Colchicine versus prednisone in the treatment of idiopathic pulmonary fibrosis. A randomized prospective study. Members of the Lung Study Group.

Twenty-six symptomatic subjects with clinical evidence plus either high-resolution computed tomography (HRCT, n = 25) or open-lung biopsy (OLB, n = 1) patterns typical for idiopathic usual interstitial pneumonia (idiopathic UIP) were entered into a randomized prospective treatment trial using high-dose prednisone (n = 12) versus colchicine (n = 14). The minimum dose of prednisone used was 60 mg/d for 1 mo, tapered to 40 mg/d over the second month, tapered to 40 mg every other day during the third month, with subsequent doses adjusted as clinically indicated. The dose of colchicine was 0.

Colchicine versus prednisone as treatment of usual interstitial pneumonia.

Objective: To assess the results with colchicine and prednisone as initial single-drug therapy in patients with usual interstitial pneumonia (UIP).

Material And Methods: We reviewed the serial pulmonary function test results in 22 patients with typical clinical and high-resolution computed tomographic features of UIP who were treated with colchicine as initial single-agent therapy and compared them with a group of 22 historical patients with UIP of similar severity diagnosed by open-lung biopsy who were given prednisone as initial single-drug therapy.

Results: No significant difference was detected in the rate of decline of pulmonary function or in the time to "failure" between the two study groups.

Carcinoid tumors and sarcoidosis--does a link exist?

Objective: To attempt to determine whether a relationship exists between carcinoid tumors and sarcoidosis.

Material And Methods: We present a series of seven case reports and discuss hypotheses about possible disease associations.

Results: Certain malignant lesions have tended to occur in patients with sarcoidosis.

Lung toxicity associated with cyclophosphamide use. Two distinct patterns.

Cyclophosphamide-induced lung toxicity may be difficult to recognize because of the presence of confounding variables such as concomitant use of other cytotoxic drugs, opportunistic infections, diffuse pulmonary malignancy, radiation pneumonitis, and oxygen toxicity. The purpose of this retrospective analysis was to identify the clinical spectrum of pulmonary toxicity of cyclophosphamide. In our review of case records, we sought to identify patients in whom cyclophosphamide was the only identifiable etiologic factor for lung toxicity.

The spectrum of pulmonary vasculitis.

Wegener's granulomatosis and Churg-Strauss syndrome are the predominant pulmonary vasculitides. Next in frequency are the various diffuse alveolar haemorrhage syndromes, which may be related to the antineutrophil cytoplasmic autoantibody (ANCA)-associated diseases, such as Wegener's granulomatosis and Churg-Strauss syndrome, or may be a part of a collagen vascular disease, such as lupus erythematosus, or associated with antiglomerular basement membrane antibody (AGBM) and fall within the definition of Goodpasture's syndrome. Whereas Behçlet's disease and Takayasu's arteritis have major pulmonary manifestations, they are rare diseases.

Nasal and nasopharyngeal angiocentric T-cell lymphomas.

Thirty patients (24 mean and 6 women) with a median age of 44.5 years who had angiocentric T-cell lymphoma were studied. The neoplastic cells in each had a T-cell phenotype.

Wegener's granulomatosis.

Since its first description by Wegener in 1936, Wegener's granulomatosis has undergone significant changes in terms of clinical scope, diagnosis, and treatment. It is no longer tenable to insist on the fulfillment of the Wegener's triad to make the diagnosis. The wide range of clinical presentations is encompassed by the ELK (ear, nose, and throat; lung; kidney) classification in which any combination or singular involvement of the major sites can be considered within the Wegener's spectrum if supported by the appropriate pathologic findings or the presence of a cytoplasmic antineutrophil cytoplasmic antibody pattern.

Neurologic manifestations of Churg-Strauss syndrome.

Objective: To determine the frequency and the types of neurologic involvement in a series of patients with Churg-Strauss syndrome (CSS).

Design: We reviewed the medical records of 47 consecutive patients with CSS who were examined at the Mayo Clinic between January 1974 and June 1992.

Material And Methods: The study patients were classified into two groups: (1) those with a histopathologically confirmed diagnosis of CSS who had evidence of either vasculitis or Churg-Strauss granuloma, the presence of asthma, and peripheral eosinophilia (more than 10% eosinophils) on at least one differential leukocyte count (N = 33) and (2) those with a clinical diagnosis of CSS who had evidence of vasculitis based on either multiple mononeuropathy or necrotizing cutaneous lesions, the presence of asthma, and peripheral eosinophilia (more than 10% eosinophils) on at least one differential leukocyte count (N = 14).

Sarcoidosis.

Sarcoidosis is a systemic granulomatous process of unknown cause. Pathologically, it is characterized by noncaseous granuloma, and in more than 90% of patients, the lung or intrathoracic lymph nodes are affected. A roentgenographic staging system (stages I, II, and III), based on the appearance of the plain chest roentgenogram, conveys important information about prognosis, degree of symptoms, and pulmonary function abnormalities.

Tracheobronchial involvement in Wegener's granulomatosis.

This study was designed to characterize the clinical spectrum and course of tracheobronchial involvement in Wegener's granulomatosis (WG). Of the 51 patients with biopsy-proven WG who underwent bronchoscopy at least once at our institution between January 1982 and November 1993, 30 (59%) had endobronchial abnormalities due to WG. Initial findings included subglottic stenosis in five (17%), ulcerating tracheobronchitis with or without inflammatory pseudotumors in 18 (60%), tracheal or bronchial stenosis without inflammation in four (13%), and hemorrhage without identifiable source in two (4%) patients.

Cutaneous Wegener's granulomatosis: clinical, histopathologic, and immunopathologic features of thirty patients.

Background: Wegener's granulomatosis (WG) is a systemic disease characterized by necrotizing granulomatous inflammation and vasculitis. Its cutaneous manifestations vary.

Objective: We reviewed and characterized the clinical, pathologic, and immunopathologic features of the specific cutaneous manifestations of WG and investigated the sensitivity and the specificity of anti-neutrophilic cytoplasmic antibody (c-ANCA) in the cutaneous manifestations of this disease.

Lesions of the respiratory tract associated with the finding of anti-neutrophil cytoplasmic autoantibodies with a perinuclear staining pattern.

Objective: To characterize the clinicopathologic spectrum of respiratory tract involvement in patients with positive results of immunofluorescence microscopy for anti-neutrophil cytoplasmic autoantibodies with a perinuclear staining pattern (p-ANCA) and to assess the clinical value of p-ANCA testing.

Design: We retrospectively reviewed the medical records of all patients at Mayo Clinic Rochester in whom p-ANCA were detected by indirect immunofluorescence microscopy during 1992.

Material And Methods: Additional target antigen identification was performed with use of enzyme-linked immunosorbent assays for antibodies against myeloperoxidase (MPO) and proteinase 3.

Severe asthma complicated by bilateral diaphragmatic paralysis attributed to Parsonage-Turner syndrome.

Progressive dyspnea that developed in a 52-year-old woman with a lifelong history of asthma did not respond to high-dose orally administered glucocorticoids. Initially, a diagnosis of allergic bronchopulmonary aspergillosis or hypersensitivity pneumonia was suggested as the cause of the worsening dyspnea. Pulmonary function tests demonstrated severe airway obstruction; substantial improvement was noted after bronchodilator therapy.

Sarcoidosis and Wegener's granulomatosis: a comparative analysis.

Sarcoidosis and Wegener's granulomatosis (WG) share membership in the family of granulomatous diseases. Both are systemic processes that have major expressions in common sites, such as the respiratory tract, kidneys, skin, nervous system, eye and orbit, musculoskeletal system, and heart. In spite of these common features, they are sharply divergent in clinical course and pathology, sarcoidosis being a more benign, indolent disease of low mortality, in contrast to the highly lethal more dramatic course of WG.