Angiography Revealing a Possible Paraneoplastic Vasculitis: A Case Report.

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.

Recurrent Oral Ulcers: Are They Horses or Zebras?

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.

Granulomatosis With Polyangiitis: Cardiac, Renal, and Respiratory Involvement.

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.

Sterile Endocarditis and Splenic Infarct: A Rare Masquerading Presentation of Granulomatosis With Polyangiitis in a Patient With Pulmonary Aspergillosis.

Granulomatosis with polyangiitis (GPA) is a rare multisystem disease characterized by vasculitis affecting small vessels, resulting in the formation of necrotising granulomata, primarily affecting the lungs, the upper respiratory tract, and kidneys. Almost all patients have upper and lower respiratory involvement; up to 85% of patients with GPA develop kidney disease within two years of diagnosis. Cutaneous, neurological, and ocular manifestations are also seen with varying frequencies. However, cardiac manifestations of the disease are rare and scarcely reported in the literature. Here, we report a case of a 65-year-old female with an initial diagnosis of pulmonary aspergillosis based on the presence of septate hyphae branching at acute angles on lung biopsy and elevated serum galactomannan, who, over the following months, developed a multitude of issues such as myocardial infarction, sterile endocarditis, splenic infarction, and heart block, as well as the challenges faced in establishing a diagnosis and managing its complications.

Lung Involvement in Pulmonary Vasculitis: A Radiological Review.

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.

Cutaneous Small-Vessel Vasculitis Induced by Escitalopram: A Case-Based Brief Review of the Literature.

A 65-year-old male with multiple comorbidities and recently diagnosed with diabetic kidney disease developed upper and lower extremity rash following escitalopram initiation for his depressive mood. Clinical assessment and skin biopsy confirmed cutaneous small-vessel vasculitis (CSVV), prompting drug discontinuation and oral methylprednisolone therapy. The resolution of the rash was achieved within a week. This rare case of CSVV induced by escitalopram highlights the importance of timely recognition and management of drug-induced CSVV and adds to the limited literature on selective serotonin reuptake inhibitor-associated CSVV.

Classification Criteria for ANCA Associated Vasculitis - Ready for Prime Time?

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. 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.

Hydralazine-Induced Antineutrophilic Cytoplasmic Antibody (ANCA)-Associated Vasculitis Presenting as Crescentic Glomerulonephritis.

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.

Leukocytoclastic vasculitis in a patient with ankylosing spondylitis: A case report.

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. Abstract: 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.

Renal tubular estrogen ß receptors are expressed at high levels in small vessel vasculitis and are primarily localized in the distal tubule.

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.

An Unusual Presentation of Renal Failure: A Case of Myeloperoxidase-Antineutrophil Cytoplasmic Antibody (MPO-ANCA) Vasculitis.

Myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) vasculitis manifests as a neutrophilic inflammation impacting small vessels across multiple organs, notably the lungs, kidneys, and skin. We present a unique case of MPO-ANCA vasculitis in a 77-year-old female characterized by glomerulosclerosis, rapidly progressive renal failure necessitating hemodialysis (HD), bullous skin lesions, and hypoxic respiratory failure. The patient, who had a history of type 2 diabetes, presented with progressive dyspnea, hypoxia, and acute kidney injury superimposed on chronic kidney disease (CKD) progressing to renal failure requiring dialysis. A renal biopsy highlighted globally sclerosed glomeruli, interstitial fibrosis, and tubular atrophy, along with increased immunoglobulin M (IgM) deposits on immunofluorescence, differing from typical findings. Prompt initiation of prednisone led to respiratory and cutaneous improvement; however, despite therapy, extensive renal damage led to the permanent requirement of dialysis.  MPO vasculitis primarily targets small vessels, frequently affecting kidneys, with only a subset of patients progressing rapidly to end-stage renal failure necessitating HD, as observed in our case. Contrary to classical histopathological patterns, our patient exhibited augmented IgM deposits. Left untreated, MPO vasculitis with renal involvement poses a mortality risk of up to 90%, underscoring the significance of prompt detection and corticosteroid intervention to avert renal failure and improve patient outcomes. Early recognition and timely treatment are pivotal in mitigating the dire consequences of this condition, emphasizing the importance of considering MPO vasculitis in patients with rapidly deteriorating renal function.

Amiodarone-Induced Leukocytoclastic Vasculitis in a Decompensated Heart Failure Patient: A Case Report.

Leukocytoclastic vasculitis (LCV) is a small-vessel vasculitis characterized by inflammation and damage to the walls of small blood vessels. It typically presents with palpable purpura and can be associated with various systemic conditions. Although its etiology is diverse, LCV has been associated with systemic diseases, infections, medications, and autoimmune disorders. Here, we present a case of LCV in a patient with decompensated heart failure. A 58-year-old man presented with progressively deteriorating swelling in both his lower limbs and scrotum, a persistent dry cough associated with minor ulcerative lesions on his shins, and a patchy rash with pustules and flat reddish spots. He was hospitalized three days prior due to atrial fibrillation and rapid ventricular rate, for which he was commenced on amiodarone. This rash persisted for three days, yet he denied experiencing any discomfort or itchiness along with it. Based on his clinical picture, laboratory evaluations, and imaging findings, he was diagnosed with LCV induced by amiodarone.

[Vasculitides]. / Vaskulitiden.

In everyday clinical practice, immunologically mediated systemic vasculitides are among the rare diseases, meaning that basic knowledge of major symptoms and indicative laboratory findings is crucial for the inclusion of these complex clinical entities in differential diagnostic considerations. For many years, systemic vasculitides have been classified according to the primarily affected vessel size, distinguishing large, medium-sized, and small vessels. Pain is very often one of the main complaints of these diseases, be it, for example, the temporally accentuated headache in giant cell arteritis, the early morning myalgias in the shoulder and hip girdle in polymyalgia rheumatica, or the mononeuritis multiplex in eosinophilic granulomatosis with polyangiitis. General symptoms such as fever, weight loss, and night sweats are often accompanied by greatly increased parameters of inflammation. In addition, organ-specific symptoms and/or laboratory abnormalities may provide crucial information. These include ENT symptoms, pulmonary or skin manifestations, as well as signs of renal involvement, such as peripheral edema, rise in blood pressure, hematuria, proteinuria, or a rapid loss of kidney function. If there is reasonable suspicion of disease, patients should be transferred to specialized centers with an interdisciplinary team. In most cases, an immunosuppressive therapy regimen is required, although in recent years the path towards avoiding high glucocorticoid doses with many side effects has been paved by the use of novel therapies.

Uncommon presentation of granulomatosis with polyangiitis: Prostate involvement.

Granulomatosis with polyangiitis (GPA) is an autoimmune granulomatous disease of unknown etiology; frequently associated with anti-proteinase 3 antineutrophil cytoplasmic antibody (PR3-ANCA). Although any organ can be involved, prostatic involvement in GPA is very rare. We present a male patient with GPA, 26 years old, with pulmonary manifestations and prostatic involvement who underwent exhaustive evaluation. The patient's laboratory tests and imaging scans showed evidence of lesions in multiple areas, including the prostate. Histopathological testing confirmed that the lesions were consistent with granulomatosis with polyangiitis. The patient was treated with oral steroids and rituximab and showed significant improvement. He was later maintained on azathioprine without any relapse.

Neuroimaging Scoring Tools to Differentiate Inflammatory Central Nervous System Small-Vessel Vasculitis: A Need for Artificial Intelligence/Machine Learning?-A Scoping Review.

Neuroimaging has a key role in identifying small-vessel vasculitis from common diseases it mimics, such as multiple sclerosis. Oftentimes, a multitude of these conditions present similarly, and thus diagnosis is difficult. To date, there is no standardized method to differentiate between these diseases. This review identifies and presents existing scoring tools that could serve as a starting point for integrating artificial intelligence/machine learning (AI/ML) into the clinical decision-making process for these rare diseases. A scoping literature review of EMBASE and MEDLINE included 114 articles to evaluate what criteria exist to diagnose small-vessel vasculitis and common mimics. This paper presents the existing criteria of small-vessel vasculitis conditions and mimics them to guide the future integration of AI/ML algorithms to aid in diagnosing these conditions, which present similarly and non-specifically.

Gabapentin-Induced Cutaneous Leukocytoclastic Vasculitis: A Case Report.

Leukocytoclastic vasculitis (LCV) is a type of small vessel vasculitis, characterized by a perivascular neutrophilic inflammatory infiltrate with fibrinoid necrosis and fragmentation of nuclei ("leukocytoclasia"). Although up to half of the cases of LCV are idiopathic, infections and drugs are the most common secondary triggers for this condition. We present the case of an 88-year-old woman who developed an erythematous maculopapular rash on both thighs three days after starting gabapentin for neuropathic leg pain, without other associated symptoms. Skin biopsy was compatible with cutaneous vasculitis with a leukocytoclastic pattern. The skin lesions resolved within about 10 days after discontinuing gabapentin, supporting the diagnosis. To our knowledge, there are only four published cases of LCV secondary to gabapentin. This case highlights the importance of being alert for diagnosing drug-related cutaneous manifestations, even if the drug is used in our daily practice and vasculitis is not a common side effect, since discontinuing the suspected agent is crucial to resolve skin lesions and to avoid more serious complications.

Childhood-Onset ANCA- Associated Vasculitis: single center experience from Central California.

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.

Utility of Dermoscopy in Cutaneous Small Vessel Vasculitis: Preliminary Observations from a Study of 30 Cases.

Background: Dermoscopy is a non-invasive diagnostic technique that provides an added advantage to the routine clinical diagnostic exercise. Role of dermoscopy in cutaneous small vessel vasculitis has not been explored well. Objective: This study was intended to delineate the dermoscopic features of cutaneous small vessel vasculitis and to correlate them with histopathological findings of the disease. Materials and Methods: This was a cross-sectional study involving 30 patients with cutaneous small vessel vasculitis confirmed by histopathology and direct immunofluorescence. In each patient, dermoscopic features of early/evolving and established lesions were recorded. Dermoscopic-histopathological correlation was assessed for established lesions. Results: On dermoscopy, the early/evolving lesions showed a dull red background in all the 30 (100%) patients, red globules in 8 (26.7%), and red dots in 4 (13.30%) patients. The established lesions showed red background in 28 (93.3%) patients, white and yellow structureless areas in 19 (63.33%) patients each, red globules in 18 (60%), and red dots in 16 (53.3%) patients. A statistically significant association between red globules and red blood cell extravasation was noted (P-0.01). White and yellow structureless areas also showed a statistically significant association between sparse (P-0.023) and dense (P-0.007) perivascular infiltrates, respectively. Conclusion: Dermoscopy of cutaneous small vessel vasculitis exhibits fairly reliable and reproducible features correlating well with histopathological aspects of the disease. Hence, inclusion of dermoscopy in the clinical diagnostic protocol for cutaneous small vessel vasculitis is beneficial in complementing the clinical diagnosis and in differentiating from other inflammatory purpuras.

Risk factors for relapse in non-infectious cryoglobulinemic vasculitis, including type I cryoglobulinemia: a systematic review.

Background: Data on non-infectious cryoglobulinemic vasculitis (NICV) is scarce, especially concerning the management of relapses, which are troublesome. We aimed to investigate risk factors for relapse in NICV. Methods: A systematic literature search of CINAHL, Embase, MEDLINE, Scopus, and the Web of Science databases was implemented until April 2023. Eligible studies included randomized control trials, observational studies, and case series with ≥4 patients. Two reviewers independently extracted data and assessed the quality of the eligible studies. Results: A total of 3,724 articles were retrieved from a database search, with 27 studies meeting the inclusion criteria for review. Most studies (n = 23) detailed relapses, with the time to relapse varying between 1 and 80 months. The relapse rate was reported at 28% in Type I NICV and ranged from 22% to 60% in mixed NICV. Risk factors for relapse in NICV were identified based on the cryoglobulin subtype and correlated with clinical and immunological responses to varying treatment regimens. Type I NICV with an associated lymphoproliferative disorder exhibited a response-relapse pattern. Cutaneous and articular involvement and incomplete clinical and immunological responses to treatment, particularly corticosteroid monotherapy and occasionally rituximab, influence the risk of relapse in Type II and Type III NICV. Conclusion: Our findings underscore the significance of attaining both clinical and immunological responses and identifying risk factors for relapse in NICV. Appropriate risk stratification for NICV patients is essential for the successful implementation of effective treatment strategies. Systematic review registration: https://www.crd.york.ac.uk/prospero/, identifier CRD42023408140.

An Unexpected Case of Cutaneous Vasculitis Following Zoledronic Acid Infusion.

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.