Peripheral ulcerative keratitis: A review of aetiology and management

Background: Peripheral ulcerative keratitis (PUK) is a severe inflammatory disease of the peripheral cornea that can be caused by local factors or systemic inflammatory disease.Aim: The purpose of this review is to give an overview of the pathophysiology, aetiology, clinical features, diagnosis, and management of PUK.Method: A PubMed search was conducted using the keywords, ‘peripheral ulcerative keratitis’ and ‘Mooren’s ulcer’.Results: The peripheral cornea has unique characteristics the predispose to the development of PUK. These include fine capillary arcades that allow for deposition of immune complexes and subsequent activation of an inflammatory cascade with corneal melt. Several conditions have been implicated in the aetiology of PUK. The most commonly cited causes are rheumatoid arthritis (RA), granulomatosis with polyangiitis (GPA) and various dermatoses. In patients with RA, PUK usually presents in established disease, whereas in GPA, PUK may be the presenting feature in up to 60% of cases. In RA it heralds the onset of a systemic vasculitis with significant associated morbidity and mortality. The management of PUK follows an individualised stepwise approach. All patients require supportive measures to encourage healing and halt the process of keratolysis. Systemic autoimmune conditions need a systemic corticosteroid as a fast-acting agent to halt the inflammatory process while cytotoxic therapy maintains long term disease control. Failure to achieve disease control with CTT, necessitates the use of a biologic agent.Conclusion: Peripheral ulcerative keratitis is a severe inflammatory disease of the peripheral cornea that needs a thorough diagnostic workup and stepwise management approach.

Kawasaki Shock Syndrome with Initial Presentation as Neck lymphadenitis: A Case Report

Kawasaki disease (KD) is an acute systemic vasculitis of unknown cause that mainly affects infants and children and can result in coronary artery complications if left untreated. A small subset of KD patients with fever and cervical lymphadenitis has been reported as node-first-presenting KD (NFKD). This type of KD commonly affects the older pediatric population with a more intense inflammatory process. Considering its unusual initial presentation, a delay in diagnosis and treatment increases the risk of coronary artery complications. Herein, we report the case of a 9-year-old female with fever and neck mass that rapidly deteriorated to shock status. A diagnosis of KD was made after the signs and symptoms fulfilled the principal diagnostic criteria. The patient’s heart failure and blood pressure improved dramatically after a single dose of intravenous immunoglobulin. This case reminds us that NFKD could be the initial manifestation of KDSS, which is a potentially fatal condition. We review the literature to identify the overlapping characteristics of NFKD and KDSS, and to highlight the importance of early recognition of atypical KD regardless of age. We conclude that unusually high C-reactive protein, neutrophilia, and thrombocytopenia serve as supplemental laboratory indicators for early identification of KDSS in patients with NFKD.

A case of vasculitis triggered by infective endocarditis in a patient undergoing maintenance hemodialysis: a case report

Background Immunoglobulin A vasculitis (IgA vasculitis) is one of the most common forms of vasculitis in children. It rarely occurs in adults. It is a systemic vasculitis with IgA deposition and is characterized by the classical tetrad of purpura, arthritis/arthralgia, gastrointestinal and renal involvement. Certain types of infections, and pharmacological agents have been reported to be associated with IgA vasculitis. Here, we describe a case of IgA vasculitis triggered by infective endocarditis in a patient undergoing maintenance hemodialysis. Case presentation A 70-year-old man undergoing hemodialysis was admitted because of skin purpura, abdominal pain, diarrhea, and lower back pain. We suspected him as IgA vasculitis based on the clinical features and skin biopsy findings. Transesophageal echocardiography revealed infective endocarditis, which predisposed him to IgA vasculitis. He was treated with antibiotics and low-dose corticosteroids, which led to resolution of vasculitis. Conclusions This is the first case of IgA vasculitis triggered by infective endocarditis in a patient undergoing hemodialysis. Patients undergoing hemodialysis are at a high risk of infection because of immune dysfunction and frequent venipuncture. The incidence of infective endocarditis associated with IgA vasculitis is very low, but it has been repeatedly reported. Therefore, it is necessary to consider infective endocarditis in patients with clinical features that indicate IgA vasculitis.

Stroke and Internal Carotid Dissection as the First Manifestation of Giant Cell Arteritis: A Case Report

Introduction: Giant Cell Arteritis (GCA) is a systemic vasculitis that involves medium-sized and larger arteries.GCA rarely can affect the brain-arteries, resulting in ischemic strokes and transient ischemic attacks, whereby the most affected region is vertebrobasilar. Also, it's very unusual that cocurate with large artery dissections. We describe a patient with anterior brain territory stroke and right internal carotid dissection as the first manifestation of GCA.Case report: A 51-year-old man was presented with sudden onset of right-side blurred vision, frozen movements, and ptosis in the right eye and left side paresis. There was a history of right-sided frontotemporal headache, diabetes mellitus, and dyslipidemia. The Laboratory tests show erythrocyte sedimentation rate (ESR)=90, C-reactive protein (CRP)=15mg/L. CT scan and brain MRI indicated an acute ischemic infarction in the right frontal region. CT-Angiography has shown internal carotid artery dissection. Due to the presentation and lab tests, we started Methylprednisolone 1 gr for treating GCA, and temporal artery biopsy was positive for GCA in pathology findings. After ten days, inflammatory markers were reduced (ESR:40). Besides improving headaches, there was no significant change in eye deficits and reduction of left limb's force.Conclusion: Due to the noisy symptoms in patients with Stroke and carotid dissection, the diagnosis of GCA may be neglected as an underlying cause. The association of high CRP levels and the rate of ESR with Stroke, although nonspecific, should draw some attention to vasculitis, topped by GCA.

Biologics Drugs in Behçet’s Disease: A Single Centre Experience

Introduction: Behçet´s disease (BD) is a systemic vasculitis of unknown cause. Several cytokines, such as tumor necrosis factor-alpha (TNF-α), appear to play a substantial role. Therefore, biologics such as anti-TNF-α agents are rising to control severe or refractory BD´s manifestations. We aimed to describe the biological therapy´s outcomes in BD patients. Methods: A longitudinal, prospective, unicentric cohort study with patients followed in a specialized outpatient clinic. We collected data regarding BD´s manifestations, treatments, and outcomes during follow-up. Results: Our cohort includes 243 patients, of whom 31% were male. During follow-up, 20 patients (8%) were treated with biological drugs. Patients who received biological therapies were younger (p = 0.030), had less frequently genital aphthosis (p = 0.009), and more frequently erythema nodosum (p = 0.009), polyarthritis (p = 0.002), spondyloarthritis (p = 0.024), retinal vasculitis (p = 0.011) and gastrointestinal manifestations (p = 0.024), namely gastroduodenal ulcer (p = 0.035), digestive bleeding from ulcers (p = 0.002), and bowel perforation (p = 0.004). Anti-TNF-α agents were used in all of these patients, most frequently infliximab. Patients started biologicals after classical immunosuppressors failure, and most went into remission (93%). Three patients developed tuberculosis during treatment, regardless of regular screening tests. It was possible to stop biological therapy in five patients, so far, without recurrence, with 33 months of mean follow-up time after suspension. Discussion: Anti-TNF-α agents are highly effective for refractory BD´s manifestations, although they are not innocuous. Little is known about the optimal duration of these therapies, regarding when and how to stop these drugs. This issue is essential not only to avoid relapses but also to reduce therapy side-effects.

Recombinant Human Soluble Thrombomodulin Suppresses Arteritis in a Mouse Model of Kawasaki Disease

<b><i>Introduction and Objective:</i></b> Kawasaki disease (KD) is associated with diffuse and systemic vasculitis of unknown aetiology and primarily affects infants and children. Intravenous immunoglobulin (IVIG) treatment reduces the risk of developing coronary aneurysms, but some children have IVIG-resistant KD, which increases their risk of developing coronary artery injury. Here, we investigated the effect of recombinant human soluble thrombomodulin (rTM), which has anticoagulant, anti-inflammatory, and cytoprotective properties on the development of coronary arteritis in a mouse model of vasculitis. <b><i>Methods:</i></b> An animal model of KD-like vasculitis was created by injecting mice with <i>Candida albicans</i> water-soluble fraction (CAWS). This model was used to investigate the mRNA expression of interleukin (IL)-10, tumour necrosis factor alpha (TNF-α), and tissue factor (TF), in addition to histopathology of heart tissues. <b><i>Results:</i></b> rTM treatment significantly reduces cardiac vascular endothelium hypertrophy by 34 days after CAWS treatment. In addition, mRNA expression analysis revealed that rTM administration increased cardiac IL-10 expression until day 27, whereas expression of TNF-α was unaffected. Moreover, in the spleen, rTM treatment restores IL-10 and TF expression to normal levels. <b><i>Conclusion:</i></b> These findings suggest that rTM suppresses CAWS-induced vasculitis by upregulating IL-10. Therefore, rTM may be an effective treatment for KD.

Sneddon’s Syndrome as rare and underdiagnosed cause of stroke: about two cases / A Síndrome de Sneddon como causa rara e subdiagnosticada de AVC: cerca de dois casos

Case 1. Female, 27 years old, with migraine with aura. Use of combined contraceptive therapy. She presented visual and cutaneous complaints for eight months, using prednisone 60mg/day due to the hypothesis of systemic vasculitis. On examination, right temporal hemianopsia, normal fundus of the eye, Raynaud ‘s phenomenon with necrosis stitches in fingers and livedo were noted. Brain MRI with diffusion restriction in the left occipital lobe, hypersignal foci in the parietal lobe, and head of the left caudate nucleus. Reticular Livedo (RL) compatible biopsy. Anticardiolipin IgM (13.3 U/mL), ESR (47mm/h in the 1st hour and 52mm/h in the 2nd hour), and VDRL 1:8 reagents. Normal CSF. Initially treated with warfarin, she has been using rivaroxaban for two years, remaining asymptomatic. Case 2. Female, 39 years old, with a report of sudden dysarthria and right hemiparesis, with a previous episode of vertigo. A lumbosacral spine MRI was performed, presenting no alterations. Normal CSF. Antiphospholipid antibodies: Beta-2-glycoprotein I IgM 21 UR/mL and IgG 9 UR/mL, lupus anticoagulant 0.98, anticardiolipin IgG 9.4 GPL and IgM 31 MPL. Cranial MRI showed old ischemic gaps and hyperintense areas on T2/FLAIR with diffusion restriction affecting the left paramedian portion of the pons, related to acute stroke. Presence of livedo in the distal region of the limbs. In current use of warfarin 7.5mg/day, presenting remission of symptoms. Discussion: Sneddon Syndrome (SS) is a non-inflammatory thrombotic vasculopathy characterized by the concomitant presence of livedo reticularis and stroke. Neurological manifestations are present in up to 77% of cases. It is more prevalent in women between 20 and 42 years old. Treatment is aimed at preventing potentially serious, disabling, and even fatal new vascular events. Final Comments: SS is a rare syndrome that probably results from a series of acquired or congenital hemostatic abnormalities that preferentially involve the cutaneous and cerebral vascular beds. Although it has no specific biomarker, there are reports associating SS with antiphospholipid antibodies and VDRL reactor. Clinical presentation, laboratory findings and skin biopsy associated with an excellent response to anticoagulant treatment were fundamental in the diagnostic elucidation.

Complications and causes of mortality in patients with primary necrotizing systemic vasculitis

The article reviews the clinical symptoms, main complications in patients with ANCA-associated systemic vasculitis (granulomatous with polyangiitis, microscopic polyangiitis, eosinophilic granulomatous with polyangiitis) and polyarteritis nodosa during the first three years of the disease. We evaluated the relationship between complications and long-term glucocorticosteroid therapy. Patients with granulomatosis had the most unfavourable course of the disease with a high risk of complications. A high incidence of ischemic strokes and chronic kidney disease in patients with microscopic polyangiitis has been identified. We also analyzed cases of lethal outcomes in patients with primary systemic vasculitis.

Behcet’s disease: a review

Behcet's disease (BD) is a complex systemic vasculitis with an etiopathogenesis that remains unclear. It has a strong geographic association as well as a genetic propensity linked to the HLA-B51 factor and interactions between genetic and environmental factors. The typical age of onset is 25 to 40 years old and it is more common in men, who also have a more severe condition. Occlusive vasculitis is the hallmark of this condition, which can affect vessels of all diameters. Oral ulcers, genital ulcers, skin lesions, pathergy reaction as well as involvement of other systems and organs such as ophthalmic, neurological and vascular lesions, among others, are used to make the diagnosis. Uncontrolled neutrophil activation, activation of the humoral and cell immune systems, toxic proteins and infectious agents such as herpes simplex and streptococci are all involved in their pathophysiology. Due to the heterogeneity and several systems affected, the treatment is individualized and focused on treating each clinical manifestation.

Osteoporosis in rheumatic diseases

The article presents a review of studies that have examined osteoporosis in rheumatic diseases, including rheumatoid arthritis, spondylarthritis, psoriatic arthritis, systemic connective tissue diseases, and systemic vasculitis. The review discusses the pathogenesis, diagnosis and treatment of osteoporosis in these diseases, presents the results of epidemiological studies assessing the risk factors and the prevalence of osteoporosis in rheumatic diseases. There was a high prevalence of osteoporosis and fractures in rheumatic diseases, exceeding the population, associated primarily with systemic and local inflammation, as well as with the intake of glucocorticoids. It is indicated that the existing strategies for the treatment of rheumatic diseases may partially reduce bone loss, but long-term administration of glucocorticoids, on the contrary, increase bone resorption. The review presents data on the medications for the treatment of osteoporosis and approaches to the treatment of glucocorticoid osteoporosis.