity. Eighteen patients with newly diagnosed GCA received 500 mg methylprednisolone intravenously for 3 consecutive days followed by tocilizumab monotherapy from day 3 until week 52. Cranial, thoracic and abdominal MRI exams were performed at baseline (active, new-onset disease), and at weeks 24, 52 (remission on-treatment), and 104 (remission off-treatment). MRI findings typical for PMR as well as extent and severity of vasculitic disease were rated. In total, 673 vascular segments and 943 musculoskeletal regions in 55 thoracic/abdominal MRI and 490 vascular segments in 49 cranial MRI scans of 18 patients were analysed. Vasculitic vessels were still detectable in one in four cranial segments at week 24. At weeks 52 and 104, no cranial vascular segment showed a vasculitic manifestation. Large vessels, except for the ascending aorta, and PMR displayed little or no decrease in inflammatory findings over time. Vasculitic manifestations in the cranial vessels normalised after 52 weeks of treatment, whereas large vessel and PMR findings persisted despite lasting full remission. The dynamics of cranial vessel signals suggest that MRI of these arteries might qualify as a potential diagnostic tool for monitoring disease activity and for detecting relapse after 52 weeks of treatment.

Case report: Propylthiouracil-induced serious side effect: Perinuclear antineutrophil cytoplasmic antibody-associated vasculitis or IgA vasculitis?

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a rare disease characterized by the inflammation and destruction of small blood vessels and circulating ANCAs. Drugs such as antithyroid drugs (ATDs), especially propylthiouracil (PTU), have been used for the production of ANCAs and cause the development of drug-induced AAV. The pathogenesis of this disease is unclear but could be related to the physiological processes affecting the degradation of neutrophil extracellular traps (NETs). At present, PTU is widely used in patients with Graves' disease (GD) who are preparing for pregnancy and whose condition has not been controlled. Once drug-induced AAV has occurred with important organ damage, considering NETs have a significant role in the immune system, whether the cessation of drugs could stop the progression of organ damage is unclear, and a consensus regarding standard treatment has not been established. In this case report, a female patient who planned pregnancy was hospitalized with multiple joint pain, impaired renal function, and hematuria. Immunofluorescence of the renal biopsy demonstrated spherical and diffuse mesangial distribution of IgA (3+). Autoimmune serology demonstrated positivity for autoantibodies against p-ANCA and an anti-MPO titer 74.72 RU/mL. She was diagnosed with PTU-induced p-ANCA-associated and IgA-associated vasculitis (IgAV). The patient accepted low doses of glucocorticoid, immunosuppressive therapy and RAI treatment. Both her kidney function and thyroid function remained were on the mend. The authors believe that this type of patient needs to fully consider their pregnancy preparation needs, suspend pregnancy when a small chance of GD remission is indicated, and avoid the use of drugs with reproductive toxicity and other serious adverse events. The multidisciplinary combination therapy of low-dose glucocorticoids and immunosuppressants combined with iodine radiotherapy is one reasonable scheme. At the same time, it is necessary to eliminate the organ damage caused by other reasons. This report provides a clinical treatment basis for patients with drug-induced vasculitis manifestations who cannot receive an accurate diagnosis.

#1484 Development of a relapse risk score in patients with ANCA-associated vasculitis treated with cyclophosphamide induction

Abstract Background and Aims Cyclophosphamide (CYC), together with rituximab, is the mainstay for induction therapy in ANCA-associated vasculitis (AAV) [1]. The risk factors influencing the likelihood of relapse in individuals undergoing CYC induction are still unclear. This study aims to score the more robust predictors of relapse in these patients, thereby building a clinically useful tool. Method Patients' characteristics were pooled from three cohorts from Italy, Ireland, and Spain. Eligibility criteria were a diagnosis of GPA, MPA, or EGPA, age >18 years, induction treatment with CYC [either as intravenous therapy (IV) and/or oral], and at least 12 months of follow-up. Baseline assessment included clinical data, laboratory testing, and assessment of manifestations of GPA, MPA, and EGPA in each organ system according to the Birmingham Vasculitis Activity Score (BVAS). Relapse was defined as the presence of ≥1 new vasculitis manifestation after a remission lasting at least 3 months; remission was defined as no disease activity (BVAS=0) irrespective of the glucocorticoid use. Time-to-remission and relapse-free survival probabilities were estimated using the Kaplan-Meier method. A multivariate Cox analysis, including the statistically significant factors at a previous univariate analysis, was employed to develop a practical prognostic score, defined as “Relapse Evaluation And Cyclophosphamide Treatment (REACT) score”, in which each independent predictor was assigned a number of weighted points proportional to its β regression coefficient. Results The score development cohort comprised 505 patients. 223 patients (44.2%) had MPA, 217 (43.0%) GPA, and 65 (12.9%) EGPA. 183 patients (36.2%) experienced a relapse, with a median time to relapse of 33 months (IQR: 13-61). A multivariate Cox regression analysis proved that PR3-ANCA (HR: 1, 30, 95% CI 1, 01-1, 87), IV CYC (HR: 1.78, 95% CI 1.31-2.41), cardiovascular involvement (HR: 1.82, 95% CI 1.01-3.25), arthralgias/arthritis (HR: 1.46 95% CI 1.08-1.98) and the absence of rapidly progressive glomerulonephritis (HR: 1.37, 95% CI 1.02-1.85) were all independent risk factors of relapse. These variables were used to build the REACT score, with each item receiving 1 point except for CV involvement and IV CYC, each receiving 2 points. We then identified three risk categories according to the score of each patient (Fig. 1A). We identified 138 (27%) patients with low risk of relapse (score 0-1), 252 (49%) with an intermediate risk (score 2-3), and 115 patients (22%) with a high risk (score 4-7). These three groups showed different relapse probabilities on Kaplan-Meier analysis (pairwise comparisons between the groups were all statistically significant) (Fig. 1B). Conclusion The REACT score can be employed at diagnosis to predict the risk of relapse in patients with AAV treated with cyclophosphamide induction. Its value needs to be confirmed in external cohorts.

#3033 IgA vasculitis: a unexpected diagnosis of pulmonary renal syndrome

Abstract Background IgA vasculitis is an inflammatory condition affecting blood vessels that poses a significant diagnostic and therapeutic challenge in clinical practice. Characterized by the deposition of immunoglobulin A (IgA) on vessel walls, this pathology can manifest in various ways, presenting a broad and sometimes complex clinical spectrum. Ninety percent of cases occur in the pediatric age group. Severe lung involvement, such as pulmonary hemorrhage, is rare in patients with IgAV. We present a case of a 33-year-old patient with pulmonary renal syndrome and without purpura, an atypical presentation of IgA vasculitis. Case Report We describe the case of a 33-year-old patient with no relevant medical history or regular medication, who presented to the emergency department in November 2022 with symptoms of hemoptysis, fatigue, myalgias, and pallor. On physical examination, the patient was conscious, cooperative, normotensive, tachycardic, afebrile, with bilateral axillary adenopathies, and scattered crackles on lung auscultation. Laboratory tests revealed anemia with a hemoglobin level of 5.8 g/dL, non-oliguric acute kidney injury with a creatinine level of 2.25 mg/dL, microscopic hematuria (>50/hpf), and proteinuria (UPCR of 2.8 mg/mg and UACR of 1.6 mg/mg). A chest CT scan showed bilateral and scattered ground-glass opacities consistent with alveolar hemorrhage. The patient was admitted to the Nephrology service, underwent 3 sessions of plasma exchange, and started therapy with methylprednisolone 1000 mg for 3 days. A renal biopsy revealed 24 glomeruli, with 3 showing segmental sclerosis and 1 with fibrocellular crescents, mild interstitial nephritis; immunofluorescence showed IgA+++, C3++, IgM, and IgG+- Oxford Classification: M1 E0 S1 T0 C1. Bronchofibroscopy showed a rosy bronchoalveolar lavage and macrophages with hemosiderin pigment deposition. The patient continued with prednisolone at 1 mg/kg. In January 2023 (after two months), there was a >25% reduction in albuminuria with resolution of acute kidney injury. A gradual reduction of prednisolone was initiated, and it was definitively discontinued in October 2023. In the last outpatient visit in January 2024, the patient maintained normal renal function, with no hematuria, UPCR of 100 mg/g, UACR of 70 mg/g, and no further episodes of alveolar hemorrhage. Conclusion This case stands out for its atypical presentation: an adult with alveolar hemorrhage and without other more classical manifestations of IgA vasculitis, such as purpura. This is yet another case that emphasizes the importance of the broad spectrum of diagnoses that a nephrologist should consider in the face of acute kidney injury.

Pathogenesis of Pulmonary Manifestations in ANCA-Associated Vasculitis and Goodpasture Syndrome.

Pulmonary manifestations of vasculitis are associated with significant morbidity and mortality in affected individuals. They result from a complex interplay between immune dysregulation, which leads to vascular inflammation and tissue damage. This review explored the underlying pathogenesis of pulmonary involvement in vasculitis, encompassing various forms such as granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA), and anti-GBM disease. Mechanisms involving ANCA and anti-GBM autoantibodies, neutrophil activation, and neutrophil extracellular trap (NETs) formation are discussed, along with the role of the complement system in inducing pulmonary injury. Furthermore, the impact of genetic predisposition and environmental factors on disease susceptibility and severity was considered, and the current treatment options were presented. Understanding the mechanisms involved in the pathogenesis of pulmonary vasculitis is crucial for developing targeted therapies and improving clinical outcomes in affected individuals.

Annular Leukocytoclastic Vasculitis: A New Feature of IgA Vasculitis.

Annular erythema is a rare manifestation of leukocytoclastic vasculitis. It may be associated with various drugs, infections, malignancies, or systemic diseases. A 36-year-old woman with no personal medical history presented with annular erythema with target lesions and petechial purpura. The patient had fever and joint arthralgia. A skin biopsy showed leukocytoclastic vasculitis with IgA deposits on direct immunofluorescence. The diagnosis of immunoglobulin A vasculitis with annular leukocytoclastic vasculitis was made. The patient showed global improvement with topical steroids without relapse. An annular variant of leukocytoclastic vasculitis is a rare manifestation of immunoglobulin A vasculitis. Annular erythema may reveal an IgA vasculitis.Annular leukocytoclastic vasculitis is a rare manifestation of IgA vasculitis.This presentation is treated with topical steroids.

АНЦА-ассоциированные интерстициальные заболевания легких и интерстициальная пневмония с аутоиммунными признаками

Interstitial lung disease (ILD) is a recognized manifestation of ANCA-associated vasculitis. In addition, patients with idiopathic interstitial pneumonia and ANCA positivity but without other manifestations of systemic vasculitis (ANCA-ILD) have also been reported.

Alveolar Hemorrhage in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis: Results of an International Randomized Controlled Trial (PEXIVAS).

Rationale: Diffuse alveolar hemorrhage (DAH) is a life-threatening manifestation of antineutrophil cytoplasmic antibody-associated vasculitis (AAV). The PEXIVAS (Plasma Exchange and Glucocorticoids in Severe Antineutrophil Cytoplasmic Antibody-Associated Vasculitis) (NCT00987389) trial was the largest in AAV and the first to enroll participants with DAH requiring mechanical ventilation. Objectives: Evaluate characteristics, treatment effects, and outcomes for patients with AAV with and without DAH. Methods: PEXIVAS randomized 704 participants to plasma exchange (PLEX) or no-PLEX and reduced or standard-dose glucocorticoids (GC). DAH status was defined at enrollment as no-DAH, nonsevere, or severe (room air oxygen saturation of ⩽ 85% as measured by pulse oximetry, or use of mechanical ventilation). Measurements and Main Results: At enrollment, 191 (27.1%) participants had DAH (61 severe, including 29 ventilated) and were younger, more frequently relapsing, PR3 (proteinase 3)-ANCA positive, and had lower serum creatinine but were more frequently dialyzed than participants without DAH (n = 513; 72.9%). Among those with DAH, 8/95 (8.4%) receiving PLEX died within 1 year versus 15/96 (15.6%) with no-PLEX (hazard ratio, 0.52; confidence interval [CI], 0.21-1.24), whereas 13/96 (13.5%) receiving reduced GC died versus 10/95 (10.5%) with standard GC (hazard ratio, 1.33; CI, 0.57-3.13). When ventilated, ventilator-free days were similar with PLEX versus no-PLEX (medians, 25; interquartile range [IQR], 22-26 vs. 22-27) and fewer with reduced GC (median, 23; IQR, 20-25) versus standard GC (median, 26; IQR, 25-28). Treatment effects on mortality did not vary by presence or severity of DAH. Overall, 23/191 (12.0%) with DAH died within 1 year versus 34/513 (6.6%) without DAH. End-stage kidney disease and serious infections did not differ by DAH status or treatments. Conclusions: Patients with AAV and DAH differ from those without DAH in multiple ways. Further data are required to confirm or refute a benefit of PLEX or GC dosing on mortality. Original clinical trial registered with www.clinicaltrials.gov (NCT00987389).

Histologic and Clinical Factors Associated with Kidney Outcomes in IgA Vasculitis Nephritis.

Nephritis is a common manifestation of IgA vasculitis and is morphologically indistinguishable from IgA nephropathy. While MEST-C scores are predictive of kidney outcomes in IgA nephropathy, their value in IgA vasculitis nephritis has not been investigated in large multiethnic cohorts. Biopsies from 262 children and 99 adults with IgA vasculitis nephritis ( N =361) from 23 centers in North America, Europe, and Asia were independently scored by three pathologists. MEST-C scores were assessed for correlation with eGFR/proteinuria at biopsy. Because most patients ( N =309, 86%) received immunosuppression, risk factors for outcomes were evaluated in this group using latent class mixed models to identify classes of eGFR trajectories over a median follow-up of 2.7 years (interquartile range, 1.2-5.1). Clinical and histologic parameters associated with each class were determined using logistic regression. M, E, T, and C scores were correlated with either eGFR or proteinuria at biopsy. Two classes were identified by latent class mixed model, one with initial improvement in eGFR followed by a late decline (class 1, N =91) and another with stable eGFR (class 2, N =218). Class 1 was associated with a higher risk of an established kidney outcome (time to ≥30% decline in eGFR or kidney failure; hazard ratio, 5.84; 95% confidence interval, 2.37 to 14.4). Among MEST-C scores, only E1 was associated with class 1 by multivariable analysis. Other factors associated with class 1 were age 18 years and younger, male sex, lower eGFR at biopsy, and extrarenal noncutaneous disease. Fibrous crescents without active changes were associated with class 2. Kidney outcome in patients with biopsied IgA vasculitis nephritis treated with immunosuppression was determined by clinical risk factors and endocapillary hypercellularity (E1) and fibrous crescents, which are features that are not part of the International Study of Diseases of Children classification.

COVID-19 vaccine-associated vasculitis: A systematic review.

Post-COVID-19 vaccine-associated vasculitis stands as one of the most serious side effects attributed to COVID-19 vaccines. This complication encompasses diverse manifestations which vary in presentation and severity. Moreover, it can impact patients across all age groups, with a notably elevated incidence in the elderly. This systematic review seeks to review and evaluate the spectrum of vasculitis manifestations linked to COVID-19 vaccination. A systematic review of the literature was done by searching through PubMed, Google Scholar, and Scopus up to October 2022. Articles including data about sex, age at diagnosis, vasculitis clinical manifestations, type of vaccination, most commonly used investigations, comorbid medical conditions, treatments, and clinical outcomes were included in the final analysis. Furthermore, vasculitis flare-ups post-vaccination were considered part of this review. A total number of 117 studies describing 158 patients developing vasculitis following COVID-19 vaccination were included in the final analysis. Among the patients who developed vasculitis, the most administered type of vaccination was the mRNA vaccine subtype (n = 103), followed by the viral vector vaccines (n = 42) and inactivated viral vaccines (n = 10). On the other hand, about 38% of vasculitis-related symptoms occurred after the administration of the first dose of the vaccine and 37% occurred after taking the second dose. The skin (60.7%) and the kidneys (27.8%) were the most affected organs and complete remission was achieved in 111 patients (70%), while partial remission occurred in 11% of the patient population. COVID-19 vaccine-induced vasculitis is a rare occurrence associated with COVID-19 vaccines. It generally presents a favorable prognosis and outcomes for the vast majority of patients, ultimately leading to full remission within days. This review emphasizes the notion that the advantages of COVID-19 vaccines outweigh the potential risks, particularly for individuals with compromised immune systems.

Cutaneous manifestations associated with COVID-19 infection at a university hospital in eastern China.

Coronavirus disease 2019 (COVID-19) affects different organ systems, including the skin. A retrospective analysis of skin manifestations in Chinese outpatient and inpatient settings is lacking. The study aims to analyze cutaneous manifestations in COVID-19 patients and the recurrence or aggravation of previous skin diseases. A retrospective cross-sectional study was conducted from November 2022 to July 2023 in a university hospital in eastern China. It involved reverse transcriptase polymerase chain reaction (RT-PCR)-positive COVID-19 patients, documenting various skin manifestations and the recurrence or aggravation of pre-existing skin conditions. The pattern of skin lesions and other variables were assessed. The study included 303 patients, with 127 males and 176 females. Maculopapular rash was the predominant new cutaneous manifestation (54.92%), mainly in middle-aged individuals. Other findings included urticaria (16.39%), herpes zoster (11.89%), and herpes simplex (4.10%), vesicular rashes (2.46%), purpura (2.05%), erythema multiforme (1.64%), livedo reticularis (0.41%) and so on. Severe disease was associated with herpes zoster and livedo reticularis. Critical COVID-19 cases were linked to vesicular rashes, purpura, and erythema multiforme. The mean time for skin lesion emergence post-infection varied from 3 days for seborrheic dermatitis to 17.48 days for herpes zoster. Vasculitic manifestations correlated with elevated D-dimer levels. A total of 59 cases (19.47%) of recurrent or aggravated skin diseases were reported following infection with COVID-19, with dermatitis being the most common, followed by acne and folliculitis, psoriasis, urticaria, bullous pemphigoid, pemphigus, tinea corporis and androgenetic alopecia. The cutaneous phenotypes delineated in this study expand the dermatologic spectrum associated with COVID-19. Cutaneous manifestations may result from overactive immune responses, complement activation, and microvascular damage. Herpes zoster typically occurs in elderly COVID-19 patients with weaker immune systems or more severe diseases. Purpura and livedo reticularis, although rare, may indicate disease severity. It is possible to predict the course of COVID-19 with different severity through cutaneous manifestations. Recognizing these skin manifestations could aid in predicting COVID-19 severity and guide dermatologists in managing the pandemic response.

Raynaud’s Phenomenon as a Manifestation of Vasculitis C – ANCA: Case Report and Literature Review

Raynaud's phenomenon is a vasospastic condition affecting capillaries in the distal region of the extremities. It is divided into primary and secondary and 80-90% of patients are associated with primary or idiopathic disease, while 10-20% are secondary, with multiple etiologies including connective tissue, vascular, hematologic, and endocrinopathic diseases. Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) correspond to a group of necrotizing vasculitides of medium (0.2 to 2.0 mm in diameter) and small blood vessels (arterioles, capillaries, postcapillary venules). Clinically, it is characterized by multisystem involvement, with upper and lower respiratory tract and renal damage standing out for their frequency. The least frequent manifestations are cutaneous, with digital ischemia and gangrene occurring in less than 1% of cases. The diagnosis is based on a detailed clinical history and physical examination in addition to complementary studies including a complete metabolic panel, muscle enzymes, viral panel, thyroid profile and a rheumatologic panel to assess secondary causes of Raynaud's phenomenon. Calcium antagonists are considered the first-line treatment in both etiologies, and phosphodiesterase-5 inhibitors are another alternative. This article presents a case of a patient with Raynaud's phenomenon as an initial clinical manifestation and later presentation of C-ANCA vasculitis.

Multiple systemic arterial aneurysms in large-vessel vasculitis

Large-vessel vasculitis (LVVs) is a rare inflammatory disease that predominantly affects young females' aorta and its main branches, coronary arteries, and pulmonary arteries. Clinical manifestations result from vascular stenosis, occlusion, and dilation, sometimes complicated by aneurysm rupture or dissection. Early diagnosis and treatment of LVVs are paramount to reducing the risk of ischemic complications such as visual loss and strokes, vascular stenosis and occlusion, and aortic aneurysm formation. The diagnosis of LVVs is often challenging because the presenting clinical features are nonspecific in many cases and are often shared by different types of autoimmune and inflammatory diseases including other systemic vasculitides. Prompt identification of vasculitides is important because they are associated with an increased risk of mortality. Left undiagnosed or mismanaged, these conditions may result in serious adverse outcomes that might otherwise have been avoided or minimized. We report a rare case of the LVVs with multisystemic vascular aneurysms and catastrophic complications of ruptured abdominal aortic aneurysm. Our case highlights that an early diagnosis of the diverse manifestations of vasculitis and a high index of clinical suspicion is essential to avoid delays in disease recognition that may result in permanent or life-threatening morbidity.

Інфекційна еритема та феномен Рейно в дитини після перенесеної коронавірусної хвороби: опис клінічного випадку

Purpose - to analyze practice cases with an atypical course associated with comorbid pathology; to acquaint pediatricians and family physicians with the diagnostic search algorithm. The article summarizes literature data, and describes a clinical case of erythema infectiousum in combination with Raynaud's phenomenon, which occurred in a 9-year-old child І. with coronavirus disease in anamnesis. It was established that the nature of the pathological changes: periodic hyperemia of the cheeks and auricles, “slapped cheeks”, and, at the same time, palleness of the nasolabial triangle, a pronounced change in color, marbling of the skin on the limbs and on the buttocks, with a lacy pattern of vessels, which are provoked by exposure to cold air or psycho-emotional excitement, clearly indicate both the manifestations of parvovirus infection, and the Raynaud's phenomenon mentioned above. On the other hand, post-covid syndrome is also often accompanied by non-specific changes in the skin and its derivatives (up to 20.4% of cases) in the form of urticaria, erythema, and frostbite. In the same group of patients, there are maculopapular rashes and livedo reticularis, those could be Conclusions. During the pandemic of coronavirus infection, the issues of comorbid pathological conditions in pediatrics, which have a blurred clinical picture, an atypical course, common links of pathogenesis, in particular microcirculation disorders, and require an interdisciplinary approach to the management of these diseases, rehabilitation measures, are becoming more and more relevant. interpreted both as a manifestation of systemic vasculitis, and as independent disease. Thus, the patient was diagnosed with infectious erythema caused by parvovirus B19 in combination with Raynaud's phenomenon, probably caused by a history of coronavirus disease. The multicomponent nature of the patient's symptoms is highlighted, and the diagnostic search algorithm is shown. The study was conducted in accordance with the principles of the Declaration of Helsinki. An informed parental consent was obtained for the study in children. No conflict of interests was declared by the authors.

Bilateral Staged Ureteral Stenosis Secondary to Wegener s Disease A Case report

Wegener's disease is a rare vasculitis characterized by inflammation of the endometrium of blood vessels. Ureteral stenosis is a rare manifestation of granulomatosis with polyangiitis (formerly known as Wegener's granulomatosis). We report the case of a 38-year-old woman with acute renal failure in which bilateral hydronephrosis due to staged ureteral stenosis was the second manifestation of the disease after necrotico-inflammatory skin lesions. Our patient had high titers of anti-proteinase 3 c-ANCA, but no upper or lower respiratory tract damage was demonstrated. Hydronephrosis and renal function improved rapidly under immunosuppressive therapy with high dose of corticosteroids. We reviewed the literature and found only ten other reported cases of Wegener's granulomatosis and intrinsic ureteral stenosis: in two cases the presenting clinical manifestation was unilateral hydronephrosis and in only three others bilateral hydronephrosis. All of these complications have developed on disease relapse except in one case where the first manifestation of vasculitis is bilateral ureteral stenosis. This case highlights the importance of including ANCA-related vasculitis in the differential diagnosis of unusual cases of unilateral or bilateral ureteral stenosis.