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Purpose of Review: This review aims to summarize the evolution and recent developments in the classification of ANCA associated vasculitis (AAV) and to summarize evaluations of the 2022 ACR/EULAR classification criteria of AAV in several cohorts. Recent Findings: The classification of AAV has been a field of controversy for some time. The parallel existence of classification criteria and disease definitions produced some overlap in classification, leading to challenges when comparing different cohorts. The 2022 ACR/EULAR classification criteria derived from the largest study ever conducted in vasculitis account for significant changes in vasculitis classification with the integration of ANCA and modern imaging. These criteria show good performance compared to previous ones but also raise questions as ANCA serotypes have substantial impact on classification. In addition, there are some discrepancies with earlier agreed histopathological features of AAV disease phenotypes. Summary: During the last 35 years, several sets of classification criteria have evolved to facilitate epidemiologic studies and clinical trials in AAV. While some of these criteria have been in use for many years, they were criticized due to either not using ANCA or not integrating surrogate markers for vasculitis but also due to overlapping when used in parallel. The long-awaited new ACR/EULAR criteria for AAV were published in 2022 and are the result of a large international study, introducing for the first time ANCA and modern imaging in the classification of AAV. Though the criteria show good performance, they bring several other challenges with practical application. [ABSTRACT FROM AUTHOR]

Pulmonary vasculitis identifies a heterogeneous group of diseases characterized by inflammation, damage and necrosis of the wall of pulmonary vessels. The most common approach to classify vasculitis is according to etiology, therefore dividing them into primary and secondary, with a further sub-classification of primary vasculitis based on the size of the affected vessels (large, medium, and small). Pulmonary involvement is frequently observed in patients with systemic vasculitis and radiological presentation is not pathognomonic, but may vary between diseases. The main findings using high-resolution computed tomography (HRCT) include small vessel wall thickening, nodular lesions, cavitary lesions, reticular opacities, ground-glass opacities (GGO), consolidations, interlobular septal thickening, tracheobronchial stenosis, and aneurysmal dilatation of pulmonary arteries, with or without pleural effusion. Radiological diagnosis alone is difficult since signs and symptoms of lung vessel involvement are often non-specific and might overlap with other conditions such as infections, connective tissue diseases and neoplasms. Therefore, the aim of this review is to describe the most common radiological features of lung involvement in pulmonary vasculitis so that, alongside detailed clinical history and laboratory tests, a prompt diagnosis can be performed. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Although the concurrent occurrence of vasculitis with AS is uncommon, when patients diagnosed with AS exhibit symptoms including skin petechiae, purpura, abdominal discomfort, malaise, elevated ESR, and reduced complement levels, vigilant monitoring for vasculitis is advisable following the exclusion of secondary vasculitis triggers such as malignancies, infections, and pharmaceutical agents. The primary characteristic of ankylosing spondylitis (AS) involves inflammation occurring within the sacroiliac joint and the spine, leading to destruction and eventual ankylosis. A notably infrequent complication associated with AS is vasculitis, with limited reports linking AS to vasculitis. This case study documents a 48‐year‐old male, diagnosed with HLA‐B27‐positive AS for the past 15 years, who developed abdominal pain and skin lesions following the cessation of his medication on his own. Subsequent clinical evaluations identified leukocytoclastic vasculitis (LCV) related to AS after excluding all other potential causes of LCV, including drug‐related sources, cancer, hepatitis B and C viruses, Henoch‐Schönlein purpura (HSP), and IgA nephropathy. [ABSTRACT FROM AUTHOR]

This study investigated the possible roles of renal estrogen receptors (ER) in glomerulonephritis associated with small vessel vasculitis. The relationships of ERs were investigated in antineutrophilic cytoplasmic antibody (ANCA)‐associated glomerulonephritis and immunoglobulin A (IgA) nephropathy groups, which are small vessel vasculitis subtypes with two different glomerulonephritis development pathophysiologies. The design of this study was prepared as a retrospective cohort study. The study included 42 patients with ANCA‐associated vasculitis and 18 with IgA nephropathy in the small vessel vasculitis group. For the control group, intact renal tissues of 28 patients who underwent nephrectomy due to renal cell carcinoma were used. Renal biopsy samples of the groups were stained with ER beta (ß) and ER alpha (α). Tubular ER ß expression score (TERßES) median values were found to be significantly higher in ANCA‐ associated vasculitis (B = 0.724, OR [95%CI]: 2.064 [1.141–3.731], p =.016) and IgA nephropathy (B = 0.898, OR [95%CI]: 2.454 [1.307–4.609], p =.005) than in intact kidney tissue. It was determined that tubular ERß was most frequently localized in the distal tubule at 57.9% and the second most common in the proximal tubule at 20.4%. The expression of tubular ERß is increased in glomerulonephritis due to small vessel vasculitis. Tubular ERßs are most commonly localized in the distal tubule. Further studies are needed to understand the physiological and pathophysiological effects of altered renal ER levels in small vessel vasculitis. [ABSTRACT FROM AUTHOR]

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Abstract Background Childhood-onset ANCA-associated vasculitides (AAV) are characterized by necrotizing inflammation and include granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). Pediatric data is scare and there have been no prior studies examining the characteristics of pediatric AAV in Central California. Methods This retrospective study comprised AAV patients ≤18 years of age, diagnosed between 2010 and 2021, in Central California. We analyzed initial presentation including demographics, clinical, laboratory characteristics, treatment, and initial outcomes. Results Of 21 patients with AAV, 12 were categorized as MPA and 9 with GPA. Median age at diagnosis was 13.7 years in MPA cohort and 14 years in GPA. MPA cohort were majority females (92% versus 44%). 57% of the cohort were racial/ethnic minority including Hispanics (n = 9), Asians (n = 2), multiracial (n = 1) and 43% were white (n = 9). MPA patients were more frequently Hispanic (67%), meanwhile GPA patients were frequently white (78%). Median duration of symptoms prior to diagnosis was 14 days in MPA cohort and 21 days in GPA cohort. Renal involvement was frequent (100% in MPA and 78% in GPA). GPA cohort had frequent ear, nose and throat (ENT) involvement (89%). All patients were ANCA positive. All Hispanic patients were MPO positive, meanwhile 89% of white patients were PR3 positive. MPA cohort tended towards more severe disease with 67% requiring ICU admission and 50% requiring dialysis. Two deaths were reported in MPA cohort, related to Aspergillus pneumonia and pulmonary hemorrhage. In MPA cohort, 42% received cyclophosphamide in combination with steroids and 42% received rituximab in combination with steroids. GPA patients received cyclophosphamide, either with steroids alone (78%) or in combination with steroids and rituximab (22%). Conclusions Microscopic polyangiitis was the most frequent AAV subtype with female preponderance, shorter duration of symptoms at onset and higher proportion of racial/ ethnic minority patients. Hispanic children demonstrated frequent MPO positivity. Trends towards higher rates of ICU requirement and need for dialysis upon initial presentation was noted in MPA. Patients with MPA received rituximab more frequently. Future prospective studies are needed to understand differences in presentation and outcomes in childhood onset AAV between diverse racial-ethnic groups.

Background: Hepatitis B virus (HBV) infection affects millions worldwide, predominantly targeting the liver, leading to conditions like cirrhosis and hepatocellular cancer. However, HBV can also cause extrahepatic complications, including autoimmune-mediated vasculitis. Small vessel vasculitis, often associated with antineutrophil cytoplasmic antibodies (ANCA), can occur in chronic infections like HBV. Case presentation: A 46-year-old man with chronic hepatitis B presented with high fever, diarrhoea, and painful black discolouration on his nose, fingers, and toes. Lab results revealed anaemia, leukocytosis, elevated inflammatory markers, positive c-ANCA, and HBV PCR positivity. He was diagnosed with c-ANCA-associated small vessel vasculitis secondary to chronic hepatitis B. Treatment with methylprednisolone followed by tapering prednisolone and azathioprine led to significant improvement in his condition. Conclusion: This case highlights the rare occurrence of c-ANCA-associated vasculitis in chronic HBV without typical liver symptoms. ANCA testing may aid in early diagnosis and management of such cases. Further research is needed to explore the underlying immunological mechanisms and refine therapeutic approaches.

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Objective: To determine the prognostic significance of proteinuria monitoring in patients with anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV). Methods: We retrospectively analyzed the data of kidney biopsy-confirmed patients with AAV. Proteinuria was evaluated by a urine dipstick test. Poor renal outcome was defined as stage 4/5 chronic kidney disease (CKD) (estimated glomerular filtration rate < 30 mL/min/1.73 m2). Results: We enrolled 77 patients with a median follow-up duration of 36 months (interquartile range, 18–79) in this study. Excluding 8 patients on dialysis at 6 months, 59/69 (85.5%) achieved remission after induction therapy. Patients were then divided into two groups according to the presence of proteinuria at 6 months after induction therapy (n = 29 with proteinuria, 40 without proteinuria). There was no significant difference in the rate of relapse or death according to the presence of proteinuria (p = 0.304 relapse, 0.401 death). In contrast, patients with proteinuria had significantly lower kidney function than those without proteinuria (41 vs. 53.5 mL/min/1.73 m2, p = 0.003). Multivariate analysis revealed that eGFR values at 6 months (hazard ratio [HR] 0.925; 95% CI 0.875–0.978, p = 0.006) and proteinuria at 6 months (HR 4.613; 95% CI 1.230–17.298, p = 0.023) were significantly associated with stage 4/5 CKD. Conclusion: The presence of proteinuria at 6 months after induction therapy and low renal function was significantly associated with a higher risk of stage 4/5 CKD in patients with AAV. Monitoring for proteinuria after induction therapy may help predict poor renal outcomes in patients with AAV. [ABSTRACT FROM AUTHOR]

Background: Childhood-onset ANCA-associated vasculitides (AAV) are characterized by necrotizing inflammation and include granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). Pediatric data is scare and there have been no prior studies examining the characteristics of pediatric AAV in Central California. Methods: This retrospective study comprised AAV patients ≤18 years of age, diagnosed between 2010 and 2021, in Central California. We analyzed initial presentation including demographics, clinical, laboratory characteristics, treatment, and initial outcomes. Results: Of 21 patients with AAV, 12 were categorized as MPA and 9 with GPA. Median age at diagnosis was 13.7 years in MPA cohort and 14 years in GPA. MPA cohort were majority females (92% versus 44%). 57% of the cohort were racial/ethnic minority including Hispanics (n = 9), Asians (n = 2), multiracial (n = 1) and 43% were white (n = 9). MPA patients were more frequently Hispanic (67%), meanwhile GPA patients were frequently white (78%). Median duration of symptoms prior to diagnosis was 14 days in MPA cohort and 21 days in GPA cohort. Renal involvement was frequent (100% in MPA and 78% in GPA). GPA cohort had frequent ear, nose and throat (ENT) involvement (89%). All patients were ANCA positive. All Hispanic patients were MPO positive, meanwhile 89% of white patients were PR3 positive. MPA cohort tended towards more severe disease with 67% requiring ICU admission and 50% requiring dialysis. Two deaths were reported in MPA cohort, related to Aspergillus pneumonia and pulmonary hemorrhage. In MPA cohort, 42% received cyclophosphamide in combination with steroids and 42% received rituximab in combination with steroids. GPA patients received cyclophosphamide, either with steroids alone (78%) or in combination with steroids and rituximab (22%). Conclusions: Microscopic polyangiitis was the most frequent AAV subtype with female preponderance, shorter duration of symptoms at onset and higher proportion of racial/ ethnic minority patients. Hispanic children demonstrated frequent MPO positivity. Trends towards higher rates of ICU requirement and need for dialysis upon initial presentation was noted in MPA. Patients with MPA received rituximab more frequently. Future prospective studies are needed to understand differences in presentation and outcomes in childhood onset AAV between diverse racial-ethnic groups. [ABSTRACT FROM AUTHOR]

We report a case of isolated cutaneous small vessel vasculitis (SVV) occurring after zoledronic acid (Zol) infusion in a 58-year-old postmenopausal woman with a history of sleeve gastrectomy. This was the patient's first exposure to a bisphosphonate medication. Within minutes of the Zol infusion, she developed an episode of diffuse watery diarrhea. Although the diarrheal symptoms resolved quickly, she experienced nonsteroidal anti-inflammatory drug–responsive generalized myalgias and skin tenderness in her abdomen and extremities within a few hours. These symptoms progressed in severity over the next 5 days, and she developed nonblanching, palpable purpura extending from the ankles to the knees. Prior to Zol, labs showed sufficient 25-hydroxyvitamin D and calcium as well as normal renal and liver function. On day 10, laboratory tests revealed aspartate transaminase twice and alanine transaminase thrice the upper limit of normal. The patient was diagnosed with cutaneous SVV, with a timeline highly suggestive of an idiosyncratic reaction to Zol. She was successfully treated with a prednisone taper. No prior cases of Zol-induced cutaneous vasculitis have been reported, although there are a handful of reported cases of giant cell arteritis and urticarial vasculitis after bisphosphonate therapy. Clinicians should be aware that isolated cutaneous SVV may be a rare complication of Zol. [ABSTRACT FROM AUTHOR]

Leukocytoclastic vasculitis (LCV) is an idiopathic small vessel vasculitis. Leukocytoclastic vasculitis can be found in a spectrum of diseases and is noted as a rare extraintestinal manifestation of Crohn disease. This case report examines a 55-year-old man with a previous diagnosis of Crohn disease who was admitted after 5 days with a persistent rash. A biopsy confirmed LCV, and the patient followed up with dermatology for outpatient treatment. This study adds to the sparse medical literature on LCV cases relating to Crohn disease. [ABSTRACT FROM AUTHOR]

Vasculitis is a group of diseases characterized by the inflammation of the blood vessel walls. They are classified according to the size of the main vessel involved: large vessel, medium vessel, and small vessel vasculitis. Ophthalmic manifestations are quite common in most of these diseases. Episcleritis and scleritis are the most prevalent manifestation of vasculitis. However, there are certain ocular features characteristic of specific vasculitis entities. Given the severity and potential life-threat of these diseases, knowledge of the ocular manifestations is mandatory for the ophthalmologists. [ABSTRACT FROM AUTHOR]

Relapse in antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) is associated with significant morbidity and mortality. Utility of ANCA for prediction of relapses is still controversial. PubMed/MEDLINE, Scopus, and WebOfScience were searched, screened and confirmed for inclusion [PROSPERO No: CRD42020220308]. Studies measuring serial ANCA by ELISA or indirect immunofluorescence (IF), reporting relapses with sufficient data to calculate sensitivity and specificity were included. Diagnostic odds ratio (OR), sensitivity, specificity and likelihood ratios (LR) were synthesized using a bivariate mixed-effect regression model. Sub-group analysis included a comparison between ELISA and IIF, anti-myeloperoxidase (MPO) and -proteinase 3(PR3), and type of rise in ANCA. For meta-analysis of survival outcomes, hazard ratios were synthesized using a random-effect model. QUADAS-2 was used for assessing quality of studies, I2 statistic for heterogeneity Begg's test for publication bias. 2946 abstracts and 43 full-texts were reviewed to identify 26 eligible studies that included 2623 patients with AAV and 848 relapses. Overall heterogeneity was high [I2 = 99%] and the overall risk of bias was low to moderate. ANCA positivity by either ELISA or immunofluorescence for predicting relapse of AAV had a sensitivity of 0.70(95% CI 0.58–0.81), specificity of 0.66(0.55–0.76), positive LR of 2.1(1.6–42.7) and negative LR of 0.44(0.30–0.60). ELISA performed marginally better [OR: 5(3–7)] than IIF [OR: 4(2–9)] with similar sensitivity, specificity, PLR and NLR. The area under the curve for PR3 was 0.74(0.7–0.77), while that for MPO was not computed as the number of eligible studies was only three. In the survival analysis, the hazard ratio for relapse was 3.11(1.7–5.65). The meta-analysis shows modest accuracy of ANCA in predicting relapses of ANCA vasculitis and supports the use of serial ANCA monitoring as a biomarker for relapse. [ABSTRACT FROM AUTHOR]

Simple Summary: ANCA-associated vasculitis (AAV) is a rare disease and manifests in different organs. The exact mechanism of AAV is not fully understood, but it is speculated that it is induced by autoantibody production against proteinase-3 and myeloperoxidase. Patients diagnosed with ANCA-associated vasculitis are challenging to manage and experience several relapses during and after treatment. This systematic review represents the efficacy and safety profile of rituximab in AAV patients and provides evidence that rituximab can be considered the treatment of choice for AAV patients, especially for challenging patients with refractory releases. Background and aim: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a rare multisystem autoimmune disease developed by autoantibody production against human neutrophilic granulocytes, including proteinase-3 (PR3) and myeloperoxidase (MPO). The management of AAV patients is difficult due to the multiorgan involvement, high rate of relapse, and complications of immunosuppressive agents that make it challenging. This study aims to investigate the efficacy and safety of rituximab (RTX) therapy in patients with granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) subtypes. Method: The PubMed/Medline database was searched for any studies related to RTX therapy in ANCA-associated vasculitis (GPA and MPA subtypes), from inception to 1 August 2022, and proceeded in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA). Results: Our search resulted in 1082 initial records. After the elimination of review papers, irrelevant studies, and non-English records, 223 articles were included, and the data related to the efficacy and safety of RTX therapy were extracted. Several randomized and non-randomized studies showed that RTX is an effective treatment option for patients with AAV. Most of the studies showed the very effective effect of RTX in controlling disease in AAV patients, including pediatrics, adults, and elderlies, although RTX cannot completely prevent relapse. However, maintenance therapy helps delay the disease's relapse and causes sustained remission. Not only the licensed dose (375 mg/m2 intravenous per week for 4 weeks) could induce disease remission, but studies also showed that a single infusion of RTX could be effective. Although RTX could resolve many rare manifestations in AAV patients, there are few reports showing treatment failure. Additionally, few sudies have reported the unexpeted worsening of the disease after RTX administration. Generally, RTX is relatively safe compared to conventional therapies, but some serious adverse effects, mainly infections, cytopenia, hypogammaglobinemia, malignancy, and hypersensitivity have been reported. Conclusions: RTX is an effective and relatively safe therapeutic option for AAV. Studies on the evaluation of the safety profiles of RTX and the prevention of severe RTX-related side effects in AAV patients are required. [ABSTRACT FROM AUTHOR]

Vasculitides represent a range of disorders marked by inflammation of blood vessels, often posing significant diagnostic challenges due to their diverse clinical presentations and involvement of multiple organ systems. We present the case of a 26-year-old woman who arrived with hemoptysis and a background of exertional dyspnea, chest pain, and occasional visual disturbances. Initial investigations showed elevated perinuclear anti-neutrophil cytoplasmic antibodies (P-ANCAs) and myeloperoxidase antibodies (MPOs), indicative of microscopic polyangiitis (MPA). Imaging revealed circumferential thickening of the aorta and its branches, occlusion of the left subclavian and common carotid arteries, and ground-glass opacities in the lungs, suggesting the involvement of both large and small vessels. Although the findings suggested both MPA and Takayasu arteritis (TA), differentiating between these conditions was challenging due to overlapping clinical and radiological features. Treatment with prednisone and rituximab initially brought the symptoms under control, but the patient later experienced a relapse, illustrating the complexity of managing simultaneous small- and large-vessel vasculitis. This case highlights the necessity for a comprehensive diagnostic approach and personalized treatment strategies in handling complex vasculitides with multisystem involvement., Competing Interests: Human subjects: Consent for treatment and open access publication was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Abdelhalim et al.)

Copyright of Zeitschrift für Rheumatologie is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

A male patient in his 30s with autism and depression presented to the emergency department with joint pain, a petechial/purpuric rash, sputum production, hemoptysis, and epistaxis. His mother reported a family history of autoimmune conditions. Examination revealed a non-blanching petechial/purpuric rash and tenderness in the feet and ankles. Elevated inflammatory markers, non-blanching rash, previous history of meningitis, and a chest X-ray with patchy opacifications with new oxygen requirements prompted empirical antibiotics and steroids to cover for a chest and possible central nervous system (CNS) infection. Despite antibiotics, inflammatory markers kept rising, necessitating a switch to broad-spectrum antibiotics with subsequent clinical improvement. Later, a positive anti-proteinase 3 antibody test and a CT chest scan showing bilateral ground glass opacities confirmed granulomatosis with polyangiitis (GPA). Management included a weaning course of steroids, proton pump inhibitors, and rituximab infusions. This case highlights the need to consider autoimmune conditions like GPA in autistic patients with multi-system involvement., Competing Interests: Human subjects: Consent for treatment and open access publication was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Javed et al.)

Rosai-Dorfman-Destombes (RDD) disease is a rare syndrome characterised by benign lymphoproliferative disorder with sinus histiocytosis that presents with massive lymphadenopathy. It occurs mainly in children and young adults. It can be associated with autoimmune diseases like systemic lupus erythematosus (SLE). We present a 15-year-old male case of RDD who was diagnosed with SLE with class IV lupus nephritis and central nervous system small vessel vasculitis. Sensorium improved and lymph node size decreased with steroids. Hence, SLE can present with nephrito-nephrotic syndrome and central nervous system (CNS) small vessel vasculitis without any other systemic involvement. Steroids can be helpful in the management of RDD. Therefore, we should consider the possibility of RDD in cases of SLE with generalised lymphadenopathy, even though the association is rare., Competing Interests: Human subjects: Consent for treatment and open access publication was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Kandari et al.)

IgA vasculitis (IgAV) is an inflammation of small vessels caused by perivascular deposition of IgA and activation of neutrophils. It may present as systemic vasculitis (IgAV - Henoch-Schönlein purpura) or as a variant restricted to the skin (skin-limited IgAV), while IgA nephropathy presents a variant restricted to the kidneys. Systemic IgAV affects children more frequently than adults (150 to 200 for 1; incidence 1 in 1 million/year). In the latter, disease more often leads to chronic renal disease. The dominant clinical features include round or oval and retiform palpable purpura predominantly on the lower legs, arthralgia or arthritis, gastrointestinal bleeding or pain and glomerulonephritis with mesangial IgA deposits (IgAVN). Pulmonary, cardiac, genital and neurological involvement occurs, but is rare. Immune complexes containing galactose-deficient IgA1 play a pivotal role in the pathophysiology of IgAV; via the Fc alpha receptor (CD89), they induce neutrophilic inflammation around cutaneous vessels and mesangial proliferation and inflammation in the glomerulus. In case of self-limited disease, only symptomatic treatment is recommended. Treatment of severe IgAV, nephritis or gastrointestinal manifestations, is not established, but some studies reported a benefit of corticosteroids, combined with immunosuppressive drugs. Short-term outcome depends on the severity of gastrointestinal manifestations, while long-term prognosis depends on the severity of nephritis. [ABSTRACT FROM AUTHOR]

Abstract Background Treatment of solid malignancies has been revolutionized with the introduction of immune checkpoint inhibitors (ICIs) and their use is being expanded in therapy of different cancers. However, immune related adverse events (IRAEs) can occur during treatment. These side effects occur due to stimulation of the innate and adaptive immune system and can lead to serious complications. Recently, acral ischemia has been reported in some cases during treatment with programmed death-1 (PD-1) and cytotoxic T lymphocyte associated antigen-4 (CTLA-4) inhibitors. Here, we discuss a case in which acral necrosis developed after initiation of a PD-1 inhibitor. We offer a review of the existing literature on the pathophysiology, clinical course and treatment outcomes. Case presentation A 68-year-old female was diagnosed with stage IV non-small cell lung adenocarcinoma and was started on pembrolizumab. The patient developed sudden onset numbness and discoloration of fingertips bilaterally at week 25 after initiation of ICI treatment. Extensive workup to rule out hypercoagulable, autoimmune and vascular disease was unremarkable except for mild elevation of ANA and ESR. The symptoms quickly progressed into dry gangrene within four weeks and did not respond to medical or surgical treatment. Pembrolizumab was subsequently discontinued due to progression of metastatic disease. The patient refused further interventions and transitioned to hospice care where she expired after two months. Conclusion Acral ischemia can develop during treatment of malignancies. This complication, although uncommon, canresult in digital amputation. Physicians should be aware of the possible progression of acral vascular necrosis when Raynaud’s like symptoms develop. Larger studies are needed to confirm the role of ICIs in the pathogenesis of acral vascular necrosis.

Leukocytoclastic vasculitis (LCV) is a histopathologic description of a common form of small vessel vasculitis (SVV), that can be found in various types of vasculitis affecting the skin and internal organs. The leading clinical presentation of LCV is palpable purpura and the diagnosis relies on histopathological examination, in which the inflammatory infiltrate is composed of neutrophils with fibrinoid necrosis and disintegration of nuclei into fragments ("leukocytoclasia"). Several medications can cause LCV, as well as infections, or malignancy. Among systemic diseases, the most frequently associated with LCV are ANCA-associated vasculitides, connective tissue diseases, cryoglobulinemic vasculitis, IgA vasculitis (formerly known as Henoch-Schonlein purpura) and hypocomplementemic urticarial vasculitis (HUV). When LCV is suspected, an extensive workout is usually necessary to determine whether the process is skin-limited, or expression of a systemic vasculitis or disease. A comprehensive history and detailed physical examination must be performed; platelet count, renal function and urinalysis, serological tests for hepatitis B and C viruses, autoantibodies (anti-nuclear antibodies and anti-neutrophil cytoplasmic antibodies), complement fractions and IgA staining in biopsy specimens are part of the usual workout of LCV. The treatment is mainly focused on symptom management, based on rest (avoiding standing or walking), low dose corticosteroids, colchicine or different unproven therapies, if skin-limited. When a medication is the cause, the prognosis is favorable and the discontinuation of the culprit drug is usually resolutive. Conversely, when a systemic vasculitis is the cause of LCV, higher doses of corticosteroids or immunosuppressive agents are required, according to the severity of organ involvement and the underlying associated disease. [ABSTRACT FROM AUTHOR]

BACKGROUND: Leukocytoclastic vasculitis is a group of small vessel vasculitis and is the most common cutaneous vasculitis in dermatology. OBJECTIVE: To characterize clinically and epidemiologically patients diagnosed with leukocytoclastic vasculitis who consult in the dermatology service in two highly complex institutions in Medellín, Colombia. MATERIALS AND METHODS: A retrospective descriptive observational study was done from 2014 to 2018, which included the medical records of patients who met the eligibility criteria, a non-probabilistic sampling of consecutive cases was done. RESULTS: One hundred patients were included in this study, of which 71% were female. About the pathological history, 9%, 7%, 6% and 4% were diagnosed with lupus, arthritis, antiphospholipid syndrome and Sjögren's syndrome, respectively. In the pharmacological history, the main drugs reported were immunosuppressants with 58%, among which the administration of steroids standed out with 33%. The main dermatological lesions were maculopapular (33%), hives (16%) and ulcers (15%). CONCLUSIONS: Autoimmune diseases are the most important related illnesses for establishing the diagnosis and a causal relationship. [ABSTRACT FROM AUTHOR]

Rheumatoid vasculitis (RV) constitutes a rare but serious complication of rheumatoid arthritis (RA), characterized by the inflammation of small and medium-sized blood vessels. We present a case of a 34-year-old male with chronic alcoholism who presented with epigastric pain, hematemesis, purpuric rashes, and multiple joint swelling and pain, without prior RA diagnosis. Abdominal angiography was unremarkable, ruling out Polyarteritis Nodosa (PAN) and Mallory Weiss tear. Upper GI endoscopy revealed erosive gastroduodenitis, and biopsies confirmed small vessel leukocytoclastic vasculitis with fibrinoid necrosis, initially raising suspicion of Henoch-Schonlein Purpura (HSP). However, positive rheumatoid factor (RF), anti-cyclic citrullinated peptide (anti-CCP) antibody, elevated inflammatory markers (erythrocyte sedimentation rate (ESR), C-reactive protein (CRP)), and negative immunoassay tests led to the diagnosis of RV. Treatment with intravenous hydrocortisone led to rapid improvement, and the patient is now being monitored on an outpatient basis with progressive recovery. This case underscores the unusual presentation of gastrointestinal involvement in RV. It highlights the importance of considering RV as a primary diagnosis, even in the absence of a prior RA diagnosis. Early recognition and intervention are critical for managing complications, emphasizing the need for a high index of suspicion in rheumatic diseases' unusual presentations., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Karra et al.)

This case follows an adult middle-aged female patient who developed a purpuric rash, soreness, and swelling on her legs after walking for several days in Las Vegas. With no prior petechial rash history or presence of systemic symptoms, exercise-induced purpura (EIP) was suspected due to her protracted walking in warm weather. She recovered fully with supportive treatment. EIP, also known as exercise-induced vasculitis (EIV), is often poorly recognized, misdiagnosed, and inappropriately treated. There is a need for increased awareness and clinical diagnosis of EIV based on thorough history and physical examination., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Camejo et al.)

Various conditions under the umbrella term of vasculitis have been well documented in the literature. These have been classified into small, medium, and large vessel vasculitis. In addition, vasculitis has been categorized into radiation-induced, systemic, and paraneoplastic. Of these, paraneoplastic vasculitis accounts for 2-5% of all cases of vasculitides and is less well documented. We present a case of a female patient with a history of breast cancer presenting with an upper gastrointestinal tract (GI) bleed, which subsequently revealed an underlying diagnosis of systemic vasculitis, possibly paraneoplastic. This case highlights the importance of imaging for revealing underlying vasculitis as an etiology of GI bleed., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, McLeod-van Amstel et al.)

Oral ulcers are one of the most common complaints seen by general practitioners in their offices. Recurrent aphthous stomatitis affects roughly 20% of the general population. When ulcers persist despite conventional treatment, it is crucial to consider systemic diseases such as Behçet's disease to prevent delays in care. Early recognition and appropriate management of underlying conditions are essential for improving patient outcomes and quality of life. We present a case of a 41-year-old Scottish male who came in with complaints of recurrent oral ulcers and oral thrush. Initial treatment by an infectious disease specialist resolved the oral thrush but not the ulcers. Despite further treatment attempts for three years, including biopsy and antiviral therapy, ulcers persisted. Finally, referral to rheumatology led to comprehensive autoimmune testing, revealing positive HLA B51 and a diagnosis of Behçet's disease. Treatment with topical steroids and colchicine yielded significant improvement., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Bandari et al.)

Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, is an anti-neutrophilic cytoplasmic autoantibody (ANCA)-associated small-vessel vasculitis. Typically, it causes upper and lower respiratory tract necrotizing granulomatous inflammation and necrotizing glomerulonephritis. The diagnosis is made through clinical symptoms, positive antibody testing, imaging, and kidney biopsy. We describe the case of a man in his 60s who presented with multiple complications of GPA including rapidly progressive renal failure requiring dialysis, diffuse alveolar hemorrhage, acute respiratory distress syndrome (ARDS), circulatory shock, submassive pulmonary embolism, and biventricular and dilated cardiomyopathy., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Khatiwala et al.)

BackgroundANCA-associated vasculitis (AAV) and Sjögren’s syndrome (SS) are uncommon autoimmune diseases. The co-occurrence in the same patient has been rarely described. Acromegaly has been associated with autoimmune thyroiditis, but the prevalence of other autoimmune disorders such as AAV and SS has not been evaluated in acromegaly.MethodsCharacterization of a patient with acromegaly and two rare autoimmune diseases—SS and AAV (microscopic polyangiitis (MPA))—by autoantibody-array and whole exome sequencing (WES). Single-center retrospective review of medical records of acromegaly patients to explore the prevalence of diagnosed autoimmune diseases.ResultsWe report a Caucasian woman in her 50’s with a serologically (anti-SSA/Ro, anti-MPO-ANCA antibodies) and histologically confirmed diagnosis of symptomatic SS and MPA. SS with MPO-ANCA positivity preceded MPA. An exploratory autoantigen array detected a broad spectrum of autoantibodies. WES revealed heterozygous carrier status of the PTPN22 mutation R620W, which is associated with an increased risk for autoimmunity. A similar combination of positive anti-SSA/Ro autoantibodies and ANCA was only present in 5/1184 (0.42%) other patients tested for both antibodies in our clinic over six years. Amongst 85 acromegaly patients seen at our clinic in a 20-year period, 12% had a clinically relevant associated immunological disease.ConclusionWe present a rare case of SS and AAV in a patient with acromegaly and multiple autoantibody specificities. Patients with SS and ANCA should be closely monitored for the development of (subclinical) AAV. Whether acromegaly represents a risk for autoimmunity should be further investigated in prospective acromegaly cohorts.

Acalabrutinib is a second‐generation, highly selective Bruton's Tyrosine Kinase inhibitor (BTKi) indicated for use in some mature B‐cell malignancies. The authors describe a uniquely distributed drug reaction presenting as palpable purpura over the bilateral upper limbs. BTKi is theorised to cause haemorrhage through off‐target inhibition of Tec kinases and EGFR receptors. Dermatologists play an integral role in the multidisciplinary management of these patients to limit the negative impact on patient quality of life and, more importantly, to restrict dose reduction or treatment discontinuation. [ABSTRACT FROM AUTHOR]

Background: ANCA-associated vasculitis (AAV) and Sjögren's syndrome (SS) are uncommon autoimmune diseases. The co-occurrence in the same patient has been rarely described. Acromegaly has been associated with autoimmune thyroiditis, but the prevalence of other autoimmune disorders such as AAV and SS has not been evaluated in acromegaly. Methods: Characterization of a patient with acromegaly and two rare autoimmune diseases—SS and AAV (microscopic polyangiitis (MPA))—by autoantibody-array and whole exome sequencing (WES). Single-center retrospective review of medical records of acromegaly patients to explore the prevalence of diagnosed autoimmune diseases. Results: We report a Caucasian woman in her 50's with a serologically (anti-SSA/Ro, anti-MPO-ANCA antibodies) and histologically confirmed diagnosis of symptomatic SS and MPA. SS with MPO-ANCA positivity preceded MPA. An exploratory autoantigen array detected a broad spectrum of autoantibodies. WES revealed heterozygous carrier status of the PTPN22 mutation R620W, which is associated with an increased risk for autoimmunity. A similar combination of positive anti-SSA/Ro autoantibodies and ANCA was only present in 5/1184 (0.42%) other patients tested for both antibodies in our clinic over six years. Amongst 85 acromegaly patients seen at our clinic in a 20-year period, 12% had a clinically relevant associated immunological disease. Conclusion: We present a rare case of SS and AAV in a patient with acromegaly and multiple autoantibody specificities. Patients with SS and ANCA should be closely monitored for the development of (subclinical) AAV. Whether acromegaly represents a risk for autoimmunity should be further investigated in prospective acromegaly cohorts. [ABSTRACT FROM AUTHOR]

Lupus vasculitis (LV) is one of the secondary vasculitides occurring in the setting of systemic lupus erythematosus (SLE) in approximately 50% of patients. It is most commonly associated with small vessels, but medium-sized vessels can also be affected, whereas large vessel involvement is very rare. LV may involve different organ systems and present in a wide variety of clinical manifestations according to the size and site of the vessels involved. LV usually portends a poor prognosis, and a prompt diagnosis is fundamental for a good outcome. The spectrum of involvement ranges from a relatively mild disease affecting small vessels or a single organ to a multiorgan system disease with life-threatening manifestations, such as mesenteric vasculitis, pulmonary hemorrhage, or mononeuritis multiplex. Treatment depends upon the organs involved and the severity of the vasculitis process. In this review, we provide an overview of the different forms of LV, describing their clinical impact and focusing on the available treatment strategies.

Hydralazine is a vasodilator medication commonly used for treating hypertension. While generally well-tolerated, in rare cases it can induce autoimmune reactions, including anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis. This case report presents a patient who developed ANCA-associated vasculitis resulting in crescentic glomerulonephritis (CrGN) following long-term hydralazine therapy, highlighting the importance of considering this rare adverse effect in patients with unexplained renal decline., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2024, Rodriguez et al.)