Nódulos pulmonares cavitados y vasculitis ANCA asociada: secundarios a infección por zigomicetos / Cavitated pulmonary nodules and associated ANCA vasculitis: secondary to zygomycetes infection

La presencia de nódulos pulmonares cavitados nos obliga a plantear varios diagnósticos diferenciales. El diagnóstico definitivo hay que definirlo en base a lo que nos aportan los exámenes serológicos, imagenológicos e histológicos, y correlacionar con la forma de presentación clínica. Un diagnóstico importante que debe ser considerado es la Granulomatosis de Wegener (GW) que corresponde a una vasculitis, en la mayoría de los casos sistémica y en la que encontramos anticuerpos anticitoplasma de neutrófilos de histología compatible con vasculitis. Las patologías infecciosas son otra causa importante de lesiones nodulares en pulmón. Si estamos ante pacientes con algún grado de inmunosupresión, no debemos olvidar la etiología micótica y dentro de esta la infección causada por hongos del grupo Zigomicetes (mucormicosis), sobre todo por la urgencia de realizar tratamiento agresivo y su alta mortalidad.

The presence of cavitated pulmonary nodules obliges one to pose various differential diagnoses. A definite diagnosis must be defined based on serological, imagenological and histological exams, and contrast these with the clinical manifestation. An important diagnosis that must be considered is Wegener’s granulomatosis, which corresponds to a Vasculitis, usually systemic, in which we find antineutrophil cytoplasmic antibodies and histology compatible with Vasculitis. Infectious pathologies are an important cause of pulmonary nodular lesions. If faced with a patient with a degree of immunosuppression, we must not forget the mycotic etiology, and within this the infection caused by fungi from the Zygomycetes group (mucomycosis), above all due to the urgency of aggressive treatment and its high mortality rate.

Compromiso cardiovascular en el lupuseritematoso sistémico / Cardiovascular involvement in patient with systemic lupus erythematosus

Lupus erythematosus is a multisystemic disease that compromises principally women in fertile age. The principal affected organs are kidney, SNC, bone marrow and serous membranes. Cardiovascular affection includes pericardium, conduction system, myocardium, valves and coronary arteries. The most frequent valve disease is Libman-Sacks endocarditis, although valvulitis or valve dysfunction can exist as well. The mitral valve is the most affected, followed by the aortic valve. The most frequent valve abnormality is slight to moderate aortic insufficiency, while serious insufficiency or valve disruption is very rare. A physical examination has limited efficacy in the diagnosis of valve disease. A high degree of suspicion associated with echocardiography helps to establish the diagnosis. If surgery is not needed, antiplatelet therapy is recommended for asymptomatic patients, and oral anticoagulation treatment is advised for those with valve disease and evidence of thromboembolic phenomena. Recurrence of the disease has been observed in biological grafts, which makes the use of mechanical prostheses advisable when valve replacement indication exists. The following case shows the clinical evolution of a female patient with a rare but very serious lupus erythematosus complication.

Artritis por Cryptococcus neoformans en un adulto mayor: Presentación de un caso y revisión / Cryptococcus neoformans arthritis in elderly adult: Case report and review

Cryptococcal infection is not frequent in immunocompetent patients. The most commonly involved sites are the lungs and the central nervous system. Cryptococcal osteomyelitis occurs in 5-10 percent of patients with disseminated cryptococcosis with few reports of cryptococcal arthritis in elderly adults. We describe the case of a 92-year-old woman with a Cryptococcus neoformans knee joint infection associated to osteomyelitis.

La infección por Cryptococcus neoformans es infrecuente, los sitios más comunes de presentación son pulmón y SNC. La osteomielitis criptococóccica ocurre en 5 a 10 por ciento de los pacientes con criptococcosis diseminada. La mayoría de casos ocurre entre 21 y 59 años de edad, hay pocos reportes de artritis criptococóccica en adultos mayores.

Manifestaciones clínicas de la granulomatosis de Wegener y la poliangeítis microscópica en Santiago-Chile, 1990-2001 / Clinical features of Wegener granulomatosis and microscopic polyangiitis in Chilean patients

Background: Systemic vasculitis are a group of heterogeneous diseases characterized by inflammation and necrosis of blood vessel walls. The etiology is not known, but geographic and environmental factors are implicated. Aim: To describe the clinical features of microscopic polyangiitis (MPA) and Wegener's granulomatosis (WG) in a Chilean cohort of patients. Patients and methods: Retrospective review of the medical records of 123 patients with the diagnosis of systemic vasculitis (65 MPA and 58 WG), seen from 1990 to 2001. The diagnosis were made based on the American College of Rheumatology and Chapel Hill criteria. Results: The mean follow-up for MPA was 15 months (1-120) and for WG, 20 months (1-120). The median age (years) at diagnosis for MPA was 61 (19-82) and WG 50 (20-82). Gender distribution was similar in both groups (male: 68percent and 57percent respectively).The main clinical features in the MPA group were renal involvement (68percent), peripheral nervous system involvement (57percent), pulmonary hemorrhage (28percent), and skin disease (32percent). In the WG group were alveolar hemorrhage (62percent), renal involvement (78percent), paranasal sinus involvement (57percent), and ocular disease (26percent). In both, creatinine levels above 2.0 mg/dl were associated with a higher mortality (p< 0.01). ANCA by immunofluorescence was performed in 56 MPA patients (75percent had pANCA, 4percent had cANCA and 21percent were ANCA negative) and in 55 WG patients (17percent had pANCA, 79percent had cANCA and 4percent were ANCA negative). Global mortality was 18percent and 17percent respectively, and the most common causes of death were infections. Conclusions: The clinical features of our patients are similar to other published data. In our WG and MPA patients the main predictor for death was a serum creatinine above 2 mg/dl.

Estudio del polimorfismos de receptores Fcylla en pacientes chilenos con lupus erimatoso sistémico / Polymorphisms of Fcylla receptors in chilean patients with systemic lupus erythematosus

Background: Polymorphisms of Fc receptors for IgG (FcgR) have been proposed as a genetic factor that influences susceptibility for systemic lupus erythematosus (SLE). Human FcgRIIa has 2 codominantly expressed alleles, H131 and R131, which differ at amino acid position 131 in the second extracelular domain (histidine or arginine respectively) and differ substantially in their ability to bind human IgG2. The H131 allele binds IgG2 efficiently, whereas R131 binds it poorly. Because IgG2 is a poor activator of the classical complement pathway, the H131 is essential for the disposal of IgG2 immune complexes. Aim: To determine the distribution of FcgRIIA genes in a cohort of Chilean SLE patients, with or without a history of lupus nephritis. Patients and methods: We studied 52 Chilean SLE patients fulfilling the 1982 American College of Rheumatology (ACR) criteria, 20 of whom had a history of nephritis, and 44 ethnically matched disease-free controls. FcgRIIa allotypes were genotyped by PCR. Results: No significant association was observed between the low affinity FcgRII receptor (FcgRIIa-R131) and the presence of SLE or lupus nephritis. However, genotype frequencies in SLE patients but not in controls, departed from the proportions predicted by the Hardy-Weinberg equilibrium, suggesting this locus might be related to the disease. Conclusions: Our results suggest that in Chilean patients with SLE, as well as in many other populations, the R131 allotype is not a major factor predisposing to the development of SLE or lupus nephritis