Pulmonary manifestations of primary sjögren’s syndrome.

Sjögren's syndrome (SS) is a complex autoimmune exocrinopathy with multifactorial pathogenesis and multisystem manifestation. It is called primary Sjögren's syndrome (PSS) when the manifestations are seen without any other co-existent rheumatic diseases. The incidence of respiratory system involvement varies widely in the reported medical literature, partly due to lack of a universal agreement over the diagnostic criteria of the disease and the type of study methods employed. Respiratory system manifestations are protean; upper airway symptoms are very common and so is the complaint of dry cough. The PSS patients may develop interstitial lung diseases (ILDs) such as usual interstitial pneumonia (UIP), non-specific interstitial pneumonia (NSIP), lymphocytic interstitial pneumonia (LIP), bronchiolitis obliterans and organising pneumonia (BOOP), etc. They may also develop the whole spectrum of lymphoproliferative disorders of the lung ranging from LIP to follicular bronchiolitis, nodular lymphoid hyperplasia and low-grade lymphomas. Therapeutic options include symptomatic and supportive measures and corticosteroids as the mainstay of the treatment for ILDs occurring in these patients. In recent years, rituximab (anti-CD20) has emerged as a promising treatment for this disease, though data from controlled trials are still lacking. Pulmonary involvement may be a source of significant morbidity in these patients, though only rarely, it is the cause of death.

Proliferaciones linforreticulares del pulmón / Pulmonary lymphoproliferative lesions

The present review describes the current classification of the pulmonary lymphoproliferative lesions as proposed by the WHO in 2004 with emphasis in the clinical picture and histopathological features. The definition of these entities includes the clinical picture, histopathology, immunohistochemistry and molecular features. The differential diagnosis of the most important entities is also briefly discussed

En el presente trabajo de revisión se describe la clasificación actual de las lesiones linfoproliferativas del pulmón propuesta por la OMS el año 2004 con énfasis en el cuadro clínico y los aspectos histopatológicos. La definición de estas entidades incluye cuadro clínico, histopatología, inmunohistoquímica y características moleculares. Se discute brevemente el diagnóstico diferencial de las formas más importantes

MALT lymphoma of the intestine. A clinicopathological study over a period of 13 years.

Clinicopathological features of 14 patients with histologically confirmed primary intestinal Non-Hodgkin lymphoma were analysed. Patients were mostly young males with median age of 27.2 years. Main presenting features was intestinal obstruction. Diffuse large cell lymphoma was the commonest histological type. All cases were found to be B cell type in immunohistochemistry.