Search PubMed⌕ Search

Biomedical subjects

R A DeRemee

Publications and source records attributed to R A DeRemee.

At least 19 recordsLinked to original sources
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↗
PubMed1990

Clinical implications of the histopathologic diagnosis of pulmonary lymphomatoid granulomatosis.

We reviewed the epidemiologic, laboratory, roentgenographic, pulmonary function, and survival data from 28 patients who had a histologic diagnosis of lymphomatoid granulomatosis (LG) with involvement of the lungs. The mean age at the time of diagnosis was 51 years, and the male-to-female ratio was 3:2. Ten patients had other underlying diseases before LG was diagnosed. The most prominent symptoms were cough, dyspnea, fever, and rash, which were usually present for several months before diagnosis of LG. Multiple nodules were detected on a chest roentgenogram in 68% of the patients. Immunoglobulin concentrations were abnormal in 8 of 12 patients studied. Although bronchoscopy established the diagnosis in approximately a third of the patients who underwent this procedure, open-lung biopsy was uniformly diagnostic. The median survival was 72 months, with follow-up through 12 years. In 11 patients, the original diagnosis of LG was eventually changed to lymphoma. In five of these patients, the change in diagnosis was based on immunohistologic data obtained shortly after LG was discovered. Lymphoma diagnosed in this way was associated with a better prognosis than lymphoma diagnosed on the basis of conventional histopathologic findings. In three patients, solid tumors eventually developed. The diversity of clinical outcomes and frequent revisions of the diagnosis led us to consider the possibility that LG may also represent a histopathologic finding that occurs transiently in several disease processes.

Adult↗
PubMed1990

Granulomatous vasculitis. Wegener's granulomatosis and Churg-Strauss syndrome.

Wegener's granulomatosis and the Churg-Strauss syndrome are both syndromes that appear to begin with a phase of regionally limited symptomatology before they progress at unpredictable rate to a generalized phase characterized by symptoms of systemic vasculitis. The clinical features of atopy, peripheral blood eosinophilia, and tissue eosinophilia distinguish CSS from WG, with its typical necrotizing granulomatous respiratory tract lesions. Whereas in generalized WG with renal involvement the use of cyclophosphamide usually cannot be avoided, the generalized systemic vasculitis phase of CSS appears to respond well to glucocorticoids alone. For the more limited forms of WG, adapted therapy regimens including trimethoprim-sulfamethoxazole have been reported to be successful. Anticytoplasmic autoantibodies (c-ANCA = ACPA) are a new diagnostic serum test with high specificity for WG. Serial determinations of c-ANCA are a promising tool to monitor disease activity.

Churg-Strauss Syndrome↗
PubMed1989

Anticytoplasmic autoantibodies: their immunodiagnostic value in Wegener granulomatosis.

STUDY OBJECTIVE: To determine disease specificity and sensitivity of anticytoplasmic autoantibodies (ACPA) for Wegener granulomatosis, as well as their value as a marker of disease activity. DESIGN: Blind analysis of serum samples, retrospective analysis of clinical data on patients, and prospective follow-up of a subgroup of patients with Wegener granulomatosis. PATIENTS: The study included 277 patients with Wegener granulomatosis (222 with biopsy-proven disease) and 1657 control patients. SETTING: University hospital and academic medical center. LABORATORY INVESTIGATIONS: Analysis of 2653 serum samples from 1934 patients for ACPA. Antibody detection was by indirect immunofluorescence and a new type of enzyme-linked immunoadsorbent assay (ELISA). Prospective follow-up was on 172 patients with Wegener granulomatosis. MEASUREMENTS AND MAIN RESULTS: Specificity of ACPA for Wegener granulomatosis measured by indirect immunofluorescence was 99% (CI, 98.9% to 99.7%) and 98% (CI, 97.4% to 99.2%) by ELISA. Sensitivity of ACPA depended on disease activity and extent: It was 67% (CI, 38% to 89%) by immunofluorescence and 60% (CI, 32% to 84%) by ELISA for patients with active locoregional symptomatology (n = 15); and 32% (CI, 14% to 54%) by immunofluorescence and 40% (CI, 21% to 61%) by ELISA for patients in full remission after initial locoregional symptoms (n = 25). The sensitivity was 96% (CI, 89% to 99%) by immunofluorescence and 93% (CI, 86% to 98%) by ELISA for patients with active generalized disease (n = 92). Serial testing was done; every patient with active generalized disease eventually had at least one positive serum sample. Sensitivity decreased to 41% (CI, 22% to 62%) by both immunofluorescence and ELISA for patients in full remission after active generalized disease (n = 27). Levels of ACPA expressed both as immunofluorescence titers and ELISA values (U/mL) correlated well with disease activity. CONCLUSIONS: Testing for ACPA in serum of patients with Wegener granulomatosis is valuable for differential diagnosis; furthermore, APCA can be used as a marker to follow disease activity. A new type of ELISA yielded the same results as indirect immunofluorescence for the specificity, sensitivity, and correlation with disease activity of ACPA.

Adolescent↗
PubMed1989

Two-dimensional echocardiographic findings in systemic sarcoidosis.

The records of 88 patients with systemic sarcoidosis who underwent 2-dimensional echocardiography were reviewed to define the typical echocardiographic abnormalities in patients with clinical evidence of cardiac involvement. Twelve patients (14%) had echocardiographic evidence of left ventricular systolic dysfunction attributed to cardiac sarcoidosis (segmental hypokinesis in 8 patients and global hypokinesis in 4 patients). A high incidence of congestive heart failure (9 of 12) and conduction system disease (7 of 12) was characteristic of these patients. The remaining 76 patients had normal echocardiograms or abnormalities attributed to nonsarcoid sources. In 8 (11%) of these 76 patients, there were significant clinically unexplained conduction abnormalities likely related to sarcoidosis.

Adult↗
PubMed1989

Anticytoplasmic autoantibodies in the diagnosis and follow-up of Wegener's granulomatosis.

Sixty-five patients with biopsy-proven Wegener's granulomatosis (WG), 54 with systemic vasculitis, 22 with relapsing polychondritis, 20 with sarcoidosis, 20 with malignant pulmonary lesions, and 15 with other conditions underwent determination of anticytoplasmic autoantibodies (ACPA) by the indirect immunofluorescence technique on neutrophil cytospin preparations to assess the specificity of ACPA for WG, their sensitivity in relationship to the extent and activity of the disease, and their value for follow-up of WG. Of these 65 patients with WG, 38 were ACPA positive. Two patients in the vasculitis group, best categorized as having microscopic polyarteritis, were ACPA positive. We obtained 125 serum samples from the 65 patients with WG and assigned them to one of two categories (limited or generalized), based on the extent of disease. Each of these categories was then subdivided into "active" or "in remission." Median ACPA titers were significantly different between active disease and remission in each category, as well as between active limited and active generalized disease. All patients whose disease changed from active to in remission had reductions in ACPA titer levels; those who experienced flares had titer increases. Patients with intercurrent illnesses or complications of treatment, mimicking WG flares, did not have titer increases. We conclude that ACPA determined by the indirect immunofluorescence technique is highly specific for WG. The sensitivity is dependent on the extent and activity of WG, and serial titer determinations are valuable in monitoring disease activity.

Adolescent↗
PubMed1988

Cyclophosphamide-induced bladder toxicity in Wegener's granulomatosis.

In a series of 111 patients with Wegener's granulomatosis who were given cyclophosphamide therapy, hemorrhagic cystitis, diagnosed on the basis of gross hematuria or at cystoscopy (or both), developed in 17 (15%). Most of these patients recovered uneventfully, with or without the discontinuation of cyclophosphamide, but 4 patients suffered a significant loss of blood, and bladder carcinoma developed in 3. New microhematuria also occurred in 52 patients (47%). The dose and duration of cyclophosphamide were greater in the group that had urotoxicity. Long-term followup of patients with hemorrhagic cystitis is mandatory for the detection of late recurrences or the development of bladder malignancy. New therapies are being directed at protecting the bladder from urotoxicity during cyclophosphamide treatment.

Administration, Oral↗
PubMed1988

The influence of diagnostic access bias on the epidemiology of sarcoidosis: a population-based study in Rochester, Minnesota, 1935-1984.

A population-based study was conducted among residents of Rochester, Minnesota from 1935-1984 to examine the influence of diagnostic access bias on the incidence and mode of presentation of clinically recognized sarcoidosis. Immigrants had an elevated risk of sarcoidosis and were more likely to present with asymptomatic sarcoidosis diagnosed on routine screening chest radiographs. Similar patterns were observed for health professionals, a group with markedly greater access to the diagnostic procedures which label them as having sarcoidosis. Sarcoidosis exhibits a spectrum of manifestations, ranging from a totally asymptomatic process discernible only by specific diagnostic scrutiny to a clinically obvious presentation with significant morbidity. For disease entities such as this, apparent patterns of occurrence may depend on differential access in the study population to the maneuvers necessary for diagnosis. Diagnostic access bias, in addition to other diagnostic biases, deserves more careful scrutiny in epidemiologic research.

Adolescent↗