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Biomedical subjects

Marc A Judson

Publications and source records attributed to Marc A Judson.

33 records · Page 2Linked to original sources

Noninvasive Aspergillus pulmonary disease.

Noninvasive Aspergillus pulmonary disease may be the result of a host response to Aspergillus antigens or colonization of the lung with Aspergillus species. The prototype example of each of these phenomena are allergic bronchopulmonary aspergillosis and aspergilloma. Allergic bronchopulmonary aspergillosis is a form of asthma associated with a Type I, III, and IV allergic response to Aspergillus antigens. It presents clinically as asthma but is also associated with bronchiectasis, airway destruction, and permanent lung injury if inadequately treated. Treatment consists of anti-inflammatory agents such as corticosteroids. Antifungal agents may also be useful to lower the fungal antigen load. Aspergillomas grow in areas of devitalized lung. They may manifest as asymptomatic radiographic abnormalities. The concern is that they may lead to hemoptysis that can be life threatening. Definitive treatment is surgical resection, but this is often prohibited because these patients often have inadequate lung function to tolerate thoracic surgery. Other treatment options include azoles and percutaneous instillation of antifungals.

Journal Article↗

Health-related quality of life of persons with sarcoidosis.

STUDY OBJECTIVES: To describe the health-related quality of life (HRQL) and mental health of persons with sarcoidosis, as well as to assess physician-patient agreement about the presence of sarcoidosis symptoms. DESIGN AND SETTING: Cross-sectional study at three university medical center outpatient pulmonary clinics. PATIENTS: One hundred eleven outpatients with sarcoidosis seen between March and July 2002. MEASUREMENTS: The HRQL of sarcoidosis patients was measured using generic and respiratory disease-specific forms (ie, Medical Outcomes Study 36-item short form survey [SF-36] and the St. George respiratory questionnaire [SGRQ], respectively). Depression was assessed using the Center for Epidemiologic Studies depression scale, and stress was assessed with the perceived stress scale four-item questionnaire. The kappa-statistic was calculated to compare physician-patient agreement in assessing sarcoidosis-related symptoms. RESULTS: Patients had moderate-to-severe reductions across all measured domains in perceived HRQL. Those patients who were prescribed oral corticosteroids had lower scores on both the SF-36 and the SGRQ than did those patients who were not. These differences were statistically significant and clinically important. The prevalence of depression was 66%, and that of significant stress was 55%. Spirometry values (FEV(1), 82% predicted; FVC, 86% predicted) were associated neither with HRQL nor with patients' perceived sarcoidosis symptoms, although they were correlated (r = 0.43; p < 0.0001) with physicians' perceptions that patients were symptomatic. Physicians and patients had only fair agreement (kappa-statistic range, 0.24 to 0.36 [by center]) in assessing perceived sarcoidosis symptoms. CONCLUSIONS: Outpatients with sarcoidosis had global reductions in measured HRQL and mental health indexes, although patients receiving therapy with oral corticosteroids had significantly worse HRQL. Experienced physicians based their assessments of patients' sarcoidosis symptoms on measures that were not related to issues of importance to patients. HRQL measurement may provide a unique insight into the impact that sarcoidosis may have on a patient's life that is not captured in traditional physiologic measures.

Adult↗

Allergic bronchopulmonary aspergillosis in cystic fibrosis--state of the art: Cystic Fibrosis Foundation Consensus Conference.

Because of the difficulties of recognizing allergic bronchopulmonary aspergillosis (ABPA) in the context of cystic fibrosis (because of overlapping clinical, radiographic, microbiologic, and immunologic features), advances in our understanding of the pathogenesis of allergic aspergillosis, new possibilities in therapy, and the need for agreed-upon definitions, an international consensus conference was convened. Areas addressed included fungal biology, immunopathogenesis, insights from animal models, diagnostic criteria, epidemiology, the use of new immunologic and genetic techniques in diagnosis, imaging modalities, pharmacology, and treatment approaches. Evidence from the existing literature was graded, and the consensus views were synthesized into this document and recirculated for affirmation. Virulence factors in Aspergillus that could aggravate these diseases, and particularly immunogenetic factors that could predispose persons to ABPA, were identified. New information has come from transgenic animals and recombinant fungal and host molecules. Diagnostic criteria that could provide a framework for monitoring were adopted, and helpful imaging features were identified. New possibilities in therapy produced plans for managing diverse clinical presentations.

Animals↗

The Sarcoidosis Health Questionnaire: a new measure of health-related quality of life.

The Sarcoidosis Health Questionnaire (SHQ) is a health-related quality-of-life (HRQL) instrument we designed in a two-part study to allow patients to describe their satisfaction with life as it is affected by sarcoidosis. In the Development Study, we created the SHQ from a 151-item pool generated from interviews with 107 patients, a search of the relevant literature, and discussion with sarcoidosis experts. Using clinical impact methodology and the questionnaire responses of a separate group of 149 patients, we reduced the total number of items to 29. The final SHQ has three domains: Daily Functioning, Physical Functioning, and Emotional Functioning. In the Validation Study, performed with a different group of 111 patients, we found that the SHQ had good internal consistency reliability, as well as evidence of content, criterion, and construct validity based on its comparison with other measures of HRQL (Medical Outcomes Study 36-Item short form and the St. George's Respiratory Questionnaire) and of mental health (the Center for Epidemiologic Study-Depression Scale), as well as with clinical variables including spirometry. The SHQ alone was sensitive to differences in HRQL based on the number of involved organ systems. In conclusion, we have developed a self-completed HRQL questionnaire for U.S. patients with sarcoidosis.

Activities of Daily Living↗

Pleural effusion caused by trapped lung.

We report a case of pleural effusion from trapped lung secondary to sarcoidosis. The patient presented with dyspnea, right pleural effusion, left and right upper-lobe infiltrate and a widened mediastinum. The pleural effusion and dyspnea failed to respond to a course of oral corticosteroids but was relieved by decortication with stripping of inflammatory fibrous bands encasing the visceral pleural. Histologic examination of the lung, visceral and parietal pleura, and the fibrous adhesions all revealed noncaseating granulomas.

Aged↗

The diagnostic pathway to sarcoidosis.

PURPOSE: To examine the time from the first physician visit to the diagnosis of sarcoidosis. HYPOTHESES: The time required to diagnose sarcoidosis is dependent on the initial symptoms, socioeconomic status, referral to a specialist, race, and severity of pulmonary involvement. METHODS: Patients were recruited from the Case Control Etiology of Sarcoidosis Study (ACCESS) and had biopsy-confirmed sarcoidosis. Subjects were asked to recall the date of onset of symptoms of sarcoidosis, their first physician visit, number of physician visits, and types of physicians seen. RESULTS: One hundred eighty-nine patients were enrolled. The diagnosis of sarcoidosis was made on the first physician visit in only 15.3% of cases. The presence of pulmonary symptoms was associated with prolonged time (> 6 months vs < or = 6 months, p = 0.02) until diagnosis, and the presence of skin symptoms with a shorter time (< or = 6 months vs > 6 months, p = 0.02) until diagnosis. Patients with pulmonary symptoms had more physician visits (mean +/- SEM) until the diagnosis was made compared to those without pulmonary symptoms (4.84 +/- 0.38 visits vs 3.15 +/- 0.24 visits, p = 0.0002). The mean baseline FEV(1) was greater in those diagnosed < or = 6 months from the first physician visit than those diagnosed > 6 months (87.3 +/- 1.52% predicted vs 81.2 +/- 2.5% predicted, p = 0.04). There was a significant delay in diagnosis (> 6 months vs < or = 6 months) from first physician visit with higher Scadding stages (stage 4 vs stage 2, or stage 3 vs stage 0 or 1, p = 0.04). CONCLUSIONS: The diagnosis of sarcoidosis is often delayed and seems to be more a factor of disease presentation than patient or physician characteristics. The presence of pulmonary symptoms or higher radiographic stages is associated with a prolonged time until diagnosis. The presence of skin symptoms is associated with less delay in diagnosis. It is likely that the delay in diagnosis of pulmonary sarcoidosis relates to the fact that pulmonary symptoms and parenchymal involvement are nonspecific and are often regarded as manifestations of other pulmonary diseases.

Adult↗

Treatment of corticosteroid-resistant neurosarcoidosis with a short-course cyclophosphamide regimen.

BACKGROUND/OBJECTIVES: Many patients with neurosarcoidosis have disease that is refractory to corticosteroids or they are unable to tolerate high-dose corticosteroids because of detrimental side effects. We examined a short-course, pulse-dose regimen using cyclophosphamide to treat such patients. METHODS: We identified a population of patients with neurosarcoidosis refractory to standard therapy with corticosteroids. Patients who were unable to tolerate corticosteroid therapy due to side effects were also included. Alternative therapy for these patients was initiated using i.v. cyclophosphamide. RESULTS: Seven patients were identified for treatment with our cyclophosphamide regimen. The mean duration of therapy was 5.4 months. Four of the seven patients reported symptomatic improvement on therapy, and all seven patients demonstrated objective improvement in either MRI or cerebrospinal fluid abnormalities. Mean corticosteroid dose of the group was reduced from 42 mg/d before therapy to 18 mg/d after therapy. Relapse of neurologic symptoms was noted in one patient after the completion of therapy. One patient acquired an opportunistic infection, and a second patient required hospitalization for a central venous catheter infection. CONCLUSION: Short-course cyclophosphamide appears to be a reasonable, steroid-sparing treatment option for patients with corticosteroid-refractory neurosarcoidosis.

Adult↗

Two year prognosis of sarcoidosis: the ACCESS experience.

A cohort of 215 sarcoidosis patients from the ACCESS study underwent a clinical evaluation at study enrollment and two years later. Approximately 80% of subjects had an improved or stable FVC, FEV1, chest radiograph determined by Scadding stage, and dyspnea scale. African-Americans had less improvement in FVC than Caucasians (p = 0.04). Patients with erythema nodosum at presentation were more likely to have improvement in the chest radiograph at two-year follow-up (p = 0.007). Patients with a lower annual family income were more likely to worsen with respect to dyspnea (p = 0.01) and more likely to have new organ involvement at two-year follow-up (p = 0.045). The development of new organ involvement over the two year follow-up period was more common in African-Americans compared to Caucasians (p = 0.002) and more likely in those with extrapulmonary involvement at study entry (p = 0.003). There was an excellent concordance between changes in FVC and FEV1 over the two-year period. However, changes in FVC alone were inadequate to describe the change in pulmonary status of the patients, as changes in chest radiographic findings or the level of dyspnea did often but not always move in the same direction as FVC. In conclusion, data from this heterogeneous United States sarcoidosis population indicate that sarcoidosis tends to improve or remain stable over two years in the majority of patients. Several factors associated with improved or worse outcome over two years were identified.

Adult↗

Hepatic, splenic, and gastrointestinal involvement with sarcoidosis.

Sarcoidosis is a multisystem granulomatous disease of unknown cause that primarily affects the lung and lymphatic systems of the body. However, any organ may be affected, including the abdominal organs and gastrointestinal tract. As with other forms of sarcoidosis, abdominal sarcoidosis presents diagnostic, prognostic, and therapeutic dilemmas. This article reviews the various forms of abdominal sarcoidosis, with particular attention given to methods of diagnosis and treatment options.

Journal Article↗

Thalidomide for chronic sarcoidosis.

STUDY OBJECTIVES: Thalidomide therapy has been shown to modify granulomatous diseases, such as tuberculosis and leprosy. Lupus pernio is a skin manifestation of sarcoidosis that does not remit spontaneously, and was used as a marker of efficacy of thalidomide for sarcoidosis. DESIGN: An open-label, dose-escalation trial of thalidomide. SETTING: Patients were seen at one of four specialized sarcoidosis clinics in the United States. PATIENTS: Fifteen patients with lupus pernio and other manifestations of sarcoidosis unresponsive to prior therapy were enrolled. INTERVENTIONS: Skin lesions were assessed with visual examination by the treating physician, and photographic evaluation by a blinded panel of physicians reviewing photographs of the lesions before and after therapy. MEASUREMENTS AND RESULTS: Fourteen patients completed 4 months of therapy. All patients experienced some improvement in their skin lesions subjectively, and 10 of 12 evaluable patients showed improvement using photograph scoring. Five patients were better after 1 month (treated with 50 mg/d of thalidomide), seven more patients improved after 2 months (treated with 100 mg/d of thalidomide in the second month), and two patients required an additional month of 200 mg of thalidomide to achieve a response. Patients reported increased somnolence (n = 9), numbness (n = 7), dizziness (n = 2), constipation (n = 6), rash (n = 1), and increasing shortness of breath (n = 1). One patient discontinued therapy because of new-onset dyspnea, due to probably unrelated new-onset congestive heart failure. CONCLUSION: Thalidomide was an effective form of treatment for chronic cutaneous sarcoidosis. The drug was well tolerated and may be a useful alternative to systemic corticosteroids.

Adult↗

Use of fluticasone in acute symptomatic pulmonary sarcoidosis.

BACKGROUND: Inhaled corticosteroids have been used with variable success in sarcoidosis. The role of the inhaled corticosteroid fluticasone in symptomatic pulmonary patients was studied. METHODS: Twenty-two patients at five institutions who had been given an initial dose of oral corticosteroids within the prior four weeks were enrolled in a randomized double blind trial of inhaled fluticasone. An algorithm for the dosage of prednisone including rules for reducing dose was developed and applied at all centers. RESULTS: Of the 21 patients seen for more than one visit, 10 received fluticasone and 11 placebo. There was no significant difference in the improvement of vital capacity or average daily dose of prednisone for the fluticasone versus placebo. Eight of ten patients taking fluticasone had improvement in cough, while only 6 of 11 patients on placebo had improved cough despite taking oral corticosteroids (p = 0.36, N.S.). The algorithm for decreasing corticosteroid dosage was exactly applied in over 80% of patient visits and oral corticosteroids were used throughout most of the year of treatment. Patients registered higher complaints regarding increased appetite and polyuria when on ten mg or more prednisone a day. There was no clinical difference in the rate of toxicity for the fluticasone versus placebo group. CONCLUSION: A standard approach to tapering oral corticosteroids was followed in over 80% of patient visits. Oral corticosteroids were associated with significant complaints, while inhaled corticosteroids were well tolerated.

Acute Disease↗