Necrotizing Scleritis as a Manifestation of Eosinophilic Granulomatosis With Polyangiitis: A Case Report.

Eosinophilic granulomatosis with polyangiitis (EGPA) is an ANCA-associated vasculitis characterized by the development of necrotizing granulomas rich in eosinophils and vasculitis of small and medium vessels, with compromise of the respiratory tract, peripheral nervous system, and least frequent ocular involvement. We report the case of a 54-year-old Caucasian man with a history of EGPA who presented ocular pain, red eye, vision loss, and evidence of scleral slimming compatible with necrotizing scleritis. The patient was treated with systemic steroids and cyclophosphamide, which reduced the ocular pain but did not improve visual acuity, needing surgical treatment of the scleral coverage defect. While necrotizing scleritis is an unusual manifestation of EGPA, it should be part of the differential diagnosis in patients with red eye or ocular pain as an accurate diagnosis and prompt treatment could reduce local complications.

Síndrome pulmón-riñón en pacientes con hallazgos incidentales de agentes infecciosos / Pulmonary-renal syndrome in patients with incidental findings of infectious agents

El síndrome pulmón-riñón (SPR) o síndrome reno-pulmonar es la combinación de glomerulonefritis aguda rápidamente progresiva (GNARP) y hemorragia alveolar difusa (HAD) de causa autoinmune. El SPR fue inicialmente descrito por Goodpasture en el contexto del síndrome anti-membrana basal glomerular (MBG). Actualmente, las vasculitis asociadas a ANCA (VAA) explican el 60% (rango 56­77.5%) de casos, el síndrome de Goodpasture el 15% (12.5­17.5%), y un 10% de casos se deben a otras causas. El SPR presenta un gran espectro clínico, desde la capilaritis pulmonar fulminante con HAD y falla respiratoria aguda, hasta formas más sutiles de enfermedad sólo detectables mediante lavado bronquio-alveolar (LBA). El objetivo de este estudio es presentar la primera serie peruana de SPR asociada a agentes infecciosos. Reportamos 3 casos, dos correspondieron a lupus eritematoso sistémico y uno a vasculitis asociada a poliangeítis microscópica. El primer caso se asoció a sobreinfección por C. tropicalis; el segundo caso a A. fumigatus y C. albicans; y el tercero a infestación por A. lumbricoides. Todos los casos se presentaron en mujeres, requirieron soporte ventilatorio invasivo y hemodiálisis, y dos resultaron letales. Concluimos que, el SPR es una condición clínica grave comúnmente asociada a sobreinfecciones o infestaciones, y que conlleva una elevada morbilidad y mortalidad. Puesto que no existen características clínicas específicas, resulta crucial tener un alto índice de sospecha. Las investigaciones pertinentes­pruebas inmunológicas, imagenológicas, y biopsia cutánea, renal y/o pulmonar­para precisar la etiología deben realizarse sin demora ya que el tratamiento precoz puede cambiar el pronóstico de estos pacientes(AU)

Pulmonary-renal syndrome (PRS) or reno-pulmonary syndrome is the combination of acute rapidly progressive glomerulonephritis (RPGNARP) and autoimmune diffuse alveolar hemorrhage (DAH). RPS was initially described by Goodpasture in the context of anti-glomerular basement membrane (GBM) syndrome. Currently, ANCA-associated vasculitides (AAV) explain 60% (range 56­77.5%) of cases, Goodpasture syndrome 15% (12.5­17.5%), and 10% of cases are due to other causes. PRS presents a wide clinical spectrum, from fulminant pulmonary capillaritis with ADH and acute respiratory failure, to subtler forms of disease that can only be detected by bronchoalveolar lavage (BAL). The objective of this study is to present the first Peruvian series of SPR associated with infectious agents. We report 3 cases, two corresponded to systemic lupus erythematosus and one to vasculitis associated with microscopic polyangiitis. The first case was associated with superinfection by C. tropicalis; the second case to A. fumigatus and C. albicans; and the third to infestation by A. lumbricoides. All cases occurred in women, required invasive ventilatory support and haemodialysis, and two were fatal. We conclude that SPR is a serious clinical condition commonly associated with superinfections or infestations, and that it carries high morbidity and mortality. Since there are no specific clinical features, a high index of suspicion is crucial. Relevant investigations­immunological tests, imaging tests, and skin, kidney, and/or lung biopsies­to specify the etiology should be carried out without delay, since early treatment can change the prognosis of these patients(AU)

Classification of ANCA-associated vasculitis: differences based on ANCA specificity and clinicopathologic phenotype.

The classification of vasculitis according to a schema with universal acceptance is challenging, given the heterogeneous and protean nature of these diseases. Formal nomenclature and classification criteria for anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) have suffered several changes since their first description; none provides comprehensive diagnostic and classification criteria. Different factors account for the difficulties in the classification of vasculitis, including the incomplete understanding of the pathogenesis, the multisystemic nature of the disease, the non-specific patterns of vascular involvement, the overlap between entities, and the presence of various classification systems. The present article reviews the classification of AAV considering different points of view, including clinical, serologic, pathogenetic, organ predilection, therapeutic, and prognostic factors, and provides perspectives on future challenges in the understanding of AAV. There is an unmet need for a unifying view of the disease spectrum that considers the constantly evolving paradigms.

Case for diagnosis. Cutaneous small vessel vasculitis (anti-proteinase 3 positive), fever, hemoptysis, and lung cavitation in an adult

Abstract Small vessel vasculitis with anti-proteinase antibodies 3 is an atypical clinical presentation of tuberculosis. The authors present the case of a 47-year-old male patient, with palpable purpura and palmoplantar hemorrhagic blisters, with subsequent dissemination. He presented severe pulmonary symptoms with cavitation, fever, hemoptysis, and high levels of anti-proteinase 3. Histopathological assessment of the skin revealed small vessel vasculitis; pulmonary histopathology showed granulomas with caseation. Bronchoalveolar lavage was positive for alcohol-acid-fast bacilli. In countries with a high prevalence of tuberculosis, the presence of autoantibodies in a patient with vasculitis, fever, and pulmonary cavitation requires investigation of infectious causes.

Case for diagnosis. Cutaneous small vessel vasculitis (anti-proteinase 3 positive), fever, hemoptysis, and lung cavitation in an adult.

Small vessel vasculitis with anti-proteinase antibodies 3 is an atypical clinical presentation of tuberculosis. The authors present the case of a 47-year-old male patient, with palpable purpura and palmoplantar hemorrhagic blisters, with subsequent dissemination. He presented severe pulmonary symptoms with cavitation, fever, hemoptysis, and high levels of anti-proteinase 3. Histopathological assessment of the skin revealed small vessel vasculitis; pulmonary histopathology showed granulomas with caseation. Bronchoalveolar lavage was positive for alcohol-acid-fast bacilli. In countries with a high prevalence of tuberculosis, the presence of autoantibodies in a patient with vasculitis, fever, and pulmonary cavitation requires investigation of infectious causes.

Update on vasculitis: overview and relevant dermatological aspects for the clinical and histopathological diagnosis - Part II

Abstract Vasculitis is a group of several clinical conditions in which the main histopathological finding is fibrinoid necrosis in the walls of blood vessels. This article assesses the main dermatological aspects relevant to the clinical and laboratory diagnosis of small- and medium-vessel cutaneous and systemic vasculitis syndromes. The most important aspects of treatment are also discussed.

Update on vasculitis: overview and relevant dermatological aspects for the clinical and histopathological diagnosis - Part II.

Vasculitis is a group of several clinical conditions in which the main histopathological finding is fibrinoid necrosis in the walls of blood vessels. This article assesses the main dermatological aspects relevant to the clinical and laboratory diagnosis of small- and medium-vessel cutaneous and systemic vasculitis syndromes. The most important aspects of treatment are also discussed.

Head and Neck Manifestations of Granulomatosis with Polyangiitis: A Retrospective analysis of 19 Patients and Review of the Literature.

Introduction Granulomatosis with Polyangiitis (GPA) is a small vessel vasculitis characterized by a necrositing granulomatous inflammation of the upper and lower respiratory tracts and focal/proliferative glomerulonephritis. In more than 70% of the cases, the presenting symptoms are head and neck manifestations that are often misdiagnosed as infectious or allergic in etiology. Objective The present study provides an analysis of head and neck manifestations in a series of patients diagnosed with GPA. It also evaluates their medical and surgical treatment and provides a review of the relevant literature. Methods A retrospective analysis of 19 patients diagnosed with GPA at a public tertiary care hospital between 2006 and 2017 was performed. Results A total of 19 patients were included in the present study, and 16 of them presented head and neck manifestations. Sinonasal symptoms were the most common, affecting 56% of the patients, followed by laryngotracheal (31.25%) and ear (25%) symptoms. In 7 patients, sinonasal symptoms were the first manifestation of the disease (43.75%). Four patients underwent surgery at some stage of the disease. Conclusions Head and neck involvement is common in GPA and may stand for the first or the only manifestation of the disease. The otolaryngologists play a central role in the diagnosis and long-term treatment of these patients, and they have to keep this pathology in mind when treating patients with ENT symptoms that do not respond as expected to the treatment.

Head and neck manifestations of granulomatosis with polyangiitis: a retrospective analysis of 19 patients and review of the literature

Introduction: Granulomatosis with Polyangiitis (GPA) is a small vessel vasculitis characterized by a necrositing granulomatous inflammation of the upper and lower respiratory tracts and focal/proliferative glomerulonephritis. In more than 70% of the cases, the presenting symptoms are head and neck manifestations that are often misdiagnosed as infectious or allergic in etiology. Objective: The present study provides an analysis of head and neckmanifestations in a series of patients diagnosed with GPA. It also evaluates their medical and surgical treatment and provides a review of the relevant literature. Methods: A retrospective analysis of 19 patients diagnosed with GPA at a public tertiary care hospital between 2006 and 2017 was performed. Results: A total of 19 patients were included in the present study, and 16 of them presented head and neck manifestations. Sinonasal symptoms were the most common, affecting 56% of the patients, followed by laryngotracheal (31.25%) and ear (25%) symptoms. In 7 patients, sinonasal symptoms were the first manifestation of the disease (43.75%). Four patients underwent surgery at some stage of the disease. Conclusions: Head and neck involvement is common in GPA and may stand for the first or the onlymanifestation of the disease. The otolaryngologists play a central role in the diagnosis and long-term treatment of these patients, and they have to keep this pathology in mind when treating patients with ENT symptoms that do not respond as expected to the treatment (AU)

[Clinical practice guideline for ANCA-associated vasculitis with renal involvement]. / Vasculitis asociada a ANCA con compromiso renal: guía de práctica clínica.

The ANCA-associated vasculitis (AAV) is a group of systemic conditions characterized by inflammation and necrosis in small and medium vessels. AAV with different levels of disease severity could respond to different therapeutics protocols. Early diagnosis and treatment could significantly improve the outcome of the disease. The objective was to investigate the quality evidence in different therapeutical protocols proposed to AAV with renal involvement in pediatrics and adults patients and evaluate the ANCA applicability in AAV diagnosis and outcome. Using methodological search filters, we identified literature in Medline, Embase, Lilacs and Cochrane Trials Register published between 1997 and July 2015. From 4236 articles, 59 were included. The quality of evidence was assessed using the check list designed by the Cochrane Renal Group. The strength of recommendation was determinated by Levels of Evidence (Oxford Centre for Evidence-based Medicine). On the basis of current evidence, 20 recommendations were elaborated for the treatment and monitoring of patients with AAV with renal involvement in several clinical scenarios, in order to provide physicians a rational approach in daily clinical practice.

Diagnóstico de granulomatosis de Wegener en pacientes con enfermedades oculares inflamatorias / Diagnosis of Wegener's granulomatosis in patients with ocular inflamatory disease

Objetivo. Informar las manifestaciones oftalmológicas en pacientes con granulomatosis de Wegener (GW). Método. Se revisó la base de datos del Instituto de Oftalmología Conde de Valenciana. Se recolectó la exploración oftalmológica completa, los exámenes de laboratorio y de gabinete, el tratamiento y la evolución. Resultados. Se incluyeron 11 pacientes con GW (18 ojos). Siete pacientes masculinos y 4 femeninos con edad promedio de 43.7 años (28-55). Tres habían tenido diagnóstico previo de GW y los 8 restantes se diagnosticaron en nuestro departamento. Siete presentaron cuadros bilaterales y 4 unilaterales. Las formas de presentación clínica fueron escleritis necrosante con queratitis ulcerativa periférica (QUP) (7/18), escleritis difusa (3/18), escleritis nodular (1/18), uveítis anterior no granulomatosa (1/18), neuropatía óptica isquémica (1/18), neuropatía óptica retrobulbar (1/18), desprendimiento de retina seroso (2/18) y dacriocistitis (2/18). De los 18 ojos, la capacidad visual final fue mejor o igual a 20/40 en 13, 20/400 en 3, cuenta dedos a 30cm o no-percepción de luz en 1. Actualmente 7 pacientes se encuentran en fase inactiva. Conclusiones. Las manifestaciones oftalmológicas más frecuentes en pacientes con GW fueron: escleritis necrosante y QUP. En la mayoría, la GW se diagnosticó después de las manifestaciones oftalmológicas, sin embargo, todos presentaron síntomas sistémicos u oftalmológicos previos.

OBJECTIVE: Report the ophthalmologic manifestations among patients with Wegener 's Granulomatosis (WG). METHOD: We reviewed the database of the Instituto de Oftalmologia Fundación Conde de Valenciana in order to collect information regarding complete ophthalmic examination, laboratory and cabinet tests, treatment, and disease progression. RESULTS: We included 11 patients with WG (18 eyes). Seven men and four women, mean age 43.7 years (range = 28-55). Three patients had a prior diagnosis of WG and the remaining eight patients were diagnosed by our study team. Seven subjects developed a bilateral affection and four had unilateral involvement. The clinical presentation was necrotizing scleritis with peripheral ulcerative keratitis (PUK) (7/18), diffuse scleritis (3/18), nodular scleritis (1/18), non-granulomatous uveitis (1/18), optic ischemic neuropathy (1/18), retrobulbar neuritis (1/18), serous retinal detachment (2/18), and dacryocystitis (2/18). Final visual acuity was better or equal to 20/40 (13/18), 20/400 (3/18), finger-counting or no-perception of light in 1/18. Currently, seven patients are symptom free. CONCLUSIONS: The most frequent ophthalmic manifestations among our patients with WG were: necrotizing scleritis and PUK. In most cases, WG was diagnosed after ophthalmic manifestations; however, all patients displayed prior systemic or ocular symptoms.