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The most common diagnosis among patients with interstitial lung disease is idiopathic fibrosing interstitial pneumonia, formerly known in the United States as idiopathic pulmonary fibrosis (known in the United Kingdom as cryptogenic fibrosing alveolitis ) Historically, this diagnosis was based on clinical and radiographic criteria with only a small number of patients undergoing surgical lung biopsy When biopsies were obtained, the common element of fibrosis led to the grouping together of several histologic patterns under the category of idiopathic pulmonary fibrosis These distinct histopathologic features are now recognized as being associated with different natural histories and responses to therapy (see Table 9 15) Therefore, in the evaluation of patients with idiopathic interstitial lung disease, clinicians should attempt to identify specific disorders and reserve the terms idiopathic fibrosing interstitial pneumonia, or cryptogenic fibrosing alveolitis to denote only the histologic pattern of usual interstitial pneumonitis (UIP) Patients with idiopathic fibrosing interstitial pneumonia may have any of the histologic patterns described in Table 9 15 The first step in evaluation is to identify patients

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advanced fibrosis with destruction of normal lung architecture Conventional CT and high-resolution CT imaging reveal in greater detail the findings described on chest radiograph In some cases, high-resolution CT may be strongly suggestive of a specific pathologic process

Three diagnostic techniques are in common use: bronchoalveolar lavage, transbronchial biopsy, and surgical lung biopsy, either through an open procedure or using VATS Bronchoalveolar lavage may provide a specific diagnosis in cases of infection, particularly with P jiroveci or mycobacteria, or when cytologic examination reveals the presence of

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whose disease is truly idiopathic As indicated in Table 9 14, most identifiable causes of interstitial lung disease are infectious, drug-related, or environmental or occupational agents Interstitial lung diseases associated with other medical conditions (pulmonary-renal syndromes, collagen-vascular disease) may be identified through a careful medical history Apart from acute interstitial pneumonia, the clinical presentations of the idiopathic interstitial pneumonias are sufficiently similar to preclude a specific diagnosis Chest radiographs and high-resolution CT scans are occasionally diagnostic Ultimately, many patients with apparently idiopathic disease require surgical lung biopsy to make a definitive diagnosis The importance of accurate diagnosis is twofold First, it allows the clinician to provide accurate information about the cause and natural history of the illness Second, accurate diagnosis helps distinguish patients most likely to benefit from therapy Surgical lung biopsy may spare patients with UIP treatment with potentially morbid therapies The diagnosis of UIP can be made on clinical grounds alone in selected patients A diagnosis of UIP can be made with 90% confidence in patients over 65 years of age who have (1) idiopathic disease by history and who demonstrate inspiratory crackles on physical examination; (2) restrictive physiology on pulmonary function testing; (3) characteristic radiographic evidence of progressive fibrosis over several years; and (4) diffuse, patchy fibrosis with pleural-based honeycombing on high-resolution CT scan Such patients do not need surgical lung biopsy Note that the diagnosis of UIP cannot be confirmed on transbronchial lung biopsy since the histologic diagnosis requires a pattern of changes rather than a single pathognomonic finding Transbronchial biopsy may exclude UIP by confirming a specific alternative diagnosis Treatment of idiopathic fibrosing interstitial pneumonia is controversial No randomized study has demonstrated that any treatment improves survival or quality of life compared with no treatment Clinical experience suggests that patients with DIP or RB-ILD, nonspecific interstitial pneumonia (NSIP), or COP (see Table 9 15) frequently respond to corticosteroids and should be given a trial of therapy typically prednisone, 1 2 mg/kg/d for a minimum of 2 months The same therapy is almost uniformly ineffective in patients with UIP Since this therapy carries significant morbidity, experts do not recommend routine use of corticosteroids in patients with UIP There are a number of ongoing clinical trials of antifibrotic therapies, such as pirfenidone and interferon gamma-1b, as well as smaller investigations of sildenafil, thalidomide, and biologic modifiers.

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King TE Jr Clinical advances in the diagnosis and therapy of the interstitial lung diseases Am J Respir Crit Care Med 2005 Aug 1;172(3):268 79 [PMID: 15879420] Leslie KO Pathology of interstitial lung disease Clin Chest Med 2004 Dec;25(4):657 703 [PMID: 15564015] Lynch DA et al Idiopathic interstitial pneumonias: CT features Radiology 2005 Jul;236(1):10 21 [PMID: 15987960] Swigris JJ et al Idiopathic pulmonary fibrosis: challenges and opportunities for the clinician and investigator Chest 2005 Jan;127(1):275 83 [PMID: 15653995]

Symptoms related to the lung, skin, eyes, peripheral nerves, liver, kidney, heart, and other tissues Demonstration of noncaseating granulomas in a biopsy specimen Exclusion of other granulomatous disorders

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