Despite the considerable progress in the classification of the idiopathic interstitial pneumonias (IIPs), the lack of an international standard has resulted in variable and confusing diagnostic criteria and terminology. The advent of high-resolution computerized tomography, the narrowed pathologic definition of usual interstitial pneumonia (UIP) and recognition of the prognostic importance of separating UIP from other IIP patterns have profoundly changed the approach to the IIPs. This is an international Consensus Statement defining the clinical manifestations, pathology, and radiologic features of patients with IIP. The major objectives of this statement are to standardize the classification of the idiopathic interstitial pneumonias (IIPs) and to establish a uniform set of definitions and criteria for the diagnosis of IIPs. The targeted specialties are pulmonologists, radiologists, and pathologists. A multidisciplinary core panel was responsible for review of background articles and writing of the document. In addition, this group reviewed the clinical, radiologic, and pathologic aspects of a wide spectrum of cases of diffuse parenchymal interstitial lung diseases to establish a uniform and consistent approach to these diseases and to clarify the terminology, definitions, and descriptions used in routine clinical practice. The final statement was drafted after a series of meetings of the entire committee. The level of evidence for the recommendations made in this statement is largely that of expert opinion developed by consensus. This classification of IIPs includes seven clinico-radiologic-pathologic entities: idiopathic pulmonary fibrosis (IPF), nonspecific interstitial pneumonia, cryptogenic organizing pneumonia, acute interstitial pneumonia, respiratory bronchiolitis-associated interstitial lung disease, desquamative interstitial pneumonia, and lymphoid interstitial pneumonia. The need for dynamic interaction between pathologists, radiologists, and pulmonologists to accurately diagnose these disorders is emphasized. The level of evidence for the recommendations made in this Statement is largely that of expert opinion developed by consensus. This Statement is an integrated clinical, radiologic, and pathologic approach to the classification of the IIPs. Use of this international multidisciplinary classification will provide a standardized nomenclature and diagnostic criteria for IIP. This Statement provides a framework for the future study of these entities. Key Messages * Unclassifiable interstitial pneumonia : Some cases are unclassifiable for a variety of reasons (see text). † This group represents a heterogeneous group with poorly characterized clinical and radiologic features that needs further study. ‡ COP is the preferred term, but it is synonymous with idiopathic bronchiolitis obliterans organizing pneumonia.

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We report findings in 70 patients with both diffuse interstitial lung disease and either polymyositis (PM) or dermatomyositis (DM). Initial presentations were most commonly either musculoskeletal (arthralgias, myalgias, and weakness) or pulmonary (cough, dyspnea, and fever) symptoms alone; in only 15 patients (21.4%) did both occur simultaneously. Pulmonary disease usually took the form of acute to subacute antibiotic-resistant community-acquired pneumonia. Chest radiographs and computed tomography most commonly demonstrated bilateral irregular linear opacities involving the lung bases; occasionally consolidation was present. Jo-1 antibody was present in 19 (38%) of 50 patients tested. Synchronous associated malignancy was present in 4 of 70 patients (5.7%). Surgical lung biopsies disclosed nonspecific interstitial pneumonia (NSIP) in 18 of 22 patients (81.8%), organizing diffuse alveolar damage (DAD) in 2, bronchiolitis obliterans organizing pneumonia (BOOP) in 1, and usual interstitial pneumonia (UIP) in 1. Treatment usually included prednisone in 40-60 mg/d dosages for initial control, followed by lower dose prednisone plus an immunosuppressive agent such as azathioprine or methotrexate for disease suppression. Survival was significantly better than that observed for historical control subjects with idiopathic UIP, and was more consistent with survival previously reported in idiopathic NSIP. There was no difference in survival between Jo-1 positive and Jo-1 negative groups.

It is commonly believed that acetylcholine (ACh) is the physiological transmitter of sympathetic nerve impulses at the adrenal medulla. The reasons are the following: the sympathetic nerve fibres that innervate the adrenal medullary cells are developmental homologues of the preganglionic fibres elsewhere in the sympathetic nervous system which are known to be cholinergic; on stimulation of the adrenal nerves an acetylcholine-like substance is released from the adrenal gland (Feldberg, Minz & Tsudzimura, 1934); ACh is a powerful stimulant of adrenal medullary secretion; and finally, transmission at the adrenal medulla is affected by a variety of drugs in much the same way as these drugs affect transmission at the established sites of cholinergic transmission at sympathetic ganglionic synapses.The purpose of the present experiments was to study the mechanism by which ACh brings about adrenal medullary secretion and, as a first approach, to observe how the response of the gland to ACh might be influenced by changes in the ionic composition of the extracellular environment. Our experiments show that the excitant action of ACh on the adrenal medulla is dependent on the presence of calcium, and suggest that ACh evokes adrenal medullary secretion by causing calcium ions to penetrate the adrenal medullary cells.

Although evidence has existed for many years that the sympathetic nerves innervating the adrenal medulla are cholinergic, it is only quite recently that a number of observations have been made which together provide a possible explanation of how acetylcholine, the chemical mediator, stimulates the chromaffin cells to secrete the catecholamines adrenaline and noradrenaline. The recent evidence has come from studies on perfused adrenal glands showing that the stimulant effect of acetylcholine involves some calcium-dependent process (Douglas & Rubin, 1961). Thus acetylcholine was found to be without effect on catecholamine release when calcium was omitted from the perfusion fluid, and its efficacy in releasing catecholamines was directly related to the extracellular calcium concentration over a wide range. Moreover, calcium itself, in conditions known to increase the permeability of cell membranes, proved to be an adequate stimulus for catecholamine secretion. These findings, considered along with the known ability of acetylcholine to increase membrane permeability at some other sites in the body where it also acts as a chemical transmitter, led us to suggest that acetylcholine evokes adrenal medullary secretion through some action on the chromaffin cell membranes leading to an increased uptake or influx of calcium ions, and that calcium ions provide the immediate stimulus for the release of catecholamines. Support for this idea has come from more recent studies showing that the rate of 45Ca uptake by the adrenal medulla is indeed increased by ACh (Douglas & Poisner, 1961, 1962.The present experiments have been carried out to examine further the role of calcium and other inorganic ions in the process we have termed 'stimulus-secretion coupling' at the adrenal medulla.

Clinical classification of OP is useful to predict clinical course and outcome. Cryptogenic OP most often was a symptomatic bilateral lung process that had an overall favorable prognosis with prolonged corticosteroid therapy. Patients with secondary OP had a high mortality rate when the disease was associated with predisposing conditions or drugs. Patients with asymptomatic focal OP had an excellent prognosis.

Usual interstitial pneumonia (UIP) is a specific histological pattern of interstitial pneumonia most often associated with the clinical syndrome of idiopathic pulmonary fibrosis (IPF). There is controversy regarding the use of surgical lung biopsy in the diagnosis of UIP, and the risk of lung biopsy in these patients is largely unknown. This study investigated the 30 day surgical mortality rate in patients undergoing surgical lung biopsy for UIP.Patients undergoing surgical lung biopsy over a 10-yr period from 1986–1995 with the ultimate diagnosis of UIP (with or without underlying connective tissue disease) were identified. Pathology, computed tomography, medical records, and survival were assessed.Ten of sixty patients with usual interstitial pneumonia were found to be dead within 30 days of surgical biopsy. All of these were patients with idiopathic UIP, unassociated with connective tissue disease (clinical condition of IPF).In conclusion, patients with usual interstitial pneumonia of the idiopathic type, who present with atypical features, may be at higher risk for death following surgical biopsy than patients presenting with more typical features or patients with other interstitial illnesses.

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