Vasculitis reumatoidea: una manifestación poco habitual. Presentación de tres casos clínicos y revisión de la literatura

La vasculitis reumatoidea es una complicación sistémica y poco frecuente de la Artritis Reumatoidea. Si bien su incidencia ha descendido en los últimos años con el advenimiento de las nuevas terapias inmunosupresoras y biológicas, continua teniendo una alta morbimortalidad. Predomina en el sexo masculino, en pacientes seropositivos y con un largo período de la enfermedad establecida. Requiere de alta presunción diagnostica, siendo el compromiso cutáneo y nervioso periférico el más frecuente. La biopsia de nervio o piel es requerida habitualmente para su diagnóstico. El tratamiento se basa en corticoides e inmunosupresores. Presentamos tres casos clínicos y realizamos una revisión de la literatura.

Rheumatoid vasculitis is a rare systemic complication of rheumatoid arthritis. Although its incidence has decreased in recent years with the advent of new immunosuppressive and biological therapies, it continues to have a high morbidity and mortality. It predominates in males, in seropositive patients and with a long period of established disease. It requires high diagnostic presumption, with skin and peripheral nervous involvement being the most affected. Nerve or skin biopsy is usually required for diagnosis. Treatment is based on corticosteroids and immunosuppressants. We present three clinical cases and carry out a review of the literature.

A vasculite reumatóide é uma complicação sistêmica rara da artrite reumatóide. Embora sua incidência tenha diminuído nos últimos anos com o advento de novas terapias imunossupressoras e biológicas, continua apresentando elevada morbidade e mortalidade. Predomina no sexo masculino, em pacientes soropositivos e com longo período de doença estabelecida. Exige alta presunção diagnóstica, sendo o envolvimento cutâneo e nervoso periférico os mais afetados. A biópsia de nervo ou pele geralmente é necessária para o diagnóstico. O tratamento é baseado em corticosteroides e imunossupressores. Apresentamos três casos clínicos e realizamos uma revisão da literatura.

Development of Palpable Purpura in a Patient With Infective Endocarditis: A Case Report.

Infective endocarditis (IE) can present with a variety of signs and symptoms, including skin lesions. The few papers describing a relationship between IE and vasculitis are split between IE being able to mimic vasculitis and between IE indeed being associated with a vasculitis involving the skin, kidney, gastrointestinal tract, or peripheral nerves. It is important for clinicians to distinguish between an isolated vasculitis, infective endocarditis, and IE-associated vasculitis because the treatments and outcomes are different. We report a case of a patient with a history of intravenous (IV) drug use who initially presented with chest pain, was started on vancomycin following diagnosis of methicillin-resistant Staphylococcus aureus (MRSA) IE, left against medical advice (AMA), and then returned to the hospital due to development of a purpuric rash. We contend that while he did not have a skin biopsy due to time delay, his symmetrically distributed purpura was consistent with cutaneous vasculitis. His symptoms, including his rash and an acute kidney injury (AKI), improved with antibiotics to treat the endocarditis.

Update on targeted treatments for ANCA-associated vasculitis.

Targeted therapy has revolutionized the management of ANCA-associated vasculitis (AAV) over the last fifteen years. Rituximab, an approved induction and maintenance agent for severe AAV, is no less effective than cyclophosphamide as induction therapy and particularly useful in relapsing or refractory disease, or in women. In patients with relapsing AAV, granulomatosis with polyangiitis or PR3-ANCA, it is more effective than cyclophosphamide. Rituximab maintenance is superior to the conventional immunosuppressive drugs that it replaces. Low-dose preemptive rituximab infusions are recommended every six months for 18 months, followed by re-evaluation to decide whether 4 additional biannual infusions should be administered, balancing the probability of relapse and the risk of serious infections on rituximab. A growing body of experimental and clinical data shows that C5a pathway inhibition is a promising therapeutic option for AAV, which could reduce glucocorticoids needs. Avacopan is a first approved oral C5A receptor antagonist, used when there is a high risk that glucocorticoids will cause serious adverse events. In eosinophilic granulomatosis with polyangiitis, the importance of IL-5 for eosinophil activation and survival led to evaluation and approval of mepolizumab, a humanized monoclonal antibody directed against IL-5. Mepolizumab showed a steroid-sparing effect. Its effectiveness in active vasculitis remains uncertain and is currently being evaluated. Benralizumab targeting the IL-5 receptor was recently shown to be noninferior to mepolizumab. Rituximab has had disappointing results in non-severe active vasculitis and is being evaluated as maintenance therapy. Plasma exchange is not indicated as first-line treatment but remains recommended when creatinine levels exceed 300 µmol/L.

Comparison of different doses of Tripterygium glycosides treating in IgA vasculitis nephritis: A Bayesian network meta-analysis.

Background: Tripterygium glycosides (TG) is extracted from the roots of Tripterygium wilfordii Hook F (Lei gong teng, a traditional Chinese medicine). It is widely used in China to treat immunoglobulin A vasculitis nephritis (IgAVN), which is a common secondary glomerular disease. As there are no guidelines for the rational application of TG, we performed this study to evaluate the efficacy and safety of different doses of TG and to determine the optimal treatment for IgAVN. Methods: Ten databases were searched from their inception to April 2023 for randomised controlled trials (RCTs) using TG, TG combined with glucocorticoids (GC), or TG combined with traditional Chinese medicine (TCM) to treat IgAVN. A network meta-analysis was performed following the protocol (CRD42023401645). Results: Forty-four eligible RCTs involving 3402 patients were included. For effective rate, TG 1.5 mg/kg/d (TG1.5) + TCM was ranked as the best intervention, followed by TG 1.0 mg/kg/d (TG1.0) + TCM, TG1.5, TG1.0+GC, TG1.0, TCM, GC, and routine treatment (RT). TG1.0+TCM ranked best in reducing recurrence, followed by TG1.0+GC, GC, TG1.5, and RT. Compared with TG1.0, TG1.0+TCM and TG1.5+TCM effectively reduced liver injury events. Compared with TG1.5, TG1.5+TCM and TG1.0+TCM effectively reduced leukopenia events. No significant differences in the reduction of gastrointestinal events were observed between the interventions. Subgroup analyses explored the effects of the participants' age. The intervention rankings of the outcomes generally remained consistent. Only a small difference was observed in gastrointestinal events. TCM was the best treatment for reducing gastrointestinal events in paediatric patients. Conclusions: The results showed a positive correlation between dose and efficacy, whereas no relationship was found between dose and adverse events. TCM can boost the efficacy and reduce adverse events when combined with TG. In conclusion, we consider TG1.5+TCM as the best treatment for IgAVN. However, further research is required to confirm these findings.

Granulomatosis With Polyangiitis Presenting As Orbital Apex Syndrome.

Granulomatosis with polyangiitis (GPA) is a rare small-vessel vasculitis that typically presents with a triad of sinonasal, pulmonary, and renal symptoms. Here, we present the case of a 43-year-old female with a history of substance use disorder who presented with vision changes and worsening left eye pain over five days. Previous evaluations raised concerns about GPA versus cocaine-induced vasculitis, but diagnostic confirmation was hindered by a lack of medical follow-up. Prompt multidisciplinary intervention led to significant improvement following steroid therapy and IV antibiotics, and the patient was ultimately diagnosed with a high GPA. This case highlights the complexities involved in diagnosing and managing GPA presenting as orbital apex syndrome, particularly in patients with comorbidities and non-adherence to medical follow-up.

An Abysmal Quadrad: A Rare Case of Ankylosing Spondylitis, Ulcerative Colitis, and Takayasu Aortoarteritis Complicated by Secondary Renal Amyloidosis.

Ankylosing Spondylitis (AS) is a chronic inflammatory arthritis that typically manifests in young males and may present with extra-articular manifestations. Takayasu aortoarteritis (TA) is a large vessel vasculitis that predominantly affects young and middle-aged females. Despite the limited number of studies examining the potential association between these two diseases, we report a unique case of an individual with ankylosing spondylitis and ulcerative colitis who subsequently developed Takayasu aortoarteritis. This progression ultimately led to the development of secondary renal amyloidosis, attributed to a combination of inflammatory pathologies.

Adalimumab-induced leukocytoclastic vasculitis in a patient with ankylosing spondylitis: A case report.

Clinical Key Message: In patients receiving anti-TNF-α drugs for ankylosing spondylitis, monitoring purpuric and ischemic skin lesions is crucial. This case underscores the significance of identifying and addressing drug-induced vasculitis while stressing the necessity for prompt evaluation and exploration of alternative treatment options to safeguard patient well-being. Abstract: The case discusses a 38-year-old female with a history of ankylosing spondylitis (AS) who presented with skin lesions, including purpuric skin lesions and ischemia of her right foot digits, after initiating treatment with adalimumab. After excluding other potential causes, such as infections and malignancies, the patient received a diagnosis of moderate-sized vascular vasculitis associated with adalimumab use. Discontinuation of adalimumab and treatment with high dose glucocorticoids and intravenous pulse of cyclophosphamide resulted in the resolution of her ischemic lesions. This case underscores the importance of considering drug-related side effects in patients with new skin lesions, particularly in the context of rheumatic diseases such as AS.

Severe Occlusive Retinal Vasculitis in an Immunocompetent Patient with Chronic CMV Anterior Uveitis.

PURPOSE: We report a unique case of non-necrotizing occlusive retinal vasculitis presenting two years following chronic hypertensive uveitis. METHODS: Case Report. RESULTS: A 32-year-old Iraqi woman with a history of Posner-Schlossman Syndrome diagnosed 10 years prior presented with blurred vision and redness in her left eye. Examination demonstrated ocular hypertension, keratic precipitates, and inflammatory cells in the anterior chamber. Quantitative real-time PCR confirmed the presence of cytomegalovirus in the aqueous humor, and dilated posterior segment examination was negative for any signs of intraocular inflammation, retinitis, or vasculitis. Her uveitis workup was otherwise negative, and she was treated with valganciclovir for 6 months. Two years after her initial presentation, she was noted to have a new vitreous hemorrhage in the left eye. Fluorescein angiography demonstrated an occlusive retinal vasculitis with extensive neovascularization without retinitis. Quantitative real-time PCR again demonstrated the presence of cytomegalovirus in the anterior chamber. Her uveitis workup was repeated, which has now returned positive for HLA-B51. She otherwise did not demonstrate any systemic signs of Behcet's Disease. She was restarted on valganciclovir and oral prednisone and referred to rheumatology for consideration of adalimumab initiation. Thus far she has responded very well to treatment. CONCLUSION: This case highlights the importance of serial posterior segment examinations and HLA-B51 testing in individuals with cytomegalovirus anterior uveitis.

Cystic fibrosis dermatitis arthritis syndrome: A series of four cases.

Dermatologic manifestations of cystic fibrosis (CF) include nutrient deficiency dermatoses, vasculitis, transient reactive papulotranslucent acrokeratodema, digital clubbing, and increased rates of atopy and drug reactions. Few cases of a characteristic eruption in patients with episodic arthritis of CF have been described with prior reports primarily occurring outside of the dermatology literature. We report four cases consistent with this presentation to add to the literature and propose a new and unifying name to recognize this entity as cystic fibrosis dermatitis arthritis syndrome (CF-DAS). Clinical suspicion should remain high in young female patients with cystic fibrosis presenting with episodic joint pain and rash, independent of pulmonary exacerbations.

Macrophage Activation Syndrome in the Setting of Rheumatic Diseases.

Patients with established rheumatic disorders may develop complications of macrophage activation syndrome due to severe flares of the underlying disease (adult-onset Still's disease, SLE); however, in most other rheumatic disorders, MAS develops in association with identified viral or other infectious triggers. It is therefore important to pursue appropriate studies to identify potential infectious triggers in rheumatic disease patients who develop MAS. Management is best directed toward treatment of the triggering infections and combinations of high-dose corticosteroids, calcineurin inhibitors, and biologic therapies targeting IL-1 and/or IL-6 to suppress the associated cytokine storm.

Pauci-immune necrotizing glomerulonephritis in a 24-year-old female with negative ANCA antibodies: A rare case report.

Key Clinical Message: Pauci-immune necrotizing glomerulonephritis (PING) is a small vessel renal vasculitis usually associated with the presence of antineutrophil cytoplasmic antibodies (ANCA) against myeloperoxidase or proteinase. A small proportion of PING patients do not have ANCA antibodies. Abstract: A condition known as Pauci-immune necrotizing glomerulonephritis, or PING for short, is a type of kidney inflammation that affects small blood vessels. This condition is typically linked with the existence of certain antibodies, specifically antineutrophil cytoplasmic antibodies or ANCA, which target myeloperoxidase or proteinase. However, it's worth noting that a minor percentage of individuals diagnosed with PING do not possess these ANCA antibodies. A 24-year-old woman with no previous medical history arrived at the ER due to various symptoms including joint pain, fever, difficulty swallowing, and shortness of breath. Despite multiple symptoms suggesting systemic lupus erythematosus (SLE), this diagnosis was ruled out based on the EULAR/ACR 2019 classification criteria and laboratory tests. Other potential diagnoses such as rheumatoid arthritis (RA) and eosinophilic garnulomatosis with polyaniitis (EGPA) were also excluded based on respective criteria. The patient was treated with a 3-day course of methylprednisolone, followed by prednisolone, which improved her creatinine levels. Subsequent tests for P-ANCA and C-ANCA were negative. A kidney biopsy confirmed necrotizing glomerulonephritis, consistent with pauci-immune vasculitis. A bronchoscopy revealed bleeding and hemorrhage in her lungs, but bacterial culture analysis was negative. The patient was given piperacillin, tazobactam, and vancomycin for septic coverage, as well as intravenous immunoglobulin (IVIg), which led to symptom improvement.

Recurrent, atraumatic acute compartment syndrome secondary to IgA vasculitis: A case report.

Acute compartment syndrome is a surgical emergency requiring rapid recognition in the emergency department to minimize morbidity and mortality. It is most commonly caused by traumatic extremity fractures, which account for about 75% of cases. Atraumatic acute compartment syndrome is substantially less common with current evidence mostly limited to case reports, and diagnosis is made more challenging by the absence of an obvious traumatic injury. We present the case of a young adult female patient with IgA vasculitis who developed recurrent, atraumatic acute compartment syndrome and was successfully managed with prompt fasciotomy. This is the first case of spontaneous intramuscular hemorrhage, a rare sequela of IgA vasculitis, leading to recurrent, atraumatic acute compartment syndrome. This case highlights the importance of both a thorough physical exam and maintaining a high suspicion for acute compartment syndrome in the absence of injury to ensure patients receive prompt surgical evaluation for definitive care.

Epidemiology of ANCA vasculitis in Northern Spain.

OBJECTIVES: The incidence of anti-neutrophil cytoplasmic antibodies (ANCA) associated vasculitis (AAV) shows disparate results due to variable classification criteria and heterogeneous-population series. We aimed to estimate the incidence of AAV in a well-defined population with standardized classification criteria. METHODS: Population-based study of AAV patients diagnosed from January 2000 to December 2023 in Cantabria, Northern Spain. Patients were classified according to ACR/EULAR 2022 into granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), or unclassified vasculitis if the criteria were not met. Eosinophilic granulomatosis with polyangiitis (EGPA) patients were not included. The annual incidence rates were estimated by cases over 1,000 000 (106) (95% CI) including overall AVV, type of AAV, sex, and year of diagnosis. A literature review was also performed. RESULTS: We included 152 (80/72 men; mean age; 70.6 ± 13.18 years) patients. They were classified as MPA (67; 44%), GPA (64; 42.2%), and unclassified vasculitis (21; 13.8%). Annual incidence was 13.4 (10-16.8)/106 [male 14.5 (10.5-18.5); female 12.1 (8.7-15.6)]. The Annual incidence of MPA was 5.9 (4-7.8)/106 and GPA 5.6 (3.9-7.3)/106. The mean Annual incidence increased from 6.1 (4.5-7.7)/106-16.5 (5.6-27.4)/106 in the last three years, particularly, in GPA from 2.3 (0.3-4.9)/106-8.2 (2-14.5)/106. The prevalence of AAV was 184.7 (181-188)/106. CONCLUSION: During a 20-year period we found that the incidence of AAV (GPA and MPA) in Northern Spain is higher than Southern Spain, but lower than Northern European countries. An increase in the incidence was observed in the last years.

Relapses and serious adverse events during rituximab maintenance therapy in ANCA-associated vasculitis: a multicentre retrospective study.

OBJECTIVES: There are limited real-life data regarding the efficacy and safety of rituximab (RTX) as a remission-maintenance agent in microscopic-polyangiitis (ΜPA) and granulomatosis-with-polyangiitis (GPA). We aimed to estimate the incidence and risk factors for relapses, as well serious-adverse-events (SAEs) in MPA/GPA patients during RTX-maintenance. METHODS: Retrospective cohort of newly-diagnosed/relapsing GPA/MPA patients who received RTX-maintenance (≥1 RTX-cycle, ≥6 months follow-up) following complete-remission (Birmingham-Vasculitis-Activity-Score-version-3 = 0 plus prednisolone ≤7.5 mg/day) with induction regimens. SAEs included serious-infections, COVID-19-associated hospitalizations, deaths, cardiovascular-events, malignancies and hypogammaglobulinemia. Incidence-rates (IR) and relapse-free survival through Kaplan-Meier plots were estimated. Cox-regression was conducted to investigate factors associated with the time-to-relapse. RESULTS: 101 patients were included; 48% females, 69% GPA, 53% newly diagnosed, median age: 63 years. During follow-up (294.5 patient-years, median: 3 RTX-cycles), 30 relapses (57% major) occurred among 24 patients (24%, IR 10.2/100 patient-years). Kidney involvement (adjusted-Hazard-Ratio/aHR: 0.20; 95% CI: 0.06-0.74, p= 0.016), prior induction with RTX plus cyclophosphamide (vs RTX monotherapy: aHR = 0.02; 95% CI: 0.001-0.43, p= 0.012) and shorter time-interval until complete-remission (aHR = 1.07; 95% CI: 1.01-1.14, p= 0.023) were associated with decreased relapse-risk. We recorded 17 serious-infections (IR 5.8/100 patient-years), 11 COVID-19-associated hospitalizations (IR 3.7/100 patient-years), 4 malignancies (IR 1.4/100 patient-years), 6 cardiovascular-events (IR 2/100 patient-years) and 10 deaths (IR 3.4/100 patient-years). CONCLUSION: In this real-world study, relapses during RTX-maintenance occurred in approximately in 1 out of 4 patients. Kidney involvement, induction with RTX plus cyclophosphamide and earlier achievement of complete-remission were associated with lower relapse-risk. Serious-infections rate was consistent with previous reports, whereas an increased rate of COVID19-associated hospitalizations was observed.

Cardiovascular and cerebrovascular outcomes in anti-neutrophil cytoplasmic antibody-associated vasculitis: A systematic review with meta-analysis.

OBJECTIVE: To quantify the magnitude of the risk of total and type-specific cardiovascular and cerebrovascular diseases (CCVD) in patients with anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV). METHOD: Searches of PubMed, Embase, and the Cochrane Library were conducted. Observational studies were included if they reported data on CCVD in AAV patients. Pooled risk ratios (RR) with 95% confidence intervals were calculated. RESULT: Fourteen studies met the inclusion criteria, comprising 20,096 AAV patients (over 46,495 person-years) with 5757 CCVD events. Compared with non-vasculitis population, AAV patients showed an 83% increased risk of incident CCVD (1.83 [1.37-2.45]; n = 10), 48% for coronary artery disease (1.48 [1.26-1.75]; n = 9), and 56% for cerebrovascular accident (1.56 [1.22-1.99]; n = 9). For type-specific CCVD, the risks of myocardial infarction, stroke, heart failure were increased by 67% (1.67 [1.29-2.15]; n = 6), 97% (1.97 [1.19-3.25]; n = 8) and 72% (1.72 [1.28-2.32]; n = 4), whereas there was only a trend toward a higher risk of angina pectoris (1.46 [0.90-2.39]; n = 2), and ischemic stroke (1.88 [0.86-4.12]; n = 4). Subgroup analyses by AAV type found significantly increased CCVD risk in both granulomatosis with polyangiitis (1.87 [1.29-2.73]; n = 7) and microscopic polyangiitis (2.93 [1.58-5.43]; n = 3). In three studies reporting impact of follow-up period after AAV diagnosis, the CCVD risk was significantly higher in the first two years after diagnosis than the subsequent follow-up (2.23 [2.00-2.48] vs. 1.48 [1.40-1.56]; p < 0.01). Significant heterogeneity existed in the main analyses. CONCLUSION: This meta-analysis demonstrates that AAV is associated with increased risks of overall and type-specific CCVD, especially within two years after AAV diagnosis.

Profiles of cytokines in patients with antineutrophil cytoplasmic antibody-associated vasculitis.

Objective: This study aimed to identify plasma biomarkers that are significantly altered in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and are closely associated with AAV disease activity, as well as to explore their role in the pathogenesis of AAV. Methods: Cytokines were measured using Human Immune Response Panel 80-Plex in plasma from 59 patients with AAV and 20 healthy controls (HCs). The differentially expressed cytokines between the two groups and the possible signaling pathway involved in the pathogenesis of AAV were analyzed by bioinformatics. Relationship analysis was performed between these cytokines and clinical parameters to identify the biomarkers that can effectively indicate disease activity. Results: We identified 65 differentially expressed cytokines between the two groups. Among them, 43 cytokines significantly affected the risk of AAV. Bioinformatic analysis showed that the 43 cytokines were primarily enriched in signaling pathways such as cytokine-cytokine receptor interaction, viral protein interaction with cytokine and cytokine receptor, chemokine signaling pathway, and IL-17 signaling pathway. The levels of 25 cytokines were significantly positively correlated with Birmingham Vasculitis Activity Score (BVAS), and the levels of 2 cytokines were significantly negatively correlated with BVAS. Receiver operating characteristic analysis showed that 9 cytokines can distinguish between disease relapse and remission (PTX3: area under curve (AUC)=0.932, IL34: AUC=0.856, IL2RA: AUC=0.833, CCL23: AUC=0.826, VEGFA: AUC=0.811, TNFSF13: AUC=0.795, Granzyme A: AUC=0.788, CSF3: AUC=0.773 and IL1A: AUC=0.765). The elevated levels of these 9 cytokines suggested a risk of disease relapse. The AUC of CCL11 in disease relapse and remission was 0.811 (p=0.0116). Unlike the other 9 cytokines, a negatively association existed between CCL11 level and the risk of disease relapse. Conclusion: A group of cytokines that may be involved in AAV pathogenesis was identified. Increased PTX3, IL34, IL2RA, CCL23, and VEGFA levels correlate with active disease in AAV and may be used as biomarkers to identify the disease relapse of AAV.

Analyzing histopathological aspects and cell populations in orbital inflammatory involvement in systemic diseases: A case series from the Rheumatologist's perspective.

Orbital inflammatory disease (OID) comprises approximately 6% of orbital conditions, affecting individuals across all ages. The range of the primary orbital inflammation's differential diagnosis is extensive, encompassing autoimmune disorders such as thyroid diseases, vasculitis, sarcoidosis, connective tissue diseases, immunoglobulin G4-related disease (IgG4-RD), and giant cell myositis, whereas secondary causes span from infections to drug-induced causes. Analyzing histopathological aspects and cell populations could enhance our comprehension of the etiology of orbital inflammatory involvement in systemic diseases such as IgG4-RD. We present a series of four patients from our Rheumatology clinic, each with distinct systemic diseases, illustrating diverse manifestations of OID. This series was conducted to facilitate discussions and diagnoses of challenging cases of OID in a rheumatologic setting. The difficulty in the differential diagnosis arises from the extensive range of structures involved, resulting in a significant variation of clinical manifestations. Furthermore, the lack of definitive diagnostic laboratory tests and, often, histological findings add to the complexity. OID poses diagnostic challenges with variable clinical manifestations and overlapping imaging findings. As a diagnosis of exclusion, a comprehensive evaluation is crucial, often necessitating an orbital biopsy for confirmation. Collaborative efforts among specialists are essential for managing these intricate cases.

Ischemic stroke or transient ischemic attack due to cervical and intracranial large-vessel vasculitis secondary to primary Sjögren's syndrome: A case series and literature review.

Introduction: Primary Sjögren's syndrome (PSS) is a systemic autoimmune disease that mainly affects exocrine glands. Little is known about PSS associated cervical and intracranial cerebral large-vessel vasculitis outside of individual case reports. Methods: We present 5 cases of ischemic stroke or transient ischemic stroke (TIA) caused by PSS associated cervical and intracranial large-vessel vasculitis. Literature review was performed to summarize and identify the demographic, clinical features, treatment, and prognosis of this condition. Results: The review resulted in 8 included articles with 8 patients, plus our 5 new patients, leading to a total of 13 subjects included in the analysis. The median age was 43 (range, 17-69) years old, among which 69.2 % (9/13) were female, and 92.3 % (12/13) came from Asia. Among them, 84.6 % (11/13) presented with cerebral infarction and 70.0 % (7/10) with watershed infarction. Middle cerebral artery (MCA) (6/13, 46.2 %) and internal carotid artery (ICA) (6/13, 46.2 %) were the most frequently involved arteries. Remarkable vessel wall concentric thickening and enhancement was observed in 57.1 % (4/7) patients and intravascular thrombi was identified in 28.6 % (2/7) patients. Glucocorticoid combined with non-glucocorticoid immunosuppressants (8/12, 66.7 %) were the most often chosen medication therapy and 4 patients received surgical intervention. Conclusion: Asian females are the most vulnerable population to ischemic stroke or TIA due to PSS associated cervical and intracranial large-vessel vasculitis. Cerebral infarctions were characterized by recurrence and watershed pattern. Magnetic resonance vessel wall imaging (MR-VWI) helps to identify the inflammatory pathology of large vessel lesion in PSS.

Identification of Challenging Diagnostic Factors in Livedoid Vasculopathy: A Retrospective Study.

Background: Livedoid vasculopathy is an uncommon cutaneous ulcerative dermatosis that is challenging to diagnose. Diagnostic delay brought both pain and uncurable atrophied scar to patients. Purpose: We conducted this study to identify the factors responsible for the initial misdiagnosis of livedoid vasculopathy and to identify possible methods to increase the diagnostic accuracy of livedoid vasculopathy. Patients and Methods: We conducted a retrospective medical record review to confirm the diagnosis of livedoid vasculopathy in patients who visited the Department of Peking Union Medical College Hospital for the first time. We used the Diagnosis Error Evaluation and Research taxonomy to evaluate missed cases. Results: Twenty-three patients (85.18%) had an alternate diagnosis, including 10 (43.4%) with two or more diagnoses. The average time from disease onset to the final diagnosis of livedoid vasculopathy was 4.61 ± 0.69 years. The major diagnostic errors were clinician assessment failures and failures in the timely follow-up and rechecking of patients. Allergic vasculitis was the most common misdiagnosis. Other alternate diagnoses include Henoch-Schoenlein purpura, pigmented purpuric dermatosis, eczema, erythema nodosum, and reactive perforating collagenases. Twenty-three patients (65.21%) received systemic corticosteroid therapy before the final diagnosis of livedoid vasculopathy. Conclusion: It is critical to raise the awareness of clinicians about livedoid vasculopathy, especially when patient present with extensive livedo racemosa or long-lasting purpuric lesions on the lower limbs. Long-term follow-up is necessary, especially for younger patients. Skin biopsy is recommended before systematic therapy.