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R A DeRemee

Publications and source records attributed to R A DeRemee.

At least 37 records · Page 2Linked to original sources
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↗
PubMed1993

Wegener's granulomatosis associated with vasculitis of the temporal artery: report of five cases.

Between January 1973 and October 1991 at the Mayo Clinic, 5 of 345 patients with Wegener's granulomatosis initially had features suggestive of giant cell arteritis and subsequently were found to have biopsy-proven vasculitis of the temporal artery. All five patients were older than 60 years of age and had jaw claudication, sudden loss of vision, severe headache with or without diplopia, or polymyalgia rheumatica at the time of initial examination. The erythrocyte sedimentation rate was high at the time of onset of symptoms in four patients (and unavailable in one patient). A temporal artery biopsy specimen revealed giant cell arteritis in one patient and non-giant cell arteritis in four patients. All five patients subsequently had pulmonary and renal lesions characteristic of Wegener's granulomatosis, with typical histopathologic features on biopsy or positive cytoplasmic staining antineutrophil cytoplasmic antibodies. Thus, overlapping features of giant cell arteritis and Wegener's granulomatosis do occur in some patients.

Aged↗
PubMed1993

Colchicine in the treatment of pulmonary fibrosis.

There is no standard or known optimal treatment for idiopathic pulmonary fibrosis. Corticosteroids have been used with widely variable benefit. Colchicine has been reported to suppress fibroblast growth factors and to inhibit collagen deposition. A potential role has been proposed for colchicine in the treatment of fibrotic pulmonary diseases. We retrospectively assessed the outcome of 23 patients with idiopathic pulmonary fibrosis in whom colchicine was used as empiric treatment. Eighteen patients had received prior corticosteroid therapy. By clinical and pulmonary function criteria, five patients (22 percent) improved following colchicine, nine (39 percent) remained stable, and nine (39 percent) worsened. The average duration of follow-up was 22 months. Despite limitations of this retrospective analysis, colchicine may be of benefit in pulmonary fibrosis and should be considered for further clinical trials.

Adult↗
PubMed1992

Wegener granulomatosis.

Explore the source record for details and available documents.

Granulomatosis with Polyangiitis↗
PubMed1991

Salivary gland involvement in Wegener's granulomatosis.

Salivary gland involvement is a rare clinical feature of Wegener's granulomatosis. We report a series of five cases in which submandibular or parotid gland swelling was part of the initial manifestation of the disease. All patients had limited forms of Wegener's granulomatosis. The value of determination of serum anti-neutrophil cytoplasmic autoantibodies is demonstrated in four of the cases, and its use as a new diagnostic adjunct and promising tool to monitor disease activity is discussed. The use of trimethoprim-sulfamethoxazole as part of alternative treatment regimens for limited Wegener's granulomatosis is advocated.

Granulomatosis with Polyangiitis↗
PubMed1991

Antineutrophil cytoplasmic autoantibody-associated diseases: a pulmonologist's perspective.

The development of the antineutrophil cytoplasmic autoantibody (ANCA) test has lent support to the concept of Wegener's granulomatosis as a spectrum that may affect one or more of the major anatomic sites: upper respiratory tract (E), lung (L), or kidney (K). It is C-ANCA that has high specificity for Wegener's granulomatosis (WG). The sensitivity is highest in the fully expressed Wegener's triad, or ELK, and less in local regional disease. It is possible that isolated manifestations such as episcleritis, subglottic stricture, or diffuse alveolar hemorrhage associated with C-ANCA may be considered in the Wegener's spectrum, although traditional histopathologic criteria are lacking. Until specific etiologic agents are uncovered for the various ANCA-associated entities, classification schemes should continue to employ previously established clinicopathologic syndromes such as WG using the ANCA test to better define their clinical spectrums.

Antibodies, Antineutrophil Cytoplasmic↗
PubMed1990

Role of testing for anticytoplasmic autoantibodies in the differential diagnosis of scleritis and orbital pseudotumor.

Wegener's granulomatosis is a multisystem disease often with protean manifestations. Eye signs and symptoms can be prominent and may be the patient's initial complaint. For a definitive diagnosis of Wegener's granulomatosis, a tissue biopsy specimen must show vasculitis and necrotizing granuloma. The presence of anticytoplasmic autoantibodies in the serum of patients has been found to be highly specific for Wegener's granulomatosis and can considerably facilitate early diagnosis and be used to monitor disease activity. In two illustrative cases, the utility of this laboratory test in the differential diagnosis of scleritis and orbital pseudotumor is demonstrated.

Adult↗