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Biomedical subjects

R A DeRemee

Publications and source records attributed to R A DeRemee.

At least 19 recordsLinked to original sources
PubMed1996

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. Epstein-Barr virus RNA was detected in the neoplastic cells in 29 of 30 patients. The most common presenting symptom was nasal obstruction followed by purulent rhinorrhea. Patients with early presentation had only a friable nasal or nasopharyngeal mucosa; late clinical signs included septal perforation in 40%. Twenty-one of 30 patients received radiation therapy as initial treatment; 22 of 30 patients achieved a complete remission. Fifteen patients relapsed: 10 with local recurrence and 5 with systemic disease. In long-term follow-up, 10 patients were alive and disease free, 6 patients died of unrelated causes, and 12 patients died of disease.

Adult↗
PubMed1996

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. In a 20-yr period six patients were identified with cyclophosphamide-induced lung disease, including five men and one woman ranging in age from 42 to 78 yr. Clinical features of toxicity include dyspnea, fever, cough, new parenchymal infiltrates, gas exchange abnormalities on pulmonary function tests, and pleural thickening on chest roentgenogram. Two patterns of cyclophosphamide-induced lung toxicity were identified. A single patient presented with early-onset pneumonitis and responded to discontinuation of the drug. Five patients with late-onset pneumonitis developed progressive pulmonary fibrosis associated with bilateral pleural thickening. Patients with late-onset pneumonitis showed no response to cessation of cyclophosphamide and institution of corticosteroid therapy. Three of these patients died of respiratory failure. Careful review of the individual cases reported in the literature as cyclophosphamide lung toxicity revealed only 12 cases in whom none of the additional confounding factors could be identified. These could easily be divided in the same two categories. Early-onset pneumonitis is reversible and may respond to corticosteroid therapy. Late-onset pneumonitis, frequently associated with pleural thickening, is clinically distinct from idiopathic pulmonary fibrosis but has a chronically progressive course. It appears unresponsive to corticosteroid therapy.

Adult↗
PubMed1996

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. Entities previously confused with pulmonary vasculitis include lymphomatoid granulomatosis or polymorphic reticulosis, and benign lymphocytic angiitis and granulomatosis, which are probably in the spectrum of T-cell lymphomas. Necrotizing sarcoid and sarcoidosis can involve blood vessels, but do not follow a typical course associated with the traditional concept of vasculitis.

Churg-Strauss Syndrome↗
PubMed1995

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. Nine patients with ulcerating tracheobronchitis on initial study had subsequent bronchoscopies for continued symptoms, which in seven cases documented the progression from ulcerating tracheobronchitis to stenosis without inflammation. Bronchoscopic interventions included dilation by rigid bronchoscope in three, YAG-laser treatment in one, and placement of silastic airway stents in three patients. Only the stents provided persistent airway patency. Endobronchial biopsies were performed on 21 occasions in 17 patients. Half of the specimens were helpful in establishing the diagnosis and in all but three in assessing disease activity. While antineutrophil cytoplasmic antibody titers reflect overall disease activity, no correlation with endobronchial inflammatory activity was apparent.

Adult↗
PubMed1995

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. The diagnosis should be confirmed by histopathologic examination and exclusion of known causes for the noncaseous granulomatous reaction. When indicated, treatment with alternate-day regimens of prednisone is highly effective. Serial chest roentgenography, pulmonary function studies, and the serum angiotensin-converting enzyme level are useful for monitoring the course of the disease.

Algorithms↗
PubMed1995

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). RESULTS: Of the 47 patients, 29 (62%) had neurologic involvement. Peripheral neuropathy was detected in 25 patients: 17 had multiple mononeuropathy, 7 had distal symmetric polyneuropathy, and 1 had an asymmetric polyneuropathy. Three patients had cerebral infarctions. Less commonly identified problems included radiculopathies, ischemic optic neuropathy, and bilateral trigeminal neuropathy. Asthma preceded the onset of neurologic involvement in all cases (mean duration, 6.7 years. Follow-up data, when available, showed that corticosteroid therapy usually yielded improvement or stabilization. CONCLUSION: Neurologic involvement is common in CSS, usually manifesting as peripheral neuropathy. In this series of patients, asthma preceded the neurologic manifestations.

Adolescent↗
PubMed1994

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. Maximal inspiratory pressure was decreased, and the diffusing capacity of the lungs was abnormal. Computed tomography of the chest showed no parenchymal or mediastinal abnormalities. During a sniff test, fluoroscopy of her diaphragm disclosed paradoxical motion of both hemidiaphragms during inspiration, consistent with bilateral hemidiaphragmatic paralysis. Parsonage-Turner syndrome was diagnosed. The dose of glucocorticoids was tapered. Follow-up of the patient by telephone contact in March 1994 (9 months after her initial examination at our clinic) revealed that the dyspnea was still severe.

Asthma↗
PubMed1994

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. We summarized the clinical findings in MPO-positive and MPO-negative patients. RESULTS: Sera were positive for p-ANCA in 42 of 2,381 patients (1.8%). In 13 of these 42 patients (31%), the respiratory tract was involved. Twelve patients had chest roentgenographic abnormalities, including a diffuse alveolar filling pattern (N = 8), a diffuse interstitial pattern (N = 2), and a combined interstitial and alveolar pattern (N = 2); three others had nasal inflammation. Ten of 16 sera tested were positive for MPO, and proteinase 3 antibodies were detected in 1 specimen. All patients with alveolar hemorrhage were positive for MPO antibodies. CONCLUSION: Testing for p-ANCA by immunofluorescence microscopy discloses a wide range of clinical disorders distinct from the main cytoplasmic-staining ANCA-associated disease--namely, Wegener's granulomatosis. In particular, the respiratory tract is affected much less frequently. Further evaluation of positive p-ANCA immunofluorescence test results by enzyme-linked immunosorbent assay to determine whether MPO is the target antigen is necessary to obtain clinically useful information from this test.

Adolescent↗
PubMed1994

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. METHODS: A retrospective analysis was conducted of 244 cases of WG observed between 1988 and 1992. RESULTS: Skin involvement occurred in 14% of the patients and was more frequent in generalized WG. Skin lesions may be an early premonitory sign of renal disease. Necrotizing ulcerations resembling pyoderma gangrenosum were not uncommon. Leukocytoclastic vasculitis was the most common cutaneous pathologic pattern. Findings of c-ANCA were positive in 81% of patients with cutaneous WG. CONCLUSION: Skin involvement usually occurred at presentation with generalized disease. c-ANCA is a valuable adjunct to diagnosis and follow-up of WG.

Adolescent↗
PubMed1994

Immunohistochemical localization of transforming growth factor-beta 1 in the nonnecrotizing granulomas of pulmonary sarcoidosis.

Sarcoidosis is a chronic inflammatory disease of unknown cause characterized by the formation of nonnecrotizing granulomas in affected tissues, most notably the lungs. Granuloma healing may result in pulmonary fibrosis and respiratory impairment in some patients. Transforming growth factor-beta 1 (TGF-beta 1) is a potent cytokine that promotes fibrosis by enhancing the synthesis of extracellular matrix components, including fibronectin and the alpha 5 beta 1 fibronectin receptor. The role of TGF-beta 1 in promoting lung fibrosis in the setting of pulmonary sarcoidosis has not yet been investigated. Accordingly, we determined the extent and distribution of TGF-beta 1 in lung tissue obtained from seven patients with clinical and histologic features of pulmonary sarcoidosis. The tissue distributions of TGF-beta 1, the TGF-beta 1 binding proteoglycan decorin, fibronectin, and the alpha 5 beta 1 fibronectin receptor were assessed immunohistochemically. In all cases, the epithelioid histiocytes comprising nonnecrotizing granulomas of pulmonary sarcoidosis contained abundant TGF-beta 1. We further demonstrated decorin, fibronectin, and the alpha 5 beta 1 fibronectin receptor within nonnecrotizing granulomas and in the fibrous tissue surrounding the lesions. TGF-beta 1 staining was also observed in bronchiolar epithelial cells, hyperplastic Type II pneumocytes, and occasional alveolar macrophages. This study demonstrates enhanced tissue localization of TGF-beta 1 and related extracellular matrix proteins associated with the nonnecrotizing granulomas of pulmonary sarcoidosis. Through its actions on matrix protein synthesis, TGF-beta 1 may modulate the fibrotic repair process accompanying granuloma healing in sarcoidosis.

Adult↗
PubMed1994

Racial difference in cardiac sarcoidosis incidence observed at autopsy.

The most commonly involved organ in sarcoid related death has been reported to be the lung in western countries, while it was the heart in the Japanese autopsy series. In order to verify this different pattern of death, autopsy records were reviewed in two United States institutions and in Japanese nation-wide data. In Japanese, male to female ratio was 1:2 and a high frequency of sarcoidosis in females over 50 years of age was observed. Male to female ratio in Caucasian sarcoidosis autopsy was nearly 1:1. In Afroamericans, it was nearly 1:2, and the age distribution showed that this female predominance was due to a high number of sarcoidosis in females over 40 years of age. The frequency of sarcoidosis in Caucasian autopsies was higher than in Japanese autopsies. The number of involved organs was smaller in Caucasians than in Afroamericans or in Japanese. The incidence of cardiac sarcoid granuloma in Japanese was significantly higher than that seen in Caucasians and Afroamericans. The commonest cause of death in sarcoidosis of Caucasians and Afroamericans was from non-sarcoid diseases, while the major cause of sarcoid-attributed death was pulmonary sarcoidosis in Afroamericans. Japanese showed the highest rate of cardiac sarcoid-attributed death. Possible factors relating to this high incidence of sarcoidosis in elderly females and the racial difference in cardiac sarcoidosis were discussed.

Adolescent↗
PubMed1994

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. Both entities respond to glucocorticoids, but WG frequently requires cytotoxic agents such as cyclophosphamide. The current concepts of pathogenesis suggest sarcoidosis to involve mainly T-cell mediated mechanisms, whereas in the case of WG the presence of antineutrophil cytoplasmic autoantibodies (ANCA) points toward humeral mechanisms, although T-cells may also be involved.

Bone Diseases↗
PubMed1993

Neurological involvement in Wegener's granulomatosis: an analysis of 324 consecutive patients at the Mayo Clinic.

Neurological involvement in Wegener's granulomatosis was studied by reviewing the charts of 324 consecutive patients in whom the diagnosis was made at the Mayo Clinic. One hundred nine patients (33.6%) had neurological involvement. Peripheral neuropathy occurred in 53; cranial neuropathy, in 21; external ophthalmoplegia, in 16; cerebrovascular events, in 13; seizures, in 10; cerebritis, in 5; and miscellaneous involvement, in 25. The mean age and sex ratio were similar in the patients with and those without neurological involvement. Among the patients with peripheral neuropathy, 42 had mononeuropathy multiplex; 6, distal symmetrical polyneuropathy; and 5, unclassified peripheral neuropathy. Multiple mononeuropathy was a major presenting symptom in 8 patients. A significantly higher percentage of patients with peripheral neuropathy, compared to those without peripheral neuropathy, had kidney involvement (p < 0.001). The second, sixth, and seventh cranial nerves were most frequently affected. Multiple cranial nerves were affected in 8 patients. Unusual neurological manifestations in the miscellaneous group were spastic paraparesis, temporal arteritis, Horner's syndrome, and papilledema.

Adolescent↗