Manifestations Of Vasculitis Research Articles

Targeting the interleukin-5 pathway in EGPA: evidence, uncertainties and opportunities

Only a minority of patients with eosinophilic granulomatosis with polyangiitis (EGPA) can be weaned-off glucocorticoids (GC) using conventional treatment strategies. The development of biological agents specifically inhibiting the IL-5 pathway...

Аутоиммунитет, аутовоспаление и почки

Systemic autoimmune and autoinflammatory disease are associated with all types of diffuse kidney disease, that is, glomerulonephritis (including rapidly progressive), thrombotic microangiopathy, tubulointerstitial nephritis, and AA-amyloidosis. However, the occurrence and types of nephropathies differ significantly in patients with various systemic immunemediated inflammatory diseases. For example, glomerulonephritis is one of the leading manifestation of systemic lupus erythematosus and small-vessels vasculitis, thrombotic angiopathy occurs frequently in patients with both primary and secondary antiphosholipid syndrome, tubulointerstitial nephritis is frequently found in patients with Sjogren syndrome and IgG4-associated disease, whereas AA-amyloidosis can complicate rheumatoid arthritis, ankylosing spondilytis, psoriatic arthritis, familial Meditarranean fever, and certain other monogenic autoinflammatory diseases. Over last decades, renal survival in patients with autoimmune and autoinflammatory diseases improved significantly due to earlier diagnosis and development of effective immunosuppressive and anti-inflammatory treatments. However, a proportion of patients still present with progressive impairment of kidney function that can be unrelated to activity of underlying diseases.

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.

Cutaneous Small Vessel Vasculitis: A Practical Guide to Diagnosis and Management.

Cutaneous vasculitis may be a cutaneous manifestation of systemic vasculitis, a skin-limited variant of systemic vasculitis, or a type of single-organ vasculitis limited to the skin. Careful evaluation is necessary to distinguish these possibilities, differentiate skin-limited from systemic disease, and identify important underlying conditions, if present. Appropriate management depends on disease type and severity. In the case of cutaneous small vessel vasculitis (CSVV), treatment is primarily based on case reports and expert opinion, using a range of immunomodulatory or immunosuppressive therapies. This manuscript presents a systematic and stepwise approach to the evaluation of patients presenting with cutaneous vasculitis, as well as the management of those diagnosed with CSVV, using best available evidence. Studies relevant to the classification, evaluation, and management of skin vasculitis are ongoing.

CARDIAC MANIFESTATIONS OF EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS

SESSION TITLE: Airways Function Measurement and Treatment OutcomesSESSION TYPE: Rapid Fire Original InvPRESENTED ON: 10/19/2022 11:15 am - 12:15 pmPURPOSE: Eosinophilic Granulomatosis with Polyangiitis (EGPA) is a rare systemic disease characterized by asthma, peripheral & tissue eosinophilia & systemic vasculitis. Cardiovascular manifestations have been reported in prior studies, but the prevalence & spectrum of cardiac and vascular disease in EGPA is poorly understood.METHODS: The medical records were reviewed of a series of patients with EGPA who were evaluated from 1990-2021. For inclusion, patients had to fulfill standardized criteria for EGPA. Cardiac involvement was attributed to EGPA if findings could not be explained by other coexisting conditions.RESULTS: Two hundred and nineteen patients with EGPA were identified. The mean age at diagnosis was 52.16 years. 108 (49.3%) were female. Eighty-three (38%) had evidence of cardiac involvement. 49 (22.4%) had myocardial dysfunction. 6 (3%) with intra-cavitary cardiac thrombus; 22 (10%) with valvular involvement. 42 (19.2%) had pericardial disease (8 with tamponade and 1 with chronic constrictive pericarditis). Myocardial & pericardial involvement was also associated with greater degrees of peripheral eosinophilia -myocardial: 47, 18% vs no myocardial: 34, 20% of total WCC; p=0.0002 & pericardial: 47, 16% vs no pericardial 34, 20% of total WCC; p=0.0004. Patients with myocardial involvement with EGPA were less likely to be ANCA positive (24.5% versus 48.3%; p=0.002). ANCA positivity in those with pericardial involvement was 31.0% versus those without pericardial involvement 46.0%, p=0.077). Patients with myocardial involvement were less likely to have peripheral nerve involvement (a vasculitic manifestation) (52.1%) than those without cardiac involvement (77.7%; p=0.0008) but more likely to have pulmonary infiltrates/eosinophilic pneumonia (likely an eosinophilic manifestation) (70.8%) versus (49.4; p=0.0008). Cardiac involvement was associated with lower all-cause mortality over 10 years of follow-up (Hazard ratio 0.38 (0.15-0.98); p=0.03).CONCLUSIONS: In a large single-center series, both myocardial and pericardial involvement was common in EGPA and was associated with higher degrees of peripheral eosinophilia and the prevalence of eosinophilic pneumonia. A positive ANCA and peripheral nerve involvement were less common in those with myocardial and pericardial involvement.CLINICAL IMPLICATIONS: These findings may have implications for diagnosis, management including targeted treatment strategies of EGPA patients with cardiac involvement.DISCLOSURES: no disclosure on file for alvise berti;No relevant relationships by Garvan Kanepharmaceutical drug trial site invest. relationship with GSK Please note: $1-$1000 by Karina Keogh, value=Travelmonograph relationship with BMJ Please note: $1-$1000 by Karina Keogh, value=Honoraria SESSION TITLE: Airways Function Measurement and Treatment Outcomes SESSION TYPE: Rapid Fire Original Inv PRESENTED ON: 10/19/2022 11:15 am - 12:15 pm PURPOSE: Eosinophilic Granulomatosis with Polyangiitis (EGPA) is a rare systemic disease characterized by asthma, peripheral & tissue eosinophilia & systemic vasculitis. Cardiovascular manifestations have been reported in prior studies, but the prevalence & spectrum of cardiac and vascular disease in EGPA is poorly understood. METHODS: The medical records were reviewed of a series of patients with EGPA who were evaluated from 1990-2021. For inclusion, patients had to fulfill standardized criteria for EGPA. Cardiac involvement was attributed to EGPA if findings could not be explained by other coexisting conditions. RESULTS: Two hundred and nineteen patients with EGPA were identified. The mean age at diagnosis was 52.16 years. 108 (49.3%) were female. Eighty-three (38%) had evidence of cardiac involvement. 49 (22.4%) had myocardial dysfunction. 6 (3%) with intra-cavitary cardiac thrombus; 22 (10%) with valvular involvement. 42 (19.2%) had pericardial disease (8 with tamponade and 1 with chronic constrictive pericarditis). Myocardial & pericardial involvement was also associated with greater degrees of peripheral eosinophilia -myocardial: 47, 18% vs no myocardial: 34, 20% of total WCC; p=0.0002 & pericardial: 47, 16% vs no pericardial 34, 20% of total WCC; p=0.0004. Patients with myocardial involvement with EGPA were less likely to be ANCA positive (24.5% versus 48.3%; p=0.002). ANCA positivity in those with pericardial involvement was 31.0% versus those without pericardial involvement 46.0%, p=0.077). Patients with myocardial involvement were less likely to have peripheral nerve involvement (a vasculitic manifestation) (52.1%) than those without cardiac involvement (77.7%; p=0.0008) but more likely to have pulmonary infiltrates/eosinophilic pneumonia (likely an eosinophilic manifestation) (70.8%) versus (49.4; p=0.0008). Cardiac involvement was associated with lower all-cause mortality over 10 years of follow-up (Hazard ratio 0.38 (0.15-0.98); p=0.03). CONCLUSIONS: In a large single-center series, both myocardial and pericardial involvement was common in EGPA and was associated with higher degrees of peripheral eosinophilia and the prevalence of eosinophilic pneumonia. A positive ANCA and peripheral nerve involvement were less common in those with myocardial and pericardial involvement. CLINICAL IMPLICATIONS: These findings may have implications for diagnosis, management including targeted treatment strategies of EGPA patients with cardiac involvement. DISCLOSURES: no disclosure on file for alvise berti; No relevant relationships by Garvan Kane pharmaceutical drug trial site invest. relationship with GSK Please note: $1-$1000 by Karina Keogh, value=Travel monograph relationship with BMJ Please note: $1-$1000 by Karina Keogh, value=Honoraria

OA06 A complex case of thigh pain in Granulomatosis with Polyangiitis (GPA)

OA06 A complex case of thigh pain in Granulomatosis with Polyangiitis (GPA)

Vascular and Vasogenic Manifestations of Systemic ANCA-Associated Vasculitis with Renal Involvement in Non-Contrast Brain MRI in Patients with Acute Disease Onset.

Background. Data concerning central nervous system (CNS) alterations in ANCA-associated vasculitis with renal involvement (AAVR) are sparse. The study aimed to assess vascular and vasogenic brain alterations in patients with acute onset of AAVR and the applicability of non-contrast magnetic resonance imaging (MRI) techniques in this diagnosis. Methods. Thirty-eight patients with acute onset of AAVR were included in the study. BVAS/WG, c-ANCA, p-ANCA, renal function and perfusion, neurological assessment, and brain MRI were performed. Results. Cerebral vascular alternating narrowing and dilatation (VAND) was detected in 42.1% of patients, and the black-blood was significantly more diagnostic than the TOF technique (p < 0.001). VAND occurrence was independently associated with the concentration of p-ANCA. The vasogenic white matter lesions (VWML) were found in 94.4% of patients, and in their detection, SWAN was significantly better than the FLAIR technique (p = 0.002). The number of VWML correlated with age and cranial nerve damage. Hemosiderin deposits were found in 21.6% of patients and were associated with a gait impairment and paresthesia. Conclusions. Vascular and vasogenic alterations in the CNS are frequent in patients with acute onset of systemic ANCA-associated vasculitis with renal involvement. Non-contrast MRI is useful in the diagnosis of brain vasculitis.

Vaskulitiden im Grenzbereich von Leber- und Darmerkrankungen

BackgroundVasculitides can involve several different organ systems. Gastrointestinal manifestations regularly occur in some systemic vasculitides and require specific treatment. Hepatitis B and C virus infections can trigger certain forms of vasculitis.ObjectiveThis review highlights and discusses the interdisciplinary challenges in the diagnosis and treatment of vasculitides.ConclusionGastrointestinal lesions as a result of antineutrophil cytoplasmic antibody (ANCA) vasculitis particularly require a consistent immunosuppressive treatment and infectious complications, such as cytomegalovirus (CMV) colitis need to be distinguished from vasculitic manifestations. Some forms of vasculitis (cryoglobulinemic vasculitis and polyarteritis nodosa) can be caused by a hepatitis B or C virus infection. Patients with cryoglobulinemic vasculitis can benefit from a combination treatment consisting of antiviral treatment and B‑cell depletion.

Radiosensitivity in patients affected by ARPC1B deficiency: a new disease trait?

Actin-related protein 2/3 complex subunit 1B (ARPC1B) deficiency is a recently described inborn error of immunity (IEI) presenting with combined immunodeficiency and characterized by recurrent infections and thrombocytopenia. Manifestations of immune dysregulation, including colitis, vasculitis, and severe dermatitis, associated with eosinophilia, hyper-IgA, and hyper-IgE are also described in ARPC1B-deficient patients. To date, hematopoietic stem cell transplantation seems to be the only curative option for patients. ARPC1B is part of the actin-related protein 2/3 complex (Arp2/3) and cooperates with the Wiskott–Aldrich syndrome protein (WASp) in the regulation of the actin cytoskeleton remodeling and in driving double-strand break clustering for homology-directed repair. In this study, we aimed to investigate radiosensitivity (RS) in ARPC1B-deficient patients to assess whether it can be considered an additional disease trait. First, we performed trio-based next-generation-sequencing studies to obtain the ARPC1B molecular diagnosis in our index case characterized by increased RS, and then we confirmed, using three different methods, an increment of radiosensitivity in all enrolled ARPC1B-deficient patients. In particular, higher levels of chromatid-type aberrations and γH2AX foci, with an increased number of cells arrested in the G2/M-phase of the cell cycle, were found in patients’ cells after ionizing radiation exposition and radiomimetic bleomycin treatment. Overall, our data suggest increased radiosensitivity as an additional trait in ARPC1B deficiency and support the necessity to investigate this feature in ARPC1B patients as well as in other IEI with cytoskeleton defects to address specific clinical follow-up and optimize therapeutic interventions.

Anti-Inflammatory and/or Anti-Fibrotic Treatment of MPO-ANCA-Positive Interstitial Lung Disease: A Short Review.

The presence of a lung lesion is common in microscopic polyangiitis (MPA), and interstitial lung disease (ILD) can lead to a poor prognosis. Although myeloperoxidase antineutrophil cytoplasmic antibodies (MPO-ANCA) are often present in patients with MPA, patients with ILD and MPO-ANCA positivity but without other manifestations of systemic vasculitis have also been reported. Therefore, the possible association between MPO-ANCA, MPA, and idiopathic ILD remains unclear. This problematic matter has influenced the treatment strategy of MPO-ANCA-positive ILD patients without systemic vasculitis. Clinicians should undertake treatment with careful consideration of the four major causes of death in MPO-ANCA-positive ILD: acute exacerbation of ILD, progressive lung fibrosis, infectious comorbidities, and diffuse alveolar hemorrhage. Further, clinicians need to carefully judge whether inflammation or fibrosis is the dominant condition with reference to the patient’s clinical domain and radiopathological lung features. Recently, anti-fibrotic agents such as nintedanib and pirfenidone were shown to be effective in treating various etiologies associated with ILD and have thus led to the widening of treatment options. In this review, the clinical characteristics, radiopathology, prognosis, and therapeutic options in patients with MPO-ANCA-positive ILD are summarized using limited information from previous studies.

Wegener's granulomatosis mimicking like pulmonary tuberculosis and presenting as cavitating lung disease with mycetoma

Pulmonary tuberculosis (TB) is the most common cause of bilateral pulmonary cavities with constitutional symptoms in India being endemic and more prevalent nature of the disease, irrespective of microscopy or nucleic acid amplification test abnormalities. Pulmonary manifestations of systemic vasculitis have very diverse involvement ranging from nodule, consolidation, and cavitation. In this case report, a 45-year-old female, presented with constitutional symptoms with lung parenchymal consolidations progressed to cavitation, and started empirical anti-TB treatment without mycobacterial microscopic or genome documentation in sputum with clinical or radiological worsening. Bronchoscopy workup is inconclusive and the tropical screen for bacterial, TB, and malignancy was negative and fungal yield Aspergillus colonization. A vasculitis workup was done in the presence of clinical and radiological worsening documented PR3-antineutrophil cytoplasmic antibody positive with very highly raised titers. We have started on steroids and cyclophosphamide with antifungals and clinical response was documented with a near-complete resolution of shadows in 24 weeks.

Bronchus sign on HRCT thorax

Tuberculosis is the most common diagnosis in India in presence of constitutional symptoms such as cough, fever, and weight loss with lung parenchymal abnormality irrespective of microscopy or nucleic acid amplification test abnormalities in high TB prevalent tropical settings. Pulmonary manifestations of systemic vasculitis have very diverse involvement ranging from the nodule to consolidation. Bronchus sign is classically described in lung malignancies than Wegener disease. The acino-nodular pattern is classical of pulmonary tuberculosis, sometimes documented in fungal infections. In this case report, a 45-year-old female, presented with constitutional symptoms with lung parenchymal nodules, without mycobacterial microscopic or genome documentation, received empirical antituberculosis treatment with the progression of the disease without clinical or radiological response. Bronchoscopy workup is inconclusive and tropical screen for bacterial, fungal, TB, and malignancy was negative. Vasculitis workup was done in view of the presence of persistent fever and documented PR3-ANCA positive with very highly raised titers. We have started on steroid and cyclophosphamide and clinical response documented with near-complete resolution of shadows in 12 weeks. Pulmonary manifestations of Wegener disease are rare and underestimated and early pickup of the entity in course of illness will have a good outcome with an excellent prognosis.

Complex case of granulomatosis with polyangiitis involving the urethra and prostate causing outflow obstruction and ano-urethral fistula

Granulomatosis with polyangiitis (GPA) is an autoimmune small to medium vessel inflammatory vasculitis that can affect multiple organ systems, with predominantly pulmonary, renal and musculoskeletal manifestations. Rarely, there have been cases involving the genitourinary system, particularly causing urethritis and prostatitis. In some incidences, this may mimic prostate abscess or malignancy. We present a case of a 36-year-old man with refractory urinary retention secondary to prostate abscess due to GPA. This case necessitated transurethral resection of the prostate (TURP) and supra-pubic catheterisation, later complicated by a peri-anal abscess and severe urethritis. A potential recto-urethral fistula was noted on magnetic resonance imaging (MRI) and cystourethroscopy, which was managed conservatively with long-term catheter drainage until resolution. The patient ultimately displayed many severe clinical manifestations of a systemic vasculitis and this case report emphasises the importance of considering autoimmune vasculitis conditions as a differential diagnosis when managing inflammatory genitourinary conditions. This case report highlights the importance of obtaining sufficient tissue to make a timely diagnosis in order to initiate immunosuppressant therapy and disease-modifying anti-rheumatic drugs (DMARDs).

LEUCOCYTOCLASTIC VASCULITIS AFTER COVID-19 INFECTION IN CHILDREN

We are presenting the case of purpuric rush in 5-year-old girl, who had asymptomatic Covid 19 infection prior to clinical manifestation of vasculitis. This emphasizes that vasculitis could be the manifestation of Covid-19 in acute phase and 4-6 weeks after the infection. Children with vasculitis should be tested for the association with Covid-19.

POS0246 SEQUENTIAL RITUXIMAB AND MEPOLIZUMAB IN EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS

BackgroundRituximab (RTX) is an effective remission-induction treatment in ANCA-associated vasculitides (AAVs). Some reports have suggested that it might be effective also in Eosinophilic Granulomatosis with Polyangiitis (EGPA), to induce and maintain remission of vasculitic manifestations [1,2]. However, its effects for preventing respiratory relapses seem to be poor. Mepolizumab (Mepo) (both 100 and 300mg/month) is effective in improving respiratory manifestations and lung function, while partially controlling also systemic activity [3,4]. Isolated case reports further indicate that the sequential therapy with RTX and Mepo might be effective [5-7].ObjectivesThe study aimed to investigate the efficacy and safety of a therapeutic regimen based on sequential RTX and Mepo for the control of EGPA.MethodsA multicenter, retrospective, cohort study was conducted on adult patients diagnosed with EGPA according to the ACR classification criteria [8] or MIRRA trial criteria [3]. Only patients who received induction therapy with RTX (any dosage), and subsequent treatment with Mepo (100-300 mg/4 weeks) within 12 months from last RTX administration were included. Patients receiving other induction therapies between RTX and Mepo were excluded. The effectiveness of sequential RTX and Mepo was assessed in terms of disease activity (by the Birmingham Vasculitis Activity Score, BVAS) and daily corticosteroid dosage. Safety data were also collected.ResultsThirty-four EGPA patients treated with sequential RTX and Mepo were included (59% females, median age of 51 years (IQR 40-58); 41% ANCA positive).In most cases (26/34; 76%), RTX was started at the dosage of 1g q2w, and all except two patients had active disease at time of RTX beginning [median BVAS of 9 (IQR 6-14)]. Specifically, most patients started RTX for the control of systemic manifestations (19/34; 56%), or of both systemic and respiratory symptoms (11/34; 32%). All except one patient were receiving oral corticosteroids, at a median dosage of 25 mg/day (10-38).Mepo was started after a median of 14 months (6-23) from RTX initiation and after a median of 5 months (IQR 3-11) from the last RTX administration. Mepo was used at the dosage of 100mg/4 weeks in 32/34 (94%), mostly for the control of respiratory manifestations (25/34, 74%). At the time of starting Mepo, the median BVAS was 4 (2-8), and median prednisolone dose 10 mg/day (7-15). After a median follow-up of 28 months (IQR 23-33) from starting Mepo, the median BVAS decreased to 1.5 (IQR 0-4) and the median corticosteroid dosage to 5 mg/day (2.5-5), with 7/34 (21%) patients being off steroids. At last follow-up, most patients were off-RTX (28/34), typically due to stable disease remission (20/34; 59%).Both RTX and Mepo were well-tolerated; 5 patients had adverse events on RTX (none serious), and 5 on Mepo (including one serious infection).ConclusionSequential use of RTX and Mepo seems to be effective for remission induction and maintenance in EGPA.

Cutaneous manifestations of ANCA-associated vasculitis: a retrospective review of 211 cases with emphasis on clinicopathologic correlation and ANCA status.

ANCA-associated vasculitis (AAV) may present a wide array of dermatological manifestations. Patients may remain ANCA negative, rendering diagnosis challenging for dermatologists if they depend heavily on ANCA testing to either confirm or rule out AAV. To compare clinical and histopathological features of AAV patients with skin lesions who are ANCA positive versus those who are ANCA negative. Retrospective review of medical charts to identify patients diagnosed with AAV by clinical and pathologic criteria who also had cutaneous manifestations. Search revealed that 211 out of 932 (23%) patients had cutaneous manifestations. Of those, 40/211 (20%) patients had persistently ANCA-negative serology. Eosinophilic granulomatosis with polyangiitis (EGPA) comprised the largest cohort. Palpable purpura was the most prominent clinical feature. The most common histopathological feature was leukocytoclastic vasculitis (LCV) in 19 (29%) specimens, extravascular granuloma in 14 (22%), followed by perivascular infiltrate in 12 (18%), with eosinophils in nine. In the ANCA-negative subgroup, perivascular infiltrate was more common followed by LCV but without statistically significant difference. Diagnosis of AAV should not be based on ANCA testing alone since a considerable number of patients with cutaneous lesions may be ANCA negative. The clinical or histopathologic findings of skin lesions in this study group did not vary based on ANCA status.

Therapeutic advances in eosinophilic granulomatosis with polyangiitis.

In recent years, therapeutic advances in eosinophilic granulomatosis with polyangiitis (EGPA) have changed our treatment paradigm. This review will summarize and discuss updates in management of EGPA, with a particular focus on biologic therapies. The anti-interleukin (IL)-5 agent mepolizumab (the first FDA-approved drug specifically for EGPA) is effective in induction and maintenance of remission particularly in patients with predominantly asthma and allergic manifestations, though efficacy in ANCA-positive, vasculitic disease is unclear; additional anti-IL-5 agents are under study. Rituximab is currently recommended for remission induction in severe disease, particularly in ANCA-positive patients with vasculitic manifestations, though the supportive evidence is mostly observational. Evidence supporting use of traditional DMARDs and other biologic agents such as omalizumab remains limited and observational. Although management of this heterogeneous disease remains challenging and unanswered questions remain, advances in biologics (particularly anti-IL-5 agents and an evolving interest in rituximab) have expanded our treatment armamentarium in EGPA.

Extrarenal manifestations of systemic vasculitis in children

The observation data of 22 children with systemic vasculitis (SV) and morphologically verified nephritis is presented. Emphasis was put on extrarenal manifestations of SV in childhood, analysis of clinical, laboratory, immunological, morphological changes and factors influencing on the course and prognosis. It has been established that SV often manifests with general non-specific constitutional symptoms, involvement of the ENT and organs of vision, is characterized by multiple organ and generalized lesions, in 77% of cases in children is associated with anti-neutrophil cytoplasmic antibodies (ANCA). The presence of antibodies to myeloperoxidase (MPO) is noted in most patients with lesions of the skin, joints, lungs, and nervous system and is associated with an unfavorable renal prognosis. ENT and organs of vision, gastrointestinal tract involvement is equally often observed in both MPO and PR3 antibody positive.

Identification of 4 subgroups in juvenile dermatomyositis by principal component analysis-based cluster analysis.

Juvenile dermatomyositis (JDM) is an autoimmune disease characterised by a great heterogeneity in its clinical manifestations. In this study, we aimed to investigate the association between different clinical subtypes, laboratory data, and myositis antibodies of JDM. A total of 132 JDM patients were enrolled and their medical records were retrospectively reviewed and autoantibodies tested. Twenty-one variables, including clinical manifestations and laboratory findings, were selected for analysis. We selected principal component analysis (PCA) as a pre-processing method for cluster analysis to convert the 21 original variables into independent principal components. We then conducted a PCA-based cluster analysis in order to analyse the association between patient clusters and the clinical data, laboratory data, and myositis autoantibodies. We identified 4 distinct JDM subgroups by PCA-based cluster analysis, namely: cluster A, JDM patients with arthralgia and intense inflammation; cluster B, JDM patients with clinical manifestations of vasculitis; cluster C, hypermyopathic JDM patients; and cluster D, JDM patients with skin involvement. There were significant differences between the 4 groups in serum alkaline phosphatase levels, usage of aggressive immunosuppressive therapy, and autoantibody expression of anti-mi2, anti-MDA5, anti-Jo1, and anti-PM-Scl100. We conducted cluster analysis of a cohort of JDM patients and identified 4 subgroups that represented diverse characteristics in the distribution of laboratory data and myositis autoantibodies, indicating that multidimensional assessment of clinical manifestations is highly valuable and urgently needed in JDM patients. These subgroups may contribute to individualised treatments and improved JDM patient prognosis.

Clinical Manifestations of Leukocytoclastic Vasculitis, Treatment, and Outcome in Patients with Ulcerative Colitis: A Systematic Review of the Literature.

Leukocytoclastic vasculitis (LCV) is a rare extraintestinal manifestation (EIM) of ulcerative colitis (UC). Observations about its association with UC stem from case reports and small case series. Due to its rarity, more rigorous cross-sectional studies are scarce and difficult to conduct. The aim of this systematic review was to synthetize the knowledge on this association by reviewing published literature in the form of both case reports and case series; and report the findings according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. In contrast to LCV in Chron disease (CD), which occurs secondary to biologic therapies used for its treatment, LCV in UC is a true reactive skin manifestation. Both genders are equally affected. Palpable purpura (41%) and erythematous plaques (27%) are the most common clinical manifestations. In 41% of patients, the rash is painful, and the lower extremities are most commonly involved (73%). Systemic symptoms such as fever, arthralgias, fatigue, and malaise are seen in 60% of patients. Unlike previous reports, we found that LCV more commonly occurs after the UC diagnosis (59%), and 68% of patients have active intestinal disease at the time of LCV diagnosis. Antineutrophil cytoplasmic antibody (ANCA) is positive in 41% of patients, and 36% of patients have other EIMs present concomitantly with LCV. The majority of patients were treated with corticosteroids (77%), and two (10%) required colectomy to control UC and LCV symptoms. Aside from one patient who died from unrelated causes, all others survived with their rash typically resolving without scarring (82%).