Reporte de caso: vasculitis leucocitoclastica como forma de presentación de hepatitis b crónica / Case report: leukocytoclastic vasculitis as presenting manifestation of chronic hepatitis b virus infection

La vasculitis leucocitoclástica, también denominada angeitis cutánea leucocitoclástica, es la forma más común de vasculitis. Si bien la mayoría de los casos son idiopáticos, entre los agentes etiológicos que podemos nombrar se encuentran los agentes infecciosos, las enfermedades del tejido conectivos, las reacciones de hipersensibilidad a medicamentos y las neoplasias solidas o hematológicas. Si bien los procesos infecciosos son una causa conocida de vasculitis leucocitoclástica, la infección por virus de Virus de hepatitis B (VHB) es muy infrecuente. Presentamos una mujer de 47 años, sin antecedentes patológicos previos, que consultó por artralgias en rodillas y tobillos, mialgias en gemelos y rash purpúrico con leve prurito en ambos miembros inferiores, de un mes de evolución. La biopsia cutánea de las lesiones de miembros inferiores fue compatible con vasculitis leucocitoclástica. La serología de hepatitis B fue positiva por lo que inició tratamiento antiviral con Tenofovir y Prednisona con buena evolución de sus lesiones cutáneas

Leukocytoclastic vasculitis, also called leukocytoclastic cutaneous angiitis, is the most common form of vasculitis. Although most cases are idiopathic, etiologic agents include infectious agents, connective tissue diseases, drug hypersensitivity reactions, and solid or hematologic malignancies. Although infectious processes are a known cause of leukocytoclastic vasculitis, hepatitis B virus (HBV) infection is very rare. We present a 47-year-old woman, with no previous pathologic history, who consulted for arthralgias in the knees and ankles, myalgia's and purpuric rash with mild pruritus in both lower limbs, of one month evolution. Skin biopsy of lower extremity lesions was compatible with leukocytoclastic vasculitis. Hepatitis B serology was positive, so she started antiviral treatment with tenofovir and prednisone with good evolution of her skin lesions

Cardiovascular Manifestations of Systemic Vasculitides.

PURPOSE OF REVIEW: Vasculitis can cause heart disease and are associated with premature atherosclerosis, causing increased morbidity and mortality. Consequently, it is important to know how they can affect the cardiovascular system in order to detect and treat the abnormalities in earlier phases. RECENT FINDINGS: A clear increasing trend of inpatient burden of myocardial infarction and thromboembolic events in granulomatosis with polyangiitis has been observed lately. Behçet's disease has been linked to an increased risk of atrial fibrillation. Studies showing increased atherosclerosis and thromboembolic phenomena in vasculitis are continuously published. Improvement in imaging techniques has consistently showed that subclinical cardiovascular involvement is frequent. Vasculitis may affect seriously the cardiovascular system causing an important increase in morbidity and mortality. Subclinical involvement is frequent. Early treatment with immunosuppression and sometimes surgery, is of paramount importance to improve the prognosis.

Clinical Profile of Levamisole-Adulterated Cocaine-Induced Vasculitis/Vasculopathy: A 30-Case Series.

OBJECTIVES: The aims of this study were to describe clinical and laboratory manifestations of patients with levamisole-adulterated cocaine-induced vasculitis/vasculopathy and to propose a skin classification according to the distribution and severity of lesions. METHODS: We report the characteristics of 30 patients admitted with levamisole-adulterated cocaine-induced vasculitis/vasculopathy in 4 high-complexity institutions in Colombia, from December 2010 to May 2017. We compare our findings with the main published series. RESULTS: Median age was 31 years (interquartile range, 27-38 years) with a male-to-female ratio of 5:1. Eighty-three percent of the patients had retiform purpura affecting the limbs, buttocks, face, or abdomen; 73% had ear necrosis, 50% cutaneous ulcers, 17% genital necrosis, 13% oral ulcers, and 10% digital necrosis. Cutaneous involvement was classified according to the frequency of the compromised corporal area, and purpuric lesions were stratified in 4 grades of severity. Anti-neutrophil cytoplasmic autoantibodies were positive in 85% of the cases, lupus anticoagulant in 73%, and antinuclear autoantibodies in 57%; rheumatoid factor was negative in all cases. We found nephritis in 17 cases (57%). Prednisolone was used in most of the patients (70%), with other immunosuppressive agents being used in a lower percentage. Improvement was observed in 93% of the patients, but symptoms recurred in 40%, attributed to relapses in consumption. End-stage chronic renal disease developed in 10% of the cases, and 1 patient died. CONCLUSIONS: Because of rising cocaine consumption and levamisole adulteration frequency, levamisole-adulterated cocaine-induced vasculitis/vasculopathy is becoming more common. Detailed characterization of skin involvement coupled with multiple antibody positivity is essential for a diagnosis. Renal involvement is frequent, clinically and histologically heterogeneous, and potentially serious.

INCA (Peru) Study: Impact of Non-Invasive Cardiac Magnetic Resonance Assessment in the Developing World.

Background Advanced cardiac imaging permits optimal targeting of cardiac treatment but needs to be faster, cheaper, and easier for global delivery. We aimed to pilot rapid cardiac magnetic resonance ( CMR ) with contrast in a developing nation, embedding it within clinical care along with training and mentoring. Methods and Results A cross-sectional study of CMR delivery and clinical impact assessment performed 2016-2017 in an upper middle-income country. An International partnership (clinicians in Peru and collaborators from the United Kingdom, United States, Brazil, and Colombia) developed and tested a 15-minute CMR protocol in the United Kingdom, for cardiac volumes, function and scar, and delivered it with reporting combined with training, education and mentoring in 2 centers in the capital city, Lima, Peru, 100 patients referred by local doctors from 6 centers. Management changes related to the CMR were reviewed at 12 months. One-hundred scans were conducted in 98 patients with no complications. Final diagnoses were cardiomyopathy (hypertrophic, 26%; dilated, 22%; ischemic, 15%) and 12 other pathologies including tumors, congenital heart disease, iron overload, amyloidosis, genetic syndromes, vasculitis, thrombi, and valve disease. Scan cost was $150 USD, and the average scan duration was 18±7 minutes. Findings impacted management in 56% of patients, including previously unsuspected diagnoses in 19% and therapeutic management changes in 37%. Conclusions Advanced cardiac diagnostics, here CMR with contrast, is possible using existing infrastructure in the developing world in 18 minutes for $150, resulting in important changes in patient care.

Reporte de seis casos de úlceras genitales durante la inducción en leucemia aguda promielocítica con ácido all-transretinoico y revisión bibliográfic / Report of six cases of Genital Ulcers during induction therapy of Acute Promyelocytic Leukemia with ATRA and review of literature

El ácido all-transretinoico (ATRA) es uno de los mayores avan-ces en el tratamiento de las leucemias promielocíticas agudas (LPA). Con el uso asociado de quimioterapia y corticoides hacen de ésta leu-cemia las de mejor pronóstico hematológico con altas tasas de cu-ración. El ATRA es generalmente bien tolerado pero puede presen-tar efectos adversos sistémicos, englobados dentro del denominado sindrome ATRA (SATRA), o efectos directos gastrointestinales y mu-cocutáneos tales como las úlceras escrotales, transitorias y de buena respuesta clínica, tratándose de una entidad diferente al SATRA con presencia de vasculitis en el estudio anatomopatológico. En la revisión que realizáramos, hemos detectado 44 casos reportados en la literatu-ra a los que hemos agregado, en el siguiente documento, seis pacientes evaluados en nuestra institución con úlceras genitales asociadas al uso de ATRA, cuatro de ellos con presencia de vasculitis como lesión histo-lógica y un paciente con diagnóstico de síndrome Sweet

All-trans retinoic acid (ATRA) is one of the greatest advances in the treatment of Acute Promyelocytic Leukemia. The combination of all-trans-retinoic acid (ATRA), chemotherapy and corticoids has made acute promyelocytic leukemia (APL) a highly curable leukemia. The ATRA is generally well tolerated. Adverse effects, include ATRA syndrome (SATRA), and the gastrointestinal and mucocutaneous side effects, such us scrotal ulcers, wich are transitory and with a good clinical response. They are a different entity to SATRA, and presents vasculitis in the histological study. Of our knowledge, 44 cases were reported in the literature, we present the following document with six patients evaluated at our institution with genital ulcers associated with ATRA, four of them present vasculitis in pathological study, and one patient Sweet ́s Syndrome

Pulmonary artery aneurysms in Behçet disease / Aneurismas de artérias pulmonares na doença de Behçet

Pulmonary artery aneurysms (PAAs) are the most common type of pulmonary involvement in Behçet's disease. However, the relationships between clinical features and prognosis have not been sufficiently evaluated. This article describes the results of a comprehensive review, revealing that PAAs have a predilection for hemoptysis manifestations, increased dimensions, right lower lobar location, multiplicity and concurrent intramural thrombus formation. Surgical intervention was needed in one third of patients. Patients with massive hemoptysis and PAA rupture warranted emergency operations. Conservatively treated patients were prone to PAA progression; interventional embolization was associated with higher risks of recurrence and reintervention for PAAs; and surgically treated patients exhibited the highest mortality rates. In conclusion, PAAs in Behçet's disease are characterized by a predilection for hemoptysis manifestations, right lower lobar location, multiplicity, and concurrent intramural thrombus formation. Both the condition itself and the surgical operations it warrants are linked with high mortality due to PAA hemorrhage...

Os aneurismas das artérias pulmonares (PAA) são as manifestações mais comuns dos pulmões na doença de Behçet. No entanto, as relações entre as características clínicas e o prognóstico ainda não foram devidamente explicadas. O objetivo do presente artigo foi fazer uma ampla revisão da literatura sobre esta questão. As fontes de dados contaram com uma ampla revisão bibliográfica dos anos de 1990 a 2013, sobre os seguintes temas: doença de Behçet, síndrome de Hughes-Stovin, aneurisma de artéria pulmonar e pseudoaneurisma da artéria pulmonar. Os PAA evoluíram com predileção por hemoptise, aumento de dimensões, localização no lobo inferior direito, multiplicidade e formação de trombo intramural. A intervenção cirúrgica foi necessária em um terço dos pacientes. O tratamento cirúrgico emergencial foi indicado na vigência de ruptura do PAA e de hemoptise maciça. Os pacientes tratados conservadoramente evoluíram com propensão para a progressão do PAA. A embolização dos PAA foi associada a uma taxa maior de recidiva e de reintervenção. Houve diferença significativa entre os grupos quanto às taxas de mortalidade, tendo o grupo do tratamento cirúrgico apresentado a maior taxa. Dentre as variáveis citadas, a hemoptise, o envolvimento da artéria lobar e a ruptura do PAA foram fatores preditivos de maior risco de mortalidade. Houve diferenças significativas nas taxas de mortalidade entre os pacientes cirúrgicos e intervencionistas, e entre os três grupos de pacientes: cirúrgico, intervencionista e conservador. Os pacientes tiveram uma sobrevida global de 61,7% em um seguimento médio de 22,5 meses. Os PAA, na doença de Behçet, apresentaram as seguintes predileções: tendência a hemoptise, multiplicidade, localização no lobo inferior direito e presença de trombos intramurais. As rupturas e hemorragias dos PAA, aliadas ao necessário tratamento cirúrgico emergencial, resultaram no aumento de mortalidade destes pacientes...

Situações especiais nas vasculites pulmonares / Special situations in pulmonary vasculitis

A extensa rede vascular pulmonar e brônquica, associada à grande quantidade de células imunocompetentes, torna os pulmões importante alvo de vasculites imunomediadas. O envolvimento destes vasos sanguíneos pode provocar qualquer sintoma respiratório, dependendo do tipo, local e extensão da lesão no sistema vascular respiratório. Algumas situações ameaçadoras da vida podem resultar, abrindo ou acompanhando o quadro destas patologias e, se não prontamente identificadas e tratadas, podem resultar em risco elevado de morbimortalidade. Destacamos duas destas condições especiais - estenoses de grande vias aéreas e hemorragia alveolar disusa - e descrevemos seus aspectos fisiopatológicos, clínicos e terapêuticos

The extensive pulmonary and bronchial vascular system, associated to large amount of immunocompetent cells, makes the respiratory system an important target of immune mediated vasculitis. These blood vessels involvement can cause any respiratory symptoms, depending on the type, location, and degree of lesion in the pulmonary vascular system. Some life-thereatening conditions may ensue, opening or following the course of those deseases and, if not identified and treated promptly, result in increased risk of morbitidy and mortality. We highlight two of those special situations: large airway stenosis and diffuse alveolar hemorrhage, describing their pathophysiological clinical and therapeutic aspects

Abordagem terapêutica das vasculites pulmonares / Therapeutic approach to pulmonary vasculitis

Muitas vasculites sistêmicas acometem o trato respiratório. O acometimento mais frequente é o do parênquima pulmonar pelas vasculites de pequenos vasos ANCA associadas (antineutrophil cytoplasmic antibodies), que incluem a granulomatose com poliangite (GPA, antiga granulomatose de Wegener), a poliangite microscópica (MPA), granulomatose eosinofílica com poliangite (EGPA, antiga doença de Churg-Strauss). Este artigo propõe-se a revisar o tratamento dessas doenças de acordo com recomendações da liga europeia de combate ao reumatismo (EULAR), com base no estádio e na atividade de doença, incluindo as terapia usadas na indução e na manutenção de remissão, assim como nos casos refratários ao tratamento convencional

There are many vasculitis that affect the respiratory tract. The pulmonary parenchyma is the most frequently involved and occurs mainly in ANCA associated small vessel vasculitis (antineutrophil cytoplasmic antibodies), which include granulomatosis with polyangitis (GPA, formely Wegener's granulomatosis), microscopic polyangitis (MPA), eosinophilic granulomatosis with polyangitis (EGPA formely Churg-Strauss Syndrome). This article reviews the treatment of them, according to EULAR (European League Against Rheumatism) recommendations, which are based on stage and severity of disease, including induction and maintance therapy, and therapy of refractory disease

Vasculites pulmonares para o pneumologista: uma "cartilha introdutória" / Pulmonary vasculitis for pulmonologists: a 'introductory primer

Vasculites pulmonares são doenças raras, mas com grande possibilidade de produzir morbimortalidade nos indivíduos acometidos, não somente pela sua potencial gravidade multisistêmica, mas também por seu diagnóstico geralmente tardio. A identificação de padrões de doença que remetem a estes diagnósticos e a utilização de novos meios de investigação - incluindo ANCA (anticorpo antineutrofílico citoplasmático), reconhecendo suas vantagens e limitações - são importantes, se quisermos utilizar o moderno arsenal terapêutico da forma adequada e com sucesso. Atenção às resultantes clínicas é fundamental no acompanhamento de longo curso destes pacientes

Pulmonary vasculitis are rare illnesses, although possessing substantial morbi-mortality in affected individuals, not only due to its multisystemic nature, but also because of commonly delayed diagnosis. Disease pattern recognition that remind us of that group of diseases, and the use of modern investigation methods - including ANCA (anti-neutrophil cytoplasmic antibodies), with the knowledge of its pros and cons, are key features if the state of the art therapeutic modalities are to be properly used with success. Attention to clinical outcomes is fundamental in the long range management of these patients

Vasculitis gingival como complicación de sepsis de punto de partida enteral / Vasculitis gingival sepsis complicating enteral starting point

Vasculitis es la inflamación de vasos sanguíneos, con isquemia, necrosis y compromiso sistémico. se describe el manejo multidisciplinario de niña de 4 meses quien ingresó al hospital universitario de Maracaibo con fiebre, diarrea, deshidratación, consciente, con períodos de somnolencia, abdomen distendido, ausencia de ruidos hidroaéreos. Posteriormente presenta convulsión, deterioro del estado general, lesiones eritematosas múltiples y en rodetes gingivales, de color blanco parduzco, necróticas, manifestaciones de shock séptico. Antecedente de ingestión de n-butilbromuro de hioscina. laboratorio: anemia, leucocitosis, proteína c reactiva elevada, cultivos negativos. Biopsia del rodete gingival: vasos sanguíneos con paredes infiltradas por células inflamatoriasmononucleares y polimorfonucleares dispersas y necróticas, destrucción y oclusión del endotelio, confirmando la vasculitis gingival. Recibió antibioticoterapia, soporte hemodinámico, cirugía de zonas necrosadas. egresó a los 15 días con diagnóstico de vasculitis gingival por sepsis de origen enteral. el manejo interdisciplinario médico-odontológico facilita la resolución de situaciones generales con compromiso bucodental

Vasculitis is the inflammation of blood vessels, with ischemia, necrosis and systemic involvement. this paper describes the multidisciplinary management of a 4 month old female infant that was admitted to the hospital universitario de Maracaibo with fever,diarrhea, dehydration, periods of somnolence, distended abdomen, and absence of bowel sounds. later, the patient presented seizures, deterioration of her general condition, multiple erythematous and brownish white, necrotic lesions of the gingival border mucosa, and septic shock manifestations. the patient had a history of ingestion of n-butylbromid hyoscine. laboratory tests: anemia, leukocytosis,elevated c-reactive protein, negative cultures, biopsy of the gingival border: blood vessel walls infiltrated by scattered and necrotic polymorphonuclear and mononuclear inflammatory cells, destruction and occlusion of the endothelium confirming gingival vasculitis. treatment included antibiotics, surgery of necrotic areas and hemodynamic support. she was discharged after 15 days with the diagnosis of gingival vasculitis secondary to enteral sepsis. Medical and dentistry interdisciplinary management facilitates the resolution of generalsituations with oral involvement

Presentación de un caso de vasculitis de Wegener / Presentation of a case of Wegener’s vasculitis

Se presenta una paciente con granulomatosis de Wegener en su forma localizada y se efectúa una actualización de las manifestaciones clínicas, el diagnóstico y el tratamiento.

Evaluación del daño de órgano en pacientes con poliangeitis microscópica del Hospital Nacional Guillermo Almenara Irigoyen Enero 2005 - Diciembre 2010 / Evaluation of the damage of organ in patients with microscopic polyangiitis of the National Hospital Guillermo Almenara Irigoyen January 2005 - December 2010

OBJETIVO: Evaluar la extensión de daño de órgano en pacientes con Poliangeitis Microscópica (MPA) atendidos en el Servicio de Reumatología del Hospital Nacional Guillermo Almenara Irigoyen. METODOS: Se revisaron todos los casos con diagnóstico de MPA entre Enero 2005 y Diciembre 2010. Se realizó el análisis univariado para determinar los factores asociados al Índice de Daño de Vasculitis (VDI) a los 6 y 12 meses. Posteriormente, se realizaron modelos de regresión lineal con las variables que se encontraron asociadas ajustándolas para edad y sexo. Se utilizó el paquete estadístico SPSS v.16.0. RESULTADOS: La distribución por género fue 21 mujeres y 11 varones con edad promedio de 57.16 años. Se evaluó el VDI a los seis meses en 26 pacientes y a los doce meses en 20 pacientes, el promedio de daño fue 2.04 y 3.05 respectivamente Los factores asociados al VDI a los 6 meses fueron el compromiso pulmonar: Enfermedad Pulmonar Intersticial Difusa (EPID) más Hemorragia Alveolar (HA) (p: 0.024), compromiso renal severo (GMNRP) (p: 0.009) y el uso de ciclofosfamida (0.022). A los 12 meses sólo se encontró asociado el uso de ciclofosfamida (p: 0.009). Después de ajustar para edad y sexo, los factores que permanecieron asociados con el daño a los 6 meses fueron el compromiso renal severo (beta 0.646, p<0.001) y el compromiso pulmonar (beta 0.404, p: 0.031). CONCLUSIONES: El compromiso renal severo y el compromiso pulmonar al momento del diagnóstico se encontraron como factores asociados a mayor daño a los seis meses

Drug-associated livedoid vasculopathy

Livedoid vasculopathy is reported in a 38-year-old white female, who first presented with spontaneous skin lesions in the left ankle, at 17 years old. For the last fifteen years she used low-dose oral contraceptive (gestodene and ethinylestradiol) and persisted asymptomatic for a long period. Nevertheless, painful red papules and dark spots reappeared in the same area and progressed to an intensely tender and irregular shallow ulcer, during summer. Skin biopsy samples showed dermal vessels with subintimal fibrinoid necrosis and intraluminal thrombosis, without clear inflammation. Cutaneous lesions have improved faster after topical betamethasone was added to treatment. Data from the present case suggests a drug-associated livedoid vasculopathy.

Relata-se caso de vasculopatia livedóide em uma mulher branca de 38 anos, que inicialmente apresentou lesões cutâneas espontâneas no tornozelo esquerdo aos 17 anos de idade. Durante os últimos quinze anos, fez uso de contraceptivos orais (gestodene e etinilestradiol). Permaneceu assintomática por longo período; entretanto, no último verão, pápulas avermelhadas dolorosas e manchas escuras reapareceram na mesma área e formaram uma úlcera rasa irregular intensamente dolorosa. O exame das amostras da biópsia de pele mostrou vasos dérmicos com necrose fibrinóide e trombose intraluminar, sem reação inflamatória. As lesões cutâneas apresentaram cicatrização mais rápida após acréscimo de betametasona tópica ao esquema terapêutico. Os dados do presente caso sugerem vasculopatia livedóide associada ao uso de droga.

Aspectos clínico-terapéuticos de la púrpura de Schönlein-Henoch / Clinical-therapeutic aspects of the purple of Schönlein-Henoch

La púrpura de Schönlein-Henoch es la vasculitis más común en la infancia y su diagnóstico es mayormente clínico. Describimos el comportamiento de esta enfermedad según variables clínico- terapéuticas. Se realizó un estudio retrospectivo y descriptivo de los pacientes con diagnóstico de púrpura de Schõnlein-Henoch en un período de 4 años. Se utilizó como fuente de información las historias clínicas y se confeccionó una guía de recolección de información que contenía las variables estudiadas. La afección fue más frecuente en el grupo etario de 5 a 9 años. El 50 por cientode los pacientes padeció infecciones virales antes del comienzo de la enfermedad. En la mayoría de ellos se encontró la tríada clásica de rash, dolor abdominal y artritis. El 95,3 por ciento de los pacientes tuvo una evolución satisfactoria con tratamiento sintomático.

Schonlein-Henoch purpura is the most common infantile vasculitis and is mostly clinically diagnosed. A retrospective descriptive study of patients with Scholein-Henoch purpura diagnosis was conducted for 4 years. The source of information was medical records and a data gathering guide containing the studied variables was used. The behaviour of this disease according to clinical and therapeutic variables was described. The disease was often seen in 5-9 years-old group. Fifty percent of patients suffered viral infections before the onset of disease. The majority of them presented with classical triade of rash, abdominal pain and arthritis. Of the total number, 95,3percent of cases recovered satisfactorily with symptomatic treatment.

Update in the diagnosis and management of pulmonary vasculitis.

The term vasculitis encompasses a number of distinct clinicopathologic disease entities, each of which is characterized pathologically by cellular inflammation and destruction of the blood vessel wall, and clinically by the types and locations of the affected vessels. While multiple classification schemes have been proposed to categorize and simplify the approach to these diseases, ultimately their diagnosis rests on the identification of particular patterns of clinical, radiologic, laboratory, and pathologic features. While lung involvement is most commonly seen with the primary idiopathic, small-vessel or antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides of Wegener granulomatosis, microscopic polyangiitis, and Churg-Strauss syndrome, one should remember that medium-vessel vasculitis (ie, classic polyarteritis nodosa), large-vessel vasculitis (ie, Takayasu arteritis), primary immune complex-mediated vasculitis (ie, Goodpasture syndrome), and secondary vasculitis (ie, systemic lupus erythematosus) can all affect the lung. However, for the purpose of this review, we will focus on the ANCA-associated vasculitides.

Vasculitis en paciente crítico: síndrome riñón-pulmón / Vasculitis on the intensive care unit: pulmonary-renal syndrome

Pulmonary-renal syndrome (PRS) is a combination of diffuse pulmonary haemorrhage and glomerulonephritis it's not a single entity and is caused by a variety of conditions and pathogenic mechanisms; the majority of cases of PRS are associated with antineutrophil cytoplasmic antibodies (ANCA). The final diagnosis rests on a balance of clinical, laboratory, radiological and histological features. The exclusion of alternative diagnoses is important in assuring appropriate therapy. In patients in Intensive Care Unit it's describe a high morbility and mortality; because of that need early diagnosis and treatment. We present a 48-year-old woman with history of vasculitis ANCA-MPO (+) admitted in respiratory distress and renal failure.

Pulmonary-renal vasculitic disorders: differential diagnosis and management.

Pulmonary-renal syndrome (PRS) is a combination of diffuse pulmonary hemorrhage and glomerulonephritis. Pulmonary-renal syndrome is not a single entity and is caused by a variety of conditions, including Goodpasturés syndrome associated with autoantibodies to the glomerular and alveolar basement membranes, various forms of primary systemic vasculitis associated with serum positivity for antineutrophil cytoplasmic antibodies (ANCA), cryoglobulinemia, systemic lupus erythematosus, systemic sclerosis, antiphospholipid syndrome, environmental factors, and drugs. The majority of cases of PRS are associated with ANCAs. The antigen target in Goodpasturés syndrome is the alpha-3 chain of type IV collagen. The antigen target in PRS associated with systemic vasculitis is proteinase-3 and myeloperoxidase. Pulmonary-renal syndrome has been observed from the first to the ninth decade of life. The widespread adoption of serologic testing performed in an appropriate clinical context hopefully will limit diagnostic delay. The goals of treatment in PRS are to remove the circulating antibodies, to stop further production of autoantibodies, and to remove any antigen that stimulates antibody production. Treatment is based on plasmapheresis, steroids, and cyclophosphamide; however, infections are frequent contributors to death, and less toxic alternatives may improve outcome and prognosis resulting in a long-term survival. The degree of renal function and the percent of crescents on renal biopsy are better predictors of outcome. Renal transplantation can be safely carried out in PRS.