Lymphocytic interstitial pneumonia is a rare idiopathic interstitial pneumonia characterized by infiltration of alveoli and alveolar septa with small lymphocytes and varying numbers of plasma cells. Lymphocytic interstitial pneumonia abbreviated lip, also known as lymphoid interstitial pneumonia and lymphocytic interstitial pneumonitis, is an uncommon diffuse lung disease. B3, lymphocytic interstitial pneumonia lip, and acute cor pulmonale. Lymphocytic interstitial pneumonia is a syndrome secondary to autoimmune and other lymphoproliferative disorders this video contains general medical information if.
Differential diagnosis of lymphocytic interstitial. A few case reports have described an association with common variable immunodeficiency cvid. Respiratory bronchiolitis associated interstitial lung disease. Lymphocytic interstitial pneumonia lip is a rare lung disease on the spectrum of benign pulmonary lymphoproliferative disorders. Usual interstitial pneumonia uip is the prototype of pulmonary fibrosis with socalled temporal heterogeneity. Lymphocytic interstitial pneumonitis can occur at any age. Parenchymal cysts are present in roughly twothirds of all patients figures 15. Usual interstitial pneumonia an overview sciencedirect.
Coexistent lymphoid interstitial pneumonia, pernicious. Symptoms and signs are cough, progressive dyspnea, and crackles. Differential diagnosis pneumocystis jiroveci pneumonia pulmonary langerhans cell histiocytosis lymphangioleiomyomatosis birthogge dube syndrome discussion. Recurrent bacterial pneumonia is a rare complication of lymphoid interstitial pneu monia lip in hiv positive patients, and should be considered in the differential as a possible precipitator of recurrent pneumonia inan hiv po sitive patient. Hivrelated lymphocytic interstitial pneumonia lip may be part of a continuum of lymphocytic infiltrative disorders, such as pulmonary lymphoid hyperplasia in children and radiographically clear lymphocytic alveolitis in adults. Lymphocytic interstitial pneumonitis radiology reference. Lymphoid interstitial pneumonia in adults uptodate. Lymphocytic interstitial pneumonia information including symptoms, causes, diseases, symptoms, treatments, and other medical and health issues. Reversed halo sign on computed tomography as a presentation of lymphocytic interstitial pneumonia freeman et al. However hpe of the biopsied lung tissue is interstitial lymphoid pneumonia. Final diagnosis lymphocytic interstitial pneumonia upmc.
Corticosteroids are usually used to treat symptomatic patients but their efficacy has never been studied in a controlled trial. The clinical characteristics of druginduced interstitial lung disease ild due to elotuzumab have not been clarified. Lymphocytic interstitial pneumonia lip is a rare form of interstitial lung disease usually associated with other systemic diseases. It is regarded as part of the spectrum of pulmonary lymphoproliferative disorders, ranging in severity benign. Importance lymphocytic interstitial pneumonia in the adult is a benign disorder characterized by infiltration of the pulmonary interstitium by lymphocytes and plasma cells 1, 2. Lip may be associated with autoimmune and lymphoproliferative disorders, including rheumatoid arthritis, hashimoto thyroiditis, myasthenia gr. Lymphocytic interstitial pneumonitis lip is a benign lymphoproliferative disorder characterized by lymphocyte predominant infiltration of the lungs. Lymphocytic interstitial pneumonitis definition of. It details the potential of the lung to formulate an immune. Bye, in pediatric respiratory medicine second edition, 2008. Follicular bronchiolitis and lymphocytic interstitial. Pediatric lymphocytic interstitial pneumonia lip and follicular bronchiolitis fb are poorly characterized lymphoproliferative disorders. Frontiers of pulmonary radiology, 1st edition, 1969 in american thoracic society european respiratory society classification of the idiopathic interstitial pneumonia, idiopathic lip is categorized as a. Lymphocytic interstitial pneumonia pulmonary disorders merck.
Lymphocytic interstitial pneumonitis lip is a morphological subdivision of interstitial lung disease which is considered by some authors to represent a. Lymphoid interstitial pneumonia lip is regarded as both a disease and a nonneoplastic, inflammatory pulmonary reaction to various external stimuli or systemic diseases. The study included 17 patients with lymphocytic interstitial pneumonia and 44 patients with malignant lymphoma 35 with nonhodgkins lymphoma and nine with hodgkins disease. Lymphocytic interstitial pneumonia medical condition. Lymphocytic interstitial pneumonia libre pathology. Lymphocytic interstitial pneumonia an overview sciencedirect. In this case, lung biopsy under ct guidance was performed because unable to exclude primary lung carcinoma of the right upper lobe nodule which has spiculated margin.
Interstitial pneumonia with autoimmune features ipaf and. Lymphoid interstitial pneumonia another consideration for recurrent bacterial pneumonia in. We are not aware of an association between the former two disorders and any the chronic idiopathic interstitial pneumonias. Lymphocytic interstitial pneumonia applies to a disorders associated with both, monoclonal or polyclonal gammopathy. Lymphocytic interstitial pneumonia lip is a syndrome of fever, cough, and dyspnea, with bibasilar pulmonary infiltrates consisting of dense. Gerald j berry md robert v rouse md department of pathology stanford university school of medicine stanford ca 943055342. In this report, we describe a patient with refractory multiple myeloma who received elotuzumab in combination with lenalidomide and dexamethasone in whom fatigue, fever and diffuse pulmonary infiltration developed. The disease is more common in middleaged women 1,2,3,4. Lymphocytic interstitial pneumonia in children uptodate. Lymphocytic interstitial pneumonia and pulmonary lymphoid hyperplasia. It is classified as a subtype of interstitial lung disease. Lymphocytic interstitial pneumonitis associated with epstein barr. Lymphocytic interstitial pneumonia pulmonary disorders.
The differential diagnosis of dlpd in the context of cvid is diverse and includes infection, hypersensitivity pneumonitis, cryptogenic organizing pneumonia, lymphoma and a constellation of radiologicalpathological findings known as granulomatous and lymphocytic interstitial lung disease glild 1,8,9. Lymphocytic interstitial pneumoniapneumonitis lip is currently thought to be a diffuse hyperplasia of the bronchialassociated lymphoid tissue. Interstitial lung disease ild, or diffuse parenchymal lung disease dpld, is a group of lung diseases affecting the interstitium the tissue and space around the alveoli air sacs of the lungs. Lymphocytic interstitial pneumonia lip is an uncommon histopathologic entity characterized by infiltration of the interstitium and alveolar spaces of the lung by lymphocytes, plasma cells, and other lymphoreticular elements. Lymphocytic interstitial pneumonia lip and pulmonary lymphoid hyperplasia plh represent a spectrum of chronic lymphocytic infiltrative diseases of the lungs, occurring commonly in hivinfected. Immunohistochemical study denied lymphocyte monoclonality, and he was diagnosed as lymphocytic interstitial pneumonia lip. It was initially described in 1966, prior to the hiv era, by carrington and liebow and remains a rare form of interstitial lung disease in hiv. The study group consisted of 15 subjects encountered over a 14yr period. Lymphoid interstitial pneumonia lip is rare and its clinical course incompletely described. Lymphocytic interstitial pneumonia clinics in chest medicine. Lymphocytic interstitial pneumonitis lip the liver and the lung. Classified into 8 histologic subtypes, all are characterized by varying degrees of inflammation and fibrosis and all cause dyspnea. Lymphocytic interstitial pneumonia lip remains in the differential diagnosis for interstitial lung disease ild in childhood, although it is actually a form of pulmonary lymphoproliferative disease.
Histopathological lip is a form of interstitial pneumonia that is uncommonly seen and. Hrct at the level of the upper lobes exhibits a mixed groundglass and cystic pa. We present and quantify demographics, radiological and histopathologic patterns, treatments and their responses, and outcomes in non. Lymphocytic interstitial pneumonia in a female patient. Lymphocytic interstitial pneumonitis is a pattern of interstitial lung disease characterized by a polymorphous inflammatory infiltrate that diffusely expands alveolar septa. Lymphocytic interstitial pneumonia lip is a rare form of interstitial lung disease. Although glild is the most common cause of dlpd in cvid, the histopathological features of this disorder are incompletely characterized. Lymphocytic interstitial pneumonia lip images, diagnosis. Lymphocytic interstitial pneumonia in a female patient with sjogrens disease. Temporal heterogeneity refers to the variegated appearance of the lung biopsy in uip, where areas of advanced fibrosis are seen adjacent to entirely normal lung, with interspersed areas of active fibroblastic proliferation known as fibroblastic foci. Gurney, md, facr key facts terminology lymphoproliferative disorder ranging from follicular bronchiolitis to lowgrade lymphoma diffuse disease commonly referred to as lip focal disease commonly referred to as pseudolymphoma imaging findings radiographic findings centered on lymphatic pathways in lung groundglass. It is an uncommon condition with incidence and prevalence rates that are largely unknown. People usually cough and have difficulty breathing. First described in 1966 by carrington and liebow, lip is one.
Pdf lymphoid interstitial pneumonia lip is rare and its clinical course incompletely described. Lip may be part of a spectrum with other relatively benign lymphoid disorders of the lung, which include follicular bronchiolitis, intraparenchymal. Lymphocytic interstitial pneumonia applies to disorders associated with both monoclonal or polyclonal gammopathy. A biopsy of the largest nodule revealed fragments of lung parenchyma with lymphocytic infiltrate and proteinaceous fibrin filling the alveolar spaces, with. Lymphocytic interstitial pneumonia lip, a clinico pathologic condition, involves an inflammatory pulmonary reaction of the bronchusassociated lymphoid. It concerns alveolar epithelium, pulmonary capillary endothelium, basement membrane, and perivascular and perilymphatic tissues.
Lymphocytic interstitial pneumonia lip is a condition in which the lungs are diffusely infiltrated by benign lymphoid elements. The aim of this study was to examine the clinical features, associations and prognosis of surgical lung biopsyproven lip. It showed a diffuse severe infiltration of lymphocytes in the alveolar walls and a few epithelioid granulomas. Affected individuals generally have concurrent systemic disease, most frequently some form of immunologic dysfunction. Pdf lymphocytic interstitial pneumonitis lipthe liver. Interstitial pneumonia with autoimmune features ipaf is a term to describe individuals with both interstitial lung disease ild and combinations of other clinical, serologic, andor pulmonary morphologic features, which presumably originate from an underlying systemic autoimmune condition, but do not meet current rheumatologic criteria for a defined connective tissue. It may occur when an injury to the lungs triggers an. Ten of the patients had an associated dysproteinemia, including two patients with hypogammaglobulinemia. Because patients with lip respond well to steroid treatment, it is important to exclude other diseases to achieve a reliable diagnosis of lip.
Acute interstitial pneumonia diffuse alveolar damage acute fibrinous and organizng pneumonia. Overview of idiopathic interstitial pneumonias pulmonary. Cryptogenic organizing pneumonia boop desquamative interstitial pneumonia. Lymphocytic interstitial pneumonia and pulmonary amyloidosis in. Lung windowed axial a, coronal b, and sagittal c computed tomography images demonstrate a. Lymphocytic interstitial pneumonia lip, one of the rarest forms of interstitial lung disease ild, shares manifestations with a variety of other diseases, making it difficult to diagnose. The purpose of this study was to compare highresolution ct findings of lymphocytic interstitial pneumonia with those of malignant lymphoma of the chest. Symptoms include fever, cough, and shortness of breath. Lymphoid interstitial pneumonia lip is a rare form of interstitial pneumonia. Granulomatous and lymphocytic interstitial lung disease glild. Lymphocytic interstitial pneumonitis radiology key.
Pathology outlines lymphoid interstitial pneumonia lip. May progress to interstitial fibrosis and honeycomb change. Lymphocytic interstitial pneumonitis surgical pathology. Frontiers of pulmonary radiology, 1st edition, 1969. Ibrutinibinduced pneumonitis in patients with chronic. In 1969, leibow and carrinton first described lymphoid interstitial pneumonia lip as interstitial lung disease with diffuse and dense lymphocytic infiltration simon. Idiopathic interstitial pneumonias iips are interstitial lung diseases of unknown etiology that share similar clinical and radiologic features and are distinguished primarily by the histopathologic patterns on lung biopsy. Lymphoid interstitial pneumonia another consideration for. There is a paucity of clinical and histopathological data about hivassociated lymphocytic interstitial pneumonitis lip in adults from hiv. Lymphocytic interstitial pneumonia lip is a syndrome of fever, cough, and dyspnea, with bibasilar pulmonary infiltrates consisting of dense interstitial accumulations of lymphocytes and plasma cells. Lymphoid interstitial pneumonia lip is an uncommon form of interstitial lung disease in adults that is characterized histopathologically by infiltration of the interstitium and alveolar spaces of the lung by lymphocytes, plasma cells, and other lymphoreticular elements. Importantly, imaging abnormalities correlated with findings obtained from transbronchial biopsy specimens, which can be nonspecific in druginduced pneumonitis, but consistently revealed patterns of organizing pneumonia, interstitial inflammatory infiltrates, or granulomas that did not stain positive for acidfast bacilli or fungi. One year later, his condition deteriolated, and a large open biopsy was performed. Diagnosis is based on history, physical examination, imaging tests, and lung biopsy.