A Standardized Physical Examination Method for Joints to Determine Rheumatoid Arthritis Activity Using the Modified RAND/UCLA Appropriateness Method.

Introduction: Rheumatoid arthritis activity is evaluated based on a physical examination of each of the joints. However, the joint examination is not standardized, and the techniques are variable and difficult to reproduce due to disagreements among examiners. Objective: To recommend standardized joint examination techniques based on the modified RAND-UCLA appropriateness method. Methods: A review of the literature was conducted to determine the items to be included in the joint examination; subsequently, a consensus of rheumatologists was achieved with the modified RAND-UCLA methodology to issue the recommendations. The diagnosis of RA and differential diagnoses were excluded. Results: Two hundred fifteen rheumatologists were invited to participate. Five were included in the core group and 26 in the group of clinical experts. Clinical experience ranged between 2 and 25 years (mean 15.6 years; standard deviation 6.3 years). Most rheumatologists participated in all rounds (Round 1: 100%, Round 2: 61%, and Round 3: 61%). Of the 45 statements in the questionnaire assessing the examination techniques, 28 (62%) were retained. In addition, 6 other statements were included during the face-to-face meeting for a total of 34 final statements. Conclusion: Physical examination techniques for joints that determine RA activity are heterogeneous and differ markedly in terms of several characteristics. A list of recommendations is proposed as a guide to improve and standardize the method for the physical examination of the joints. This standardization will improve the diagnosis and results for patients with RA and help health caregivers provide better treatment.

Diagnostic error as a scientific and ethical challenge in establishing the activity of rheumatoid arthritis / El error de diagnóstico como reto científico y ético en el establecimiento de la actividad de la artritis reumatoide

ABSTRACT Introduction: The articular examination is an essential part of the physical examination of the patient with rheumatoid arthritis, since it gives information about the disease status at a given time and allows monitoring of its evolution over time. Despite the importance of the physical examination of the joints, it is noteworthy that there is no standardized technique. Methods: This paper aims to frame a discussion on whether standardization of the joint examination is justified, presenting arguments for and against. Results and discussion: The paper raises several arguments about diagnostic error as a scien-tific and ethical challenge in establishing the activity of rheumatoid arthritis. Conclusion: It is time to adopt a standardized physical joint examination technique that allows better assessment of the inflammatory activity status of the disease, avoids risks resultant from poor disease status classification, adheres to ethical principles and does not incur unnecessary expenses. Failure to do so would have scientific, economic, ethical, and public health implications.

R E S U M E N Introducción: El examen articular es una parte fundamental del examen físico del paciente con artritis reumatoide ya que permite obtener información sobre el estado de la enfermedad en un momento determinado, así como monitorizar su evolución en el tiempo. A pesar de la importancia del examen físico de las articulaciones, no existe una técnica estandarizada. Métodos: Este ensayo tiene como objetivo desarrollar una discusión sobre si la estandarización del examen conjunto está justificada, para lo cual se presentan argumentos a favor y en contra. Resultados y discusión: El ensayo plantea varios argumentos acerca del error diagnóstico como un desafío científico y ético cuando se trata de establecer la actividad de la artritis reumatoide. Conclusión: Es el momento de adoptar una técnica de exploración física conjunta y estandarizada, que permita una mejor valoración del estado de actividad inflamatoria de la artritis reumatoide, evite los riesgos derivados de una mala clasificación del estado de la enfermedad, respete los principios éticos y no incurra en gastos innecesarios. Dejar de hacerlo tendría implicaciones científicas, económicas, éticas y de salud pública.

Design of an algorithm for the diagnostic approach of patients with joint pain.

BACKGROUND: Rheumatic diseases are a reason for frequent consultation with primary care doctors. Unfortunately, there is a high percentage of misdiagnosis. OBJECTIVE: To design an algorithm to be used by primary care physicians to improve the diagnostic approach of the patient with joint pain, and thus improve the diagnostic capacity in four rheumatic diseases. METHODS: Based on the information obtained from a literature review, we identified the main symptoms, signs, and paraclinical tests related to the diagnosis of rheumatoid arthritis, spondyloarthritis with peripheral involvement, systemic lupus erythematosus with joint involvement, and osteoarthritis. We conducted 3 consultations with a group of expert rheumatologists, using the Delphi technique, to design a diagnostic algorithm that has as a starting point "joint pain" as a common symptom for the four diseases. RESULTS: Thirty-nine rheumatologists from 18 countries of Ibero-America participated in the Delphi exercise. In the first consultation, we presented 94 items to the experts (35 symptoms, 31 signs, and 28 paraclinical tests) candidates to be part of the algorithm; 74 items (25 symptoms, 27 signs, and 22 paraclinical tests) were chosen. In the second consultation, the decision nodes of the algorithm were chosen, and in the third, its final structure was defined. The Delphi exercise lasted 8 months; 100% of the experts participated in the three consultations. CONCLUSION: We present an algorithm designed through an international consensus of experts, in which Delphi methodology was used, to support primary care physicians in the clinical approach to patients with joint pain. Key Points • We developed an algorithm with the participation of rheumatologists from 18 countries of Ibero-America, which gives a global vision of the clinical context of the patient with joint pain. • We integrated four rheumatic diseases into one tool with one common symptom: joint pain. It is a novel tool, as it is the first algorithm that will support the primary care physician in the consideration of four different rheumatic diseases. • It will improve the correct diagnosis and reduce the number of paraclinical tests requested by primary care physicians, in the management of patients with joint pain. This point was verified in a recently published study in the journal Rheumatology International (reference number 31).

Renal involvement in scleroderma / Alteraciones renales en la esclerodermia

ABSTRACT Systemic sclerosis (SSc) is a systemic autoimmune disease characterized by vasculopathy, inflammation, collagen deposits and fibrosis in the skin and internal organs. Renal complications are common in SSc. The spectrum of renal complications in systemic sclerosis includes scleroderma renal crisis (SRC), abnormal renal function, proteinuria, renal chronic vascular disease, kidney injury from nephrotoxic drugs, oxalate calcium nephropathy, glomerulonephritis and vasculitis associated to anti-neutrophil cytoplasmic antibody. Renal involvement remains an important cause of morbidity and mortality in scleroderma. The objective of this review is to describe the renal complications of scleroderma.

RESUMEN La esclerosis sistémica es una enfermedad autoinmune generalizada que se caracteriza por vasculopatía, inflamación, depósito de colágeno y fibrosis en la piel y órganos internos. Las complicaciones renales son comunes y con un amplio espectro e incluyen crisis renal esclerodérmica, función renal anormal, proteinuria, enfermedad vascular renal crónica, daño renal por fármacos nefrotóxicos, nefropatía por oxalato de calcio, glomerulonefritis y vasculitis asociadas a anticuerpos contra el citoplasma de los neutrófilos. La alteración renal permanece siendo una causa importante de morbilidad y mortalidad en la esclerodermia. El objetivo de esta revisión narrativa es describir las complicaciones renales de esta enfermedad.

Pannus y artritis reumatoide: evolución histórica y fisiopatológica / Pannus and rheumatoid arthritis: Historic and pathophysiological evolution

RESUMEN La proliferación del tejido sinovial, que es llamada pannus, se ha considerado como una manifestación tardía, inactiva e irreversible de la artritis reumatoide (AR), contrario a lo que históricamente se ha estudiado. Se realizó una búsqueda de la literatura para realizar una revisión narrativa e histórica respecto al surgimiento del término pannus y su papel en la artritis reumatoide. Estudios de microscopia de luz han mostrado el carácter destructivo de este tejido con hallazgos característicos de la AR, corroborados con microscopia electrónica arios más tarde. Estos hallazgos llevaron a caracterizar el componente celular del pannus con gran número de células inmunológicas y de líneas celulares específicas con propiedades especiales como los sinoviocitos similares a fibroblastos. Este componente celular es el origen de una gran cantidad de citoquinas y proteinasas que perpetúan y causan el daño óseo y del cartílago. Este componente inflamatorio ha sido evidente también con el desarrollo de técnicas de imágenes, como la resonancia magnética y la ultrasonografía, que muestran un papel activo del tejido sinovial engrosado, junto a la hipervascularización en el daño articular y la reversibilidad de estos cambios tras el tratamiento. Las evidencias contempladas permiten concluir que el pannus como evidencia histológica (más que clínica) se refiere a la proliferación del tejido sinovial e incluye un gran componente celular activo que genera y perpetúa la inflamación y, por tanto, la enfermedad.

ABSTRACT Pannus refers to synovial tissue proliferation, and has been considered a late, inactive and irreversible manifestation of rheumatoid arthritis (RA), contrary to historical findings. A literature search was performed on terminology about pannus and its historical role in the pathophysiology of RA. Light microscopy studies have shown the destructive impact of pannus tissue with very specific abnormalities, corroborated a year later with electronic microscopy. Some of these findings are the isolation of the immunological cells inside the tissue, especially one cell line with particular capacities, called synoviocytes similar to fibroblasts. This cellular component is the source a large quantity of cytokines and proteinases that perpetuate and cause bone and cartilage damage. Inflammation has been seen in many image techniques, such as magnetic resonance and ultrasound. These show the role of tissue widening and hyper-vascularization in tissue damage, and some reversibility after treatment of RA. With the evidence presented it is possible to conclude that pannus refers to a histological (more than clinical) term for synovial hypertrophy, and includes a large component of cell activity that generates and perpetuates inflammation and thus the disease.

Tiroiditis y pancreatitis como presentación simultánea en una paciente con nefritis lúpica. Reporte de caso / Thyroiditis and pancreatitis as a simultaneous presentation in a patient with lupus nephritis. Case report

RESUMEN El lupus eritematoso sistémico se puede presentar con un amplio espectro de síntomas que en algunas ocasiones pueden enmascarar complicaciones graves asociadas a la misma enfermedad. Dentro de estas la pancreatitis es una causa poco común, y sin embargo de alta mortalidad, especialmente en pacientes con un tratamiento no oportuno. Reportamos el caso de una paciente que cursa con lupus eritematoso sistémico con compromiso renal y de sistema nervioso central, de reciente aparición, que se asocia a la aparición de pancreatitis y tiroiditis, presentando evolución satisfactoria con esquema terapéutico de ciclofosfamida y prednisolona.

ABSTRACT Systemic lupus erythematosus can present with a broad spectrum of symptoms that on some occasions may mask serious complications associated with the same disease. Within these, pancreatitis is an uncommon but high-mortality cause, especially in patients with non-oportune treatment. We report the case of a patient with systemic lupus eryt-hematosus with recent renal and central nervous system involvement that is associated with the onset of pancreatitis and thyroiditis. A satisfactory outcome was obtained with a cyclophosphamide and prednisolone therapeutic regimen.

Physical articular examination in the activity of rheumatoid arthritis: a systematic review of the literature : Systematic review of the literature regarding physical examination in rheumatoid arthritis.

To summarize evidence concerning the articular examination needed to determine rheumatoid arthritis (RA) activity (follow-up or control) via a systematic review. A search of Medline, Embase, Lilacs, SciELO, the Web of Science, the National Technical Reports Library, and the reference lists of relevant studies through March 2017 was conducted using a systematic methodology to identify studies of patients with RA older than 18 years in which a detailed description of the physical examination or a description of the components of the articular examination was provided. Of 8322 references, 74 studies were included according to the selection criteria, and 6 references were ultimately included at the end of the review. Most of the included studies (n = 5) were associated with a moderate risk of bias. There was great variability among the studies and the articular examination methods used. Some studies presented the examination with a complete specification of the technique (n = 2), the consensus of rheumatologists (n = 2), or training through audiovisual materials and face-to-face courses (n = 2), but none of the studies explicitly showed the technique by which the physical examination was performed. Despite the importance of the clinical evaluation and physical examination of patients with RA for diagnosis, prognosis, clinimetrics, and follow-up, evidence concerning how to perform the articular examination is scarce.

Esclerosis Lupoide / Lupoid sclerosis

La esclerosis lupoide es una entidad de exclusión que reúne características fisiopatológicas, clínicas e imagenológicas comunes entre e lupus eritematoso sistémico (LES) y esclerosis múltiple (EM). El propósito del artículo es revisar aspectos inmunológicos, de laboratorio y diagnósticos diferenciales que faciliten comprobar la EL como una entidad clínica y definir las mejores posibilidades terapéuticas.

Lupoid sclerosis is a diagnosis of exclusion with similar clinical, inmunological and imagenological findings between erithematous systemic lupus (SLE) and multiple sclerosis (MS). The aim of this article is to review immunology, laboratory differential diagnosis and treatment to facilitate the understanding of EL as a clinical entity.

Síndromes paraneoplásicos neurológicos: como entender la respuesta inmune y los anticuerpos paraneoplásicos / Paraneoplasics neurologic syndromes: understanding the immune response and paraneoplastic antibodies

Los síndromes paraneoplásicos neurológicos (SPN) comprometen el sistema nervioso (central periférico o neuromuscular) de forma focal o difusa y se deben a una respuesta inmune de tipo humoral o celular sin que haya metástasis o extensión directa del tumor, su incidencia es baja. El SPN hace que el sistema inmune medie su respuesta a través de las células T principalmente cuando los antígenos son de localización intracelular, pero cuando los antígenos se localizan en la membrana celular se ha observado una mediación por anticuerpos.Los SPN pueden preceder la aparición de un tumor. Los anticuerpos onconeuronales permiten limitar su espectro diferencial y facilitan el diagnóstico y tratamiento tempranos. Los síndromes neurológicos no paraneoplásicos y sin un tumor subyacente son entidades importantes para el diagnóstico diferencial, en ellos hay una mediación por anticuerpos contra las proteínas sinápticas y de la superficie celular y comparten características clínicas con los SPN; afectan niños y adultos jóvenes y pueden responder a inmunoterapia.El objetivo de este artículo es explorar las características de los SPN y los mecanismos inmunológicos que los identifican, definir los anticuerpos mejor estudiados y presentar un plan de diagnóstico y tratamiento.

Relación de los anticuerpos anti-péptido citrulinado con manifestaciones osteo-articulares en una cohorte de pacientes con esclerodermia / Relationship between anti-cyclic citrullinated protein antibodies and osteoarticular manifestations in a cohort of systemic sclerosis patients

Introducción: la esclerodermia es una alteración autoinmune caracterizada por induración y engrosamiento de la piel. Durante el curso de la enfermedad los pacientes pueden padecer síntomas de afectación musculoesquelética que incluyen artralgias, artritis, pérdida de función articular y discapacidad consecuente. Objetivos: se estudió la relación de las manifestaciones osteo-articulares clínicas y radiológicas con los anticuerpos anti-péptido citrulinado en una cohorte de pacientes con esclerodermia. Materiales y métodos: se evaluó la historia clínica de pacientes con esclerodermia buscando datos con características de la enfermedad, afectación de órgano blanco y síntomas osteo-articulares. A todos los pacientes se les realizó anticuerpos anti-péptido citrulinado de tercera generación y se les tomó radiografía de manos y pies. Resultados: se incluyeron en forma consecutiva 123 pacientes y se analizaron los datos de 100 pacientes. Ochenta y cuatro pacientes con esclerodermia limitada y 16 con esclerodermia difusa. El promedio de edad fue de 55,5 años y la mediana del tiempo de diagnóstico correspondió a 6,9 años. Catorce pacientes presentaron anticuerpos anti-péptido citrulinado positivos (14%), 4 pacientes con esclerodermia difusa y 9 pacientes con esclerodermia limitada. Dieciocho pacientes (18%) presentaron el factor reumatoide IgM positivo y no se observó relación entre la afectación de órgano blanco con los anticuerpos. El 72% de los pacientes presentaron síntomas osteo-articulares, 17 de ellos presentaron artralgias (23,6%). La combinación de artralgias y artritis se observó en 25 (34,7%) de los pacientes. Artralgias, artritis y contracturas en 10 (13,9%) pacientes. Dos pacientes presentaron artritis (2,8%), 3 contracturas (4,2%) y 5 calcinosis (6,9%). Analizando la asociación entre los anticuerpos antiCCP y la afectación clínica articular, no se encontró asociación estadísticamente significativa (p= 0,095). En el análisis radiológico se observó: En las manos con mayor frecuencia resorción ósea u osteolisis (72%). También se observó calcinosis (54%), aumento de tejidos blandos (49%) y contracturas en flexión (31%). En los pies se observó con mayor frecuencia resorción falángica u osteolisis (13%). Además calcinosis (10%), disminución del espacio articular (6%). Se evidenció una asociación de calcinosis con los antiCCP positivos. Conclusión: los hallazgos articulares fueron frecuentes en esta cohorte de pacientes con esclerodermia. La prevalencia de los anticuerpos antiCCP y factor reumatoide fueron de alrededor del 15%. Se observó más alteraciones radiológicas en las manos que los pies probablemente por factores mecánicos. Se observó una asociación de calcinosis en las manos con los antiCCP.

Introduction: scleroderma is an autoimmune disease characterized by induration and thickening of skin. Patients may suffer from musculoskeletal symptoms which include arthralgias, arthritis, loss of articular function and disability. Objectives: we sought to assess any association between radiographic features, osteoarticular manifestations, and anti-CCP antibodies in a cohort of scleroderma patients. Materials and methods: we evaluated the clinical features of patients searching for demographic characteristics, organ system involvement and osteoarticular symptoms. The presence of anti-CCP IgG was evaluated in all patients. Standard radiographs of hands and feet were taken and analyzed. Results: one hundred twenty three consecutive patients were included and 100 patients were analyzed in the present study. Eighty four patients had limited scleroderma and 16 patients had diffuse scleroderma. The mean age was 55.5 years and the median time of diagnostic was 6.9 years. Fourteen patients were positive for presence of anti-CCP (14%), 4 patient for diffuse form and 9 patients for limited form. Eighteen patients were positive for IgM rheumatoid factor test (18%). It was not seen an association between organ involvement and antibodies. Seventy two percent of patients showed osteoarticular symptoms, 17 of them had arthralgias (23.6%), 25 had arthralgias and arthritis (34.7%), and 10 had arhtralgias, arthritis and contractures (13.9%). Two patients showed arthritis (2.8%), three contractures (4.2%) and 5 calcinosis (6.9%). There was no association between the presence of anti-CCP antibodies and osteoarticular manifestations (p= 0.095). Radiographic features seen on hands were osteolysis (72%), calcinosis (54%), increasing of soft tissues (49%) and flexion contractures (31%). On feet was seen osteolysis (13%), calcinosis (10%) and decreasing of articular space (6%). There was association between calcinosis and anti-CCP antibodies. Conclusion: the articular findings were frequently seen in this scleroderma cohort. Prevalence of anti-CCP antibodies and rheumatoid factor was around 15% positive. Hands showed more alterations than feet, probably for mechanistic factors. It was seen an association between hand calcinosis and anti-CCP antibodies.

Calcifying Lupus panniculitis in a patient without manifestations of systemic lupus Erythematosus / Paniculitis Lúpica calcificante en un paciente sin manifestaciones de lupus eritematoso sistémico

Lupus panniculitis or lupus profundus is a variant of lupus Erythematosus cutaneous that primarily affects subcutaneous tissue. Clinically, it is characterized by one or several firm subcutaneous nodules and/or plaques with or without overlying epidermal changes. It is reported to occur with a frequency of 2-3% in patients with Systemic Lupus Erythematosus (SLE). Between 10 and 50 percent of patients with lupus panniculitis will have or eventually develop Systemic Lupus Erythematosus. In nearly all cases there are deep, erythematosus plaques and nodules, and some of them ulcers, which usually involve the proximal extremities, trunk, breasts, buttocks, and face. These lesions may be tender and painful and frequently heal with atrophy and scaring, turning as a chronic condition and subsequently heal with disfigurement. We describe a patient who suffers from lupus panniculitis with no association to SLE symptoms and complicated by several progressive and disabling cutaneous lesions.

La paniculitis lúpica o también llamada lupus profundus es una variante del lupus eritematoso cutáneo que afecta el tejido celular subcutáneo. Se caracteriza clínicamente por uno o varios nódulos subcutáneos que son firmes y/o placa con o sin cambios epidérmicos. Se ha informado su frecuencia en 2% a 3% de casos de Lupus eritematoso sistémico. Entre el 10 al 50% de los casos de paniculitis lúpica va a desarrollar lupus eritematoso sistémico. En casi todos los casos hay placas eritematosas y/o nódulos que en algunos casos se ulceran y que usualmente están localizados en las áreas proximales de las extremidades, tronco, mamas, nalgas y la cara. Estas lesiones pueden ser clínicamente dolorosas y sensibles a la presión y frecuentemente cicatrizan con desfiguración del área circundante. Describimos un paciente que padece de paniculitis lúpica sin asociación de lupus eritematoso sistémico y que se complicó con varias lesiones cutáneas progresivas y discapacitantes.

Asociación de lupus eritematoso sistémico y esclerosis múltiple: esclerosis lupoide. Descripción de cuatro casos / Asociation between systemic lupus erythematosus and multiple sclerosis: lupoid sclerosis

La esclerosis múltiple (EM) y el lupus eritematoso sistémico (LES) con o sin síndrome antifosfolípido son enfermedades autoinmunes. Se ha descrito en varias ocasiones la asociación de estas enfermedades o se ha descrito el cuadro clínico de la EM con características de laboratorio de LES. Cuando afectan al sistema nervioso central pueden hacerlo en forma definida para cada enfermedad pero también pueden hacerlo en forma interpuesta o combinada de las dos enfermedades, a lo que se le ha llamado esclerosis lupoide, haciendo que en algunos casos sea difícil la diferenciación de las dos enfermedades y por lo tanto direccionar el tratamiento. Presentamos cuatro casos de esclerosis lupoide, discutimos acerca de las características clínicas y de laboratorio de esta entidad y hacemos una diferenciación de la esclerosis múltiple y de la afectación neurológica del LES, especialmente por imágenes y resultados de laboratorio.

Multiple sclerosis (MS) and Systemic Lupus Erythematosus (SLE) with/without antiphospholipid syndrome are autoinmune illnesses. It has been described in many occasions the association of these two illnesses and the clinical picture of MS with characteristics of laboratory of SLE. When they affect to the central nervous system they can make it in a defined form for each illness or they can also make it in interposed or combined form of the two illnesses what has been called lupoid sclerosis; making that in some cases difficult the differentiation of the two illnesses and therefore to address the treatment. We present four cases of lupoid sclerosis, discuss the clinical and laboratory characteristics of this entity and we make a differentiation of the multiple sclerosis with the neurological affectation of SLE especially for images and laboratory results.

The use of procalcitonin determinations in evaluation of systemic lupus erythematosus.

BACKGROUND: Procalcitonin (PCT), the precursor of the calcitonin, is synthesized in the parafollicular C-cells of the thyroid. It has been used to detect and to differentiate systemic bacterial infections from flares of systemic lupus erythematosus (SLE). PCT in serum increases in severe bacterial and fungal infections, but not, or only slightly in viral infections. OBJECTIVE: To measure PCT levels in patients with active SLE and to compare them with patients without lupus activity and to determine the possible association between activity and elevation of the PCT. DESIGN: Prospective case control study. PATIENT AND METHODS: Measurements were made of PCT (METHOD: Essay immunoluminometric--and ultrasensitive--BRAHMS Diagnostika, Berlin, Germany), C-reactive protein, erythrocyte sedimentation rate, and blood and urine cultures. The index of activity of SLE was determined by Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) score of a serial group of patients seen by our rheumatology service. Samples from 53 patients were analyzed. The patients were divided in 2 groups: group I (n = 21) with little or no activity for SLE; group II (n = 32) with activity for SLE (SLEDAI >5). None of the patients had severe bacterial infection, sepsis, or systemic multiorgan failure. RESULTS: Group I had a SLEDAI score of 1.8 [95% confidence interval (CI) 1.09-2.51] with mean levels of PCT 0. 08 ng/mL (Negative smaller than 0. 5 ng/mL). Group II SLEDAI score was 14.6 (95% CI 11.95-17.23) with mean levels of PCT 0.418 ng/mL with standard deviation 1.0021 (95% CI 0.0628-0.773). The measure of association calculated by Fisher method was not significant (1.927) (P = 0. 282). In the group II, 3 patients had frankly positive PCT (3.18, 3.42, and 3.95 ng/mL) and high activity indices (14, 13, and 24). None presented with severe infection, sepsis, or systemic multiorgan failure. They had pneumonia, renal failure (PCT 3.42 ng/mL) and urinary tract infection without systemic symptoms (3.95 ng/mL). Infection was not detected in the other patient (3.18 ng/mL) that was interpreted as a false positive. CONCLUSIONS: This study demonstrates that there is no association between the activity of SLE and PCT levels. The utility of the PCT resides is in raising suspicion of a concurrent bacterial or mycotic infection in the evaluation of patients with active autoimmune diseases.

Enfermedad pulmonar intersticial asociada a enfermedades de tejido conectivo / An interstitial lung disease (ILD) belongs to a group of diffuse parenchymal lung diseases

La enfermedad pulmonar intersticial pertenece al grupo de la enfermedad pulmonar parenqui-matosa difusa. Debe ser diferenciada de otras patologías entre las que están las neumonías intersticiales (NII) asociadas a enfermedad de tejido conectivo (ETC) y las idiopáticas. Se han originado nuevos conceptos en los últimos años y se las ha clasificado en siete subgrupos bien definidos y se ha descrito la asociación de cada uno de estos subgrupos con las ETC. Su historia natural y otros aspectos de su tratamiento no se conocen completamente. Para su diagnóstico completo se requieren criterios clínicos, imagenológicos y de histopatología. La biopsia pulmonar ocupa un lugar esencial. Es importante promover y estimular la subclasificación de cada subgrupo con el fin de conocer su historia natural, dirigir el tratamiento y mejorar su pronóstico.

It should be differentiated from other pathologies among those are idiopathic and ILD associated to connective tissue diseases (CTD). New concepts have been developed in the last years, and they have been classified in seven defined subgroups. It has been described the association of each one of these subgroups with CTD. Natural History and other aspects of its treatment is not known completely. For complete diagnose it is required clinical, image, and histopathologic approaches. The biopsy lung plays an essential role. It is important to promote and to stimulate the subclasification of each subgroup with the purpose of, knowing their natural history, directing the treatment and to improve their outcome.