Secondary Vasculitis Research Articles

Peripheral neuroimmunological diseases - Neuropathological insights and clinical perspectives

This article deals with peripheral neuroimmunological diseases and briefly outlines the currently most important aspects and treatment developments. Idiopathic inflammatory myopathies have different mechanisms of development, manifestations and prognoses. New classification systems and more specific treatment concepts have been developed. The IIMs include different subgroups. These entities can have specific autoantibodies. Diagnostically, amuscle biopsy is generally desirable for aprecise diagnosis and is essential in unclear cases. Primary systemic vasculitides can be divided into different groups based on the predominant pattern of involvement, while secondary vasculitides and single organ vasculitides are also differentiated. Vasculitic myopathy cannot be equated with myositis and areliable distinction is currently only possible by a muscle biopsy. Treatment concepts should be developed on an interdisciplinary basis. Chronic inflammatory demyelinating polyneuropathy is the most frequent immune-mediated neuropathy and is characterized by apredominant demyelination of the motor and sensory nerves. The disease course runs in phases or is progressive and leads to significant disability and reduction in quality of life, despite current standard treatment. Novel treatment approaches are currently undergoing clinical trials. Myasthenia gravis, with the leading symptom of exercise-induced muscle weakness, is caused by autoantibodies against structures of the neuromuscular endplate. Autoantibody testing is the most important pillar in the diagnosis and is now also increasingly guiding treatment decisions. Overall, peripheral neuroimmunological diseases represent aheterogeneous group. Increasing knowledge of the pathophysiology is the key to numerous developments in diagnostics and treatment, which could lead to far-reaching practical changes in the future.

#1782 Clinicopathological study of renal limited pauci-immune vasculitis and its short-term outcome: a single centre observational study

Abstract Background and Aims Pauci immune crescentic glomerulonephritis, is a leading cause of RPGN. The majority of patients with PICGN have glomerular diseases as a part of a systemic small vessel vasculitis, a minority may present as renal-limited vasculitis. In north-east India, there are still no major studies in this group of patients. Therefore, our study involving north-east Indian population constitutes an effort to prospectively study the clinico-pathological feature of patients with PICGN and assess the short-term outcome. Method This single-centre, prospective, observational, study was conducted over 12 months at the Department of Nephrology, Gauhati Medical College. All renal biopsy-proven patients with pauci-immune renal vasculitis were included, excluding those with secondary vasculitis. Baseline characteristics and laboratory data were recorded, and disease activity was assessed using the Birmingham Vasculitis Activity Score. The ANCA tests were performed using a standardised essay for both indirect immunofluorescence assay and antigen-specific ELISA. The renal biopsy specimen obtained was evaluated by light microscopy and direct immunofluorescence. The sample size was calculated based on the prevalence of pauci-immune glomerulonephritis, and data were analysed using SPSS. Results The study involved 24 patients, predominantly females (70.83%), aged 14-65 years, presenting predominantly with pedal oedema and oliguria. Laboratory findings showed varying levels of haemoglobin, total count, platelet count, creatinine, and 24-hour urine protein. 50% of the patients were (MPO) positive, 33.3% were (PR3) positive while 16% were ANCA negative. Renal biopsy analysis showed a majority with mixed class according to the Berden classification. Treatment involved induction with Methylprednisolone and Cyclophosphamide, with dialysis administered to 58.3% of patients. At six months, 36.3% had a stable GFR, and 50% were on maintenance hemodialysis. Factors predicting renal loss included oliguria, dialysis at presentation, and fibrous/fibrocellular crescents. The best predictor of renal outcome was serum creatinine at presentation. Conclusion Pauci-immune CrGN is a severe disease affecting a wide age range. Early diagnosis and treatment initiation are crucial for better outcomes, including lower ESRD risk and relapse rates. Prognostic factors include baseline serum creatinine levels and fibrous crescents. Optimising treatment regimens may improves outcomes.

Non-Eosinophilic Granulomatosis with Polyangiitis Vasculitic Neuropathy: An Overview

Vasculitic neuropathy is commonly associated with systemic vasculitis, leading to ischemic damage to the peripheral nerves and axonal degeneration. The typical clinical manifestation of vasculitic neuropathy is a sensory-dominant multiple mononeuropathy often accompanied by pain. Although vasculitic neuropathy is caused by various systemic diseases, ANCA-associated vasculitis, secondary systemic vasculitis linked to various collagen diseases, and non-systemic vasculitic neuropathy hold particular significance. A comprehensive understanding of vasculitic neuropathy is crucial for its early diagnosis, contributing to an improved prognosis for this condition.

The Contribution of Vessel Wall Magnetic Resonance Imaging to the Diagnosis of Primary and Secondary Central Nervous System Vasculitis.

To describe high-resolution brain vessel wall MRI (VW-MRI) patterns and morphological brain findings in central nervous system (CNS) vasculitis patients. Fourteen patients with confirmed CNS Vasculitis from two tertiary centers underwent VW-MRI using a 3T scanner. The images were reviewed by two neuroradiologists to assess vessel wall enhancement characteristics and locations. Fourteen patients were included (six females; average age 48 ± 19 years). Diagnoses included primary CNS vasculitis (PCNSV) in six patients and secondary CNS vasculitis (SCNSV) in eight, half of which were infection-related. Thirteen patients showed vessel wall enhancement, which was intense in eleven patients (84.6%) and concentric in twelve (92.3%), affecting the anterior circulation in nine patients (69.2%), posterior in two patients (15.4%), and both circulations in two patients (15.4%). The enhancement patterns were similar across different CNS vasculitis types. DWI changes corresponded with areas of vessel wall enhancement in 77% of patients. Conclusions: CNS vasculitis is often associated with intense, concentric vessel wall enhancement in VW-MRI, especially in the anterior circulation. The consistent presence of DWI alterations in affected territories suggests a possible link to microembolization or hypoperfusion. These imaging findings complement parenchymal brain MRI and MRA/DSA data, potentially increasing the possibility of a clinical diagnosis of CNS vasculitis.

P027 Infliximab in the treatment of cerebral tuberculosis paradoxical reactions with vasculitis

Abstract Background/Aims Tuberculosis meningitis (TBM) is associated with high morbidity and mortality. Up to 25% of patients may experience paradoxical reactions (PR) after commencing anti-TB therapy, which are exaggerated inflammatory reactions resulting in parenchymal inflammation, tuberculomas and secondary vasculitis. PR are minimised with steroid co-treatment, and relapsing disease often requires escalating steroid therapy. High levels of CSF TNFα have been shown in TBM with PR and immunosuppressants such as thalidomide, azathioprine and mycophenolate have been used with limited success. Herewith we describe a patient with TBM and PR with secondary vasculitis and consequent stroke treated successfully with infliximab. Methods A 55-year-old man presented with collapse, drowsiness, anergia and lateral gaze nystagmus. He was diagnosed with MDR TB-meningitis 3 months earlier and treated with cycloserine, clofazimine, moxifloxacin, bedaquiline, linezolid and dexamthasone. Results Prior to this presentation there had been recurring headaches after weaning dexamathasone to cessation. Two days into admission, he developed an unsteady gait with bilateral intention tremor, bi-directional nystagmus and left upper limb dysdiadochokinesia. An MRI brain showed new intraparenchymal and meningeal lesions suggestive of tuberculomas with acute infarcts involving both thalami and the left external capsule with hydrocephalus. Intracranial CT angiogram showed irregular posterior cerebral arteries indicative of arteritis. Findings were characteristic for TB paradoxical reactions with a secondary vasculitis. Dexamethasone was recommenced at 6mg BD and weaned to 3mg over a one month period. Mycophenolate was commenced as a steroid sparing agent for PR and up-titrated to 1000mg BD over four weeks. There was minimal improvement in the patient’s neurological state and repeat MRI showed some improvement in tuberculomas, but new infarcts within the right globus pallidus and left thalamus. As there was minimal progress and high steroid dependence the patient was loaded with infliximab (5mg/kg) over the next 8 weeks. An MRI done after three infusions showed significant improvement in tuberculomas, resolution of arteritis and residual meningeal disease which correlated with clinical improvement. As progress was improving infliximab was stopped after six infusions, and both mycophenolate and prednisolone weaned to cessation. At six months of follow-up the patient has made a full neurological recovery. Conclusion TBM and PR are associated with significant morbidity and disability. There are no evidence based treatments for PR and corticosteroids remain the mainstay of treatment. Several case series have suggested a role for anti-TNF agents and our described case contributes to the role of anti-TNF in treating patients with TB PR with secondary vasculitis. Disclosure H. Ko: None. A. Fatima: None. A. Sivagnanaratnam: None. A. McGregor: None. S. Hamdulay: None.

Vasculitis in the Central Nervous System: Etiology, Characteristics, and Outcomes in a Large Single-Center Cohort.

For the management of central nervous system (CNS) vasculitis, it is crucial to differentiate between primary and secondary CNS vasculitis and to understand the respective etiologies. We assessed the etiology, characteristics, and outcomes of patients with CNS vasculitis. A single-center retrospective chart review was conducted at the University of Utah, Department of Neurology, between February 2011 and October 2022. The median age of the 44 included patients at diagnosis was 54years; 25.0% were men. Compared to primary CNS vasculitis, secondary CNS vasculitis exhibits higher fever incidence (observed in infectious and connective tissue disorder [CTD]-associated vasculitis), low glucose levels (mostly in infectious vasculitis) and unique cerebrospinal fluid oligoclonal bands (observed in infectious and CTD-associated vasculitis). Patients with inflammatory cerebral amyloid angiopathy (CAA) were older and more commonly had microhemorrhage than primary angiitis of the CNS (PACNS). All patients with CTD-associated vasculitis had a known history of CTD at presentation. Brain biopsies were performed on 10 of 17 PACNS patients and 4 of 8 inflammatory CAA patients, confirming vasculitis in 7 and 4 patients, respectively. Intravenous methylprednisolone was the predominant induction therapy (63.6%), and cyclophosphamide was the most used adjunctive therapy. Cyclophosphamide, rituximab, azathioprine, and mycophenolate mofetil were utilized as maintenance therapy, often with concurrent prednisone. Patients with inflammatory CAA had a higher tendency for relapse rates than PACNS. This study highlights the variations in patients' characteristics, symptoms, and treatment for CNS vasculitis. Understanding these differences can lead to more efficient diagnostic and management strategies.

Diversity and Surgical Management of Intracranial Fungal Infections.

Intracranial fungal infection is a rare entity. This disease is mainly concentrated in dry and hot climates, such as India, Africa, California, and usually occurs in patients with immune deficiency. Now, we retrospectively analyzed the clinical manifestations, pathologic manifestations, imaging features, surgical methods, and prognosis of 4 patients with fungal infection who were confirmed by postoperative pathology. Intermittent pricking on the right face was presented in 2 patients, headache in 2 patients, orbital apex syndrome in 2 patients, and 1 patient presented with fever. Imaging showed the lesions of all patients were located in the right temporal, including 2 patients involving the right orbital, 1 patient involving the right trigeminal semilunar ganglion, 1 patient involving the right brainstem and tentorium cerebellum, 1 patient involving the right internal carotid artery. Craniotomy was performed in 2 patients, endoscopic biopsy in 1 patient, and stereotactic surgery in 1 patien. Aspergilloma was the most common pathogenic bacteria. One patient relapsed repeatedly and died. Secondary aneurysm complicated with subarachnoid hemorrhage occurred in 1 patient. Therefore, the author confirmed that intracranial fungal infection has diverse clinical, imaging, and pathologic manifestations. Neurosurgeons should be aware of the possibility of intracranial fungal infection when they find abnormal intracranial lesions, neurologic deficits, and inflammation of paranasal sinuses. Combining multiple clinical data may help doctors to improve the accuracy of diagnosis. Individualized and diversified surgical protocols should be selected for diverse lesions. Notably, secondary intracranial fungal vasculitis is common, with high mortality and disability rates.

Secondary Vasculitis Attributable to Post-COVID Syndrome.

While the acute phase of the COVID-19 pandemic has largely come to pass, the chronic physiologic effects of the coronavirus continue to unfold. Specifically, the number of COVID-19-associated vasculitis cases has steadily increased since the onset of the pandemic. Data have shown that vasculitis may develop less than two weeks after COVID-19 or during a later onset of the disease. At this time, research has demonstrated that the novel coronavirus invades more than just the lungs; it can also attack the nervous system, cardiovascular system, and kidneys. In addition, there is a greater understanding of the pathogenesis regarding COVID-19-induced vasculitis via humoral immunity and immune complex disease. Recent case reports have shown an association between COVID-19 and secondary vasculitis. This review paper discusses case reports and data that suggest that COVID-19 may lead to specific vasculitis diseases such as giant cell arteritis, ophthalmic arteritis, aortitis, and Kawasaki-like disease. More research needs to be performed on this association to aid in diagnosis and treatment.

Leprosy mimicking ANCA-associated vasculitis

Vasculitis represents a large group of diseases, classified between primary and secondary. Diagnosis of primary vasculitis is a challenge in medical practice, since there are wide and heterogeneous clinical manifestations, with diagnostic criteria still scarce. Some clinical manifestations are common: constitutional status, myalgia, arthralgia, arthritis, papules, nodules and ulcers. One of the clinical forms is the limited one in which patients with upper respiratory tract involvement often evolve with systemic disease. Most patients have nasal, sinus or ear involvement that may be present weeks or months before other symptoms. Secondary vasculitis can be related to infections, drugs, toxic substances and neoplasms. The virchowian and dimorphic form of leprosy has similar clinical and serological characteristics with rheumatological diseases. As it is an endemic disease in Brazil, there is a description of a wide variety of clinical presentations, so making a differential diagnosis is essential.

VASCULITIS AND ITS VARIOUS PRESENTATIONS: A CASE SERIES

Vasculitis involves a wide spectrum of clinicopathological process with reactive damage to the involved blood vessels. There is loss of vessel integrity instigating haemorrhage & luminal compromise leading to ischemia and necrosis of the tissue supplied by the involved vessels. It may affect varied size and type of blood vessels at different locations. This case series include three cases of vasculitis affecting different organs with varied presentations. Biopsies of three patients with unusual presentations were studied. Their complete history, physical examinations, laboratory investigations including serology were analysed and clinically correlated. The patients presented with different duration of symptoms varying from as short as 1-week days to 6 weeks. Skin lesions were present in two cases. Serology and autoimmune disease markers were negative in all cases except CD56 seen in 10% of plasma in rst case, ANA + was seen in second case and Weakly positive Mi-2 and borderline positive Scl-70 antibodies was seen in third case. Elevated CRP, CRP were seen in all cases. However, pathological features were in concordance with the clinical diagnosis of vasculitis. They were further classied as vasculitis mimicking multiple myeloma, Vasculitis with gastrointestinal manifestation and Henochschonlein purpura. Vasculitis though a rare disease may manifest as an acute or chronic condition. It needs timely diagnosis by clinicopathological examination to aid in further management. It is important to assess the clinical severity in primary and secondary vasculitis, as it determines morbidity and mortality.

Clinical significance of peripheral nervous system involvement in systemic lupus erythematosus patients: Relation to disease activity and damage index

BackgroundDespite its potential impact on disease outcome, peripheral nervous (PN) system involvement in systemic lupus erythematosus (SLE) has received little attention. Aim of the workTo assess the frequency and clinical features of PN system involvement in SLE patients. Patients and methodsThe medical records of 100 patients were revised. Cases with diabetes, hepatitis, vitamin B12 deficiency or carpal tunnel syndrome were excluded. Detailed history, clinical examination and laboratory investigations were recorded. PN events were documented. The SLE disease activity index (SLEDAI) and systemic lupus international collaborating clinics damage index (SLICC-DI) were evaluated. ResultsPatients were 88 females and 12 males (F:M 7.3:1), mean age was 34.2 ± 8.3 years and disease duration 12.7 ± 5.4 years. The frequency of PN system involvement was 8 %; polyneuropathy (n = 5), mononeuritis multiplex (n = 2) and Guillain-Barré syndrome (n = 1). PN was associated with a significantly higher frequency of musculoskeletal (100 %), neuropsychiatric (100 %), pulmonary (62.5 %) and cardiovascular (37.5 %) manifestations as well as Raynaud's phenomenon (37.5 %) and secondary vasculitis (50 %) compared to those without (56.5 %,7.6 %,26.1 %,7.6 %,7.6 % and 12 %;p = 0.02,p < 0.001,p = 0.04,p = 0.03,p = 0.03,p = 0.017 respectively).anti-nuclear antibodies (87.5 % vs 92.4 %) and anti-double stranded DNA (71.4 % vs 69.6 %) positivity were comparable (p > 0.05). SLEDAI and SLICC-DI were significantly increased in patients with PN (26.5 ± 6.36 and 3.75 ± 0.98) compared to those without (11.5 ± 4.76 and 0.61 ± 0.56;p < 0.001 each). ConclusionSLE patients with PN involvement had a higher frequency of musculoskeletal, neuropsychiatric, pulmonary and cardiovascular manifestations in addition to Raynaud's and vasculitis. There is a potential association with disease activity and damage. Careful neurological assessment should be included in the workup of SLE patients.

Isolated Insular Stroke: An Interesting Report of Two Cases.

Isolated Insular Stroke: An Interesting Report of Two Cases.

Abstract WP96: Quantitative Imaging Markers On Vessel Wall Magnetic Resonance Imaging Correlate With Disease Activity And Treatment Response In Cerebral Vasculopathies

Introduction: Little is known about the utility of vessel wall magnetic resonance imaging (VWMRI) in assessing treatment response in various inflammatory and non-inflammatory intracranial vasculopathies, and if there is any correlation of vessel wall enhancement (VWE) with disease activity. We aimed to quantitatively investigate the differences in VWE patterns on follow-up VWMRI in patients with primary CNS vasculitis (PCNSV), secondary CNS vasculitis (SCNSV) and intracranial atherosclerotic disease (ICAD). Methods: VWMRI scans of patients with a confirmed diagnosis of either PCNSV, SCNSV or ICAD presenting to our tertiary care center between January 2015 and January 2021 were reviewed. Inclusion criteria were age greater than 18 years and at least 2 VWMRI scans available, including one from time of first clinical presentation with active disease. For each patient, 20 arterial segments were evaluated on VWMRI for the presence of VWE and the total number of enhancing arterial segments were reported. Percent concentricity, median number of enhancing arterial segments (MEAS) and percent change in MEAS was calculated and compared across all 3 diagnoses. Results: A total of 28 patients met study inclusion criteria (median age 47, 43% male) and had a diagnosis of either PCNSV (n=6), SCNSV (n=7) or ICAD (n=15). Median time to follow-up VWMRI was 22, 20 &amp; 11 weeks for PCNSV, SCNSV &amp; ICAD respectively. On initial VWMRI, the MEAS was 7 for each PCNSV &amp; SCNSV and 6 for ICAD. Higher percent concentricity of VWE was seen more with PCNSV (92.3%) and SCNSV (71.4%) compared to ICAD (22.2%). All (100%) patients with PCNSV and 2 (33%) patients with SCNSV were treated with immunosuppression whereas 3 (50%) patients with SCNSV were treated with antimicrobials. All patients with PCNSV and SCNSV improved clinically whereas those with ICAD progressed or remained stable. The MEAS on follow up VWMRI decreased with treatment in patients with PCNSV (43% decrease) and SCNSV (14% decrease) but increased in those with ICAD (16% increase) and correlated clinically with disease activity and treatment response. Conclusion: Percent concentricity and MEAS on VWMRI may be useful non-invasive markers to monitor disease activity &amp; treatment response in patients with intracranial vasculopathies.

Abstract WP116: Clinical And Diagnostic Features On Cerebrospinal Fluid And Vessel Wall MRI Can Differentiate Primary From Secondary CNS Vasculitis

Introduction: Differentiating primary CNS vasculitis (PCNSV) from secondary CNS vasculitis (SCNSV) can be challenging based on brain imaging and cerebrospinal fluid (CSF) profile alone. Little is known about the diagnostic nuances of differentiating these 2 entities with CSF and vessel wall magnetic resonance imaging (VWMRI) as overlapping features may exist. We aimed to look at clinical features, serum &amp; CSF biomarkers, and VWMRI differences between PCNSV &amp; SCNSV. Methods: Patients presenting to our tertiary care center with a diagnosis of PCNSV or SCNSV between January 2015 and January 2021 were extracted from electronic medical record data. We included patients older than 18 years of age with active disease who had CSF studies and VWMRI data available. Demographic, clinical and diagnostic characteristics were reviewed and descriptive statistics were reported for comparison. Results: A total of 42 patients (24 with PCNSV and 18 with SCNSV) met study inclusion criteria. The SCNSV group was older (median age 43 years vs 41.5 years), had a higher proportion of male gender (78% vs 54%) and black race (22% vs 4%) compared to PCNSV. Patients with PCNSV more commonly had headache (63% vs 50%) and seizures (21% vs 11%) but less commonly had encephalopathy (42% vs 50%), focal neurological deficits (79% vs 83%) and constitutional symptoms (17% vs 33%). Patients with PCNSV more likely had positive antinuclear antigen levels (25% vs 11%) and elevated sedimentation rate (33% vs 25%) but less likely had elevated C-reactive protein (25% vs 33%) in blood. Comparison of CSF studies showed that the PSCNV group had a lower median CSF white blood cell count (10 cells/mm3 vs 43.5 cells/mm3 , both lymphocytic), lower median CSF protein count (62 mg/dl vs 82 mg/dl) and lower IgG index (33% vs 56%) in comparison to the SCNSV group. On VWMRI, PCNSV group had less diffuse (40 vs 54%) involvement and more anterior multifocal lesions (40 vs 15%) compared to SCNSV group with more large or medium-vessel involvement (70 vs 54%). Conclusion: Clinical and diagnostic differences with regards to CSF and VWMRI exist between PCNSV and SCNSV that can aid in differentiating these two entities. This can prove useful especially in undiagnosed systemic vasculitides presenting with CNS vasculitis.

SECONDARY URTICARIAL VASCULITIS AND CHRONIC SPONTANEOUS URTICARIA ASSOCIATED WITH COVID-19 VACCINATION

<h3>Introduction</h3> COVID-19 vaccines are generally safe, however there are associated adverse reactions including delayed cutaneous manifestations. This is a case of secondary urticarial vasculitis and chronic spontaneous urticaria associated with COVID-19 vaccination. <h3>Case Description</h3> A 29-year-old male with allergic rhinitis and dermatographism presented with recurrent urticaria. He received his first dose of COVID-19 vaccine and started to have pruritic urticaria on his abdomen after 5 days. Punch biopsy of the lesion showed dermal hypersensitivity response and findings consistent with secondary urticarial vasculitis. His rash improved with topical steroids and oral antihistamines. After the second vaccine, he immediately developed similar symptoms on his face and body. Rheumatologic and infectious work up were unremarkable, and the concern for systemic vasculitis was low. Secondary urticarial vasculitis and hypersensitivity response related to the vaccine was high on the differential given the temporal association. Due to his recurrent pruritic urticaria refractory to high dose of oral antihistamines, omalizumab was started with significant improvement. He is now doing well off omalizumab and on as needed antihistamines only. <h3>Discussion</h3> There are reports of delayed cutaneous reactions associated with COVID-19 vaccines. Although rare, it is important to recognize these manifestations of urticarial vasculitis and chronic spontaneous urticaria associated with COVID-19 vaccination.

The concise review of etiology, pathology, and treatment strategy of polyarteritis nodosa

Polyarteritis nodosa (PAN) is a necrotizing vasculitis that targets medium-sized muscular arteries and can involve small-sized arteries. The pathogenesis of classic PAN remains unclear, except for secondary PAN or vasculitis that is indistinguishable from PAN due to genetic abnormalities such as deficiency of adenosine deaminase 2. The histopathological characters of PAN change over time from the onset. The type of remission induction therapy to be adopted depends on the disease severity. When it results in remission, corticosteroid dose reduction will begin and will be shifted to remission maintenance therapy.

Case report: the rare clinical picture of vasculitis of the pericardium in rheumatoid arthritis

Summary Background The cause of pericarditis is manifold. It can occur as a result of various diseases but may also be triggered by drugs. However, the data on drug-induced pericarditis are still scarce. Case report A 64-year-old female hypertensive patient with rheumatoid arthritis for 20 years presented with thoracic pain and recurrent pericardial and pleural effusions. For treatment of the recurrent effusions, the patient received glucocorticoids and colchicine in addition to the basic rheumatoid arthritis therapy, and treatment has only recently been expanded to include etanercept. On admission, she complained of malaise, dysphagia, and blood pressure was 85/55 mm Hg. She was normofrequent with elevated inflammatory parameters. On trans-thoracal echocardiography (TTE) and computer-tomography (CT), there was a 3-cm non-floating structure in the entire circumference of the pericardium. The indication for pericardiectomy was given because of hemodynamic impairment. After incision of the pericardium, 250 ml of a brown-reddish fluid drained, with brown crumbly necrotic masses visible underneath. Histopathologic findings revealed vasculitis-related chronic fibrinous pericarditis with vasculitic changes. A subclinical infection with Staphylococcus aureus was detectable by PCR analysis. Conclusion Based on the fact that tumor necrosis factor blockers can induce vasculitis, etanercept might have been responsible for the exacerbation of pericarditis. The underlying rheumatoid arthritis could also be considered as a trigger. The detection of Staphylococcus aureus DNA in the pericardium and the exacerbation of pericarditis could be attributed to secondary vasculitis after an infection with S. aureus, whereas the tendency to infection due to humoral immunodeficiency after years of immunosuppressive therapy has to be discussed as a trigger.

Primary Angiitis of the Central Nervous System - Diagnosis and Management.

Primary angiitis of central nervous system (PACNS) is a rare idiopathic disorder affecting blood vessels of brain, spinal cord, and meninges, consequently leading to infarct and less frequently hemorrhage. CNS vasculitis can also occur as part of systemic vasculitis or secondary to autoimmune diseases or infections. The clinical manifestations of PACNS are non-specific and no single laboratory investigation or neuroimaging finding can reliably diagnose this condition. Histopathological evidence of transmural inflammation of blood vessels of CNS is the gold standard, but is generally pursued subsequent to conventional angiogram (CA) because of its invasive nature. The differentials of PACNS are exhaustive and include systemic vasculitis, secondary vasculitis, non-inflammatory intracranial vasculopathies, demyelination, and neoplasm. These alternative conditions can often be distinguished by history, examination, immunological testing, cerebrospinal fluid analysis, and neuroimaging. CA can detect vasculitic changes in the large to medium cerebral arteries but the specificity is low. Recent advancements in vessel wall imaging techniques have further enabled the distinction of various intracranial vasculopathies from CNS vasculitis. The disease has considerable morbidity and fatality unless timely treatment with immunosuppressive agents is initiated. Induction therapy with glucocorticoids and cyclophosphamide followed by azathioprine, mycophenolate mofetil, or methotrexate as maintenance therapy is the cornerstone of management. Biological agents such as rituximab and anti-tumour necrosis factor alpha inhibitors (infliximab and etanercept) may be used in refractory cases. This review discusses the approach to the diagnosis, determinants of outcome, and management.

Pyoderma Gangrenosum Initially Presenting as an Ulceration of the Ear Lobule

Pyoderma Gangrenosum (PG) was first described in 1916 as “phagedenisme geometrique”, after a French dermatologist observed rapidly progressing, cutaneous necrotic lesions with sharp borders.1 In 1930, Brunsting and his colleagues at the Mayo Clinic coined the term Pyoderma Gangrenosum, because it was initially thought to arise from staphylococcal and streptococcal infections which were observed in 5 of their patients.2 The exact etiology and pathogenesis is still unknown. To date, only a few cases of PG have been shown to affect the ears, all showing no gender or age predilection.3 We report another such case.&#x0D; &#x0D; CASE REPORT&#x0D; A three-year-old girl presented at the emergency room with a non-healing, erythematous papule over her left ear lobule, allegedly following an ear piercing one month prior. She was initially treated at another institution with oral antibiotics. Despite treatment, her mother noted rapid worsening of the lesion, eventually developing into a painful ulceration and affecting the left eyelid as well. At the time of examination, the patient presented with a painful, necrotic plaque around the left eyelid with serpiginous borders (Figure 1) and ear lobule with erythematous, advancing borders (Figure 2A, B). There were no systemic co-morbidities noted. The working diagnosis then was necrotizing fasciitis and she was immediately started on systemic intravenous antibiotics which she did not respond to. Laboratory tests showed elevated CRP, but procalcitonin, C-ANCA and ANA were all normal. Tissue cultures of both eyelid and earlobe, as well as blood cultures, revealed no growth. Wedge biopsy of the eyelid ulceration revealed neutrophilic dermatitis. Biopsy of the ear lobule revealed suppurative granulomatous dermatitis with secondary leucocytoclastic vasculitis. Further workups for infection and possible systemic diseases were all unremarkable. A pathergy test was negative. A diagnosis of pyoderma gangrenosum was made after excluding systemic and infectious causes. The patient was started on systemic prednisone at a dose of 1mg/kg/day which she slowly responded to. Surgical reconstruction of the earlobe was to be planned once the ulceration completely healed; unfortunately, this patient was lost to follow-up

Гастроинтестинальные проявления системных васкулитов: спектр клинических симптомов, диагностика и лечение

Системные васкулиты представляют собой гетерогенную группу заболеваний, которые характеризуются воспалением стенок кровеносных сосудов и классифицируются в зависимости от типа и размера вовлеченных в патологический процесс сосудов. Гастроинтестинальная система может быть вовлечена в патологический процесс при первичных и вторичных васкулитах. Поражение желудочно-кишечного тракта (ЖКТ) чаще всего встречается при IgA-васкулите, АНЦА-ассоциированных васкулитах, узелковом полиартериите и синдроме Бехчета. Исходами воспалительного поражения сосудов являются аневризмы или окклюзии в брыжеечных, печеночных или селезеночных сосудах с развитием воспаления, эрозий и/или язв, которые могут привести к перфорации, кровотечению и непроходимости желудка и кишечника и быть важной причиной заболеваемости и смертности у пациентов с васкулитами. Клинические признаки поражения ЖКТ при системных васкулитах обычно неспецифичны, к ним относятся лихорадка, боль в животе, тошнота, рвота, диарея и кровотечение. Диагностика базируется главным образом на визуализации при помощи катетерной ангиографии, компьютерной томографической и/или магнитно-резонансной ангиографии, эндоскопии с проведением гистопатологического исследования. Лечение поражения ЖКТ зависит от типа васкулита и обычно включает применение высоких доз глюкокортикоидов, иммунодепрессантов, таких как циклофосфамид, азатиоприн или микофенолата мофетил, и биологических препаратов. В данном обзоре литературы представлены данные о патофизиологии, клинических данных, диагностике, лечении и исходе поражения ЖКТ при различных типах системных васкулитов.