Microscopic polyangiitis with unusual lung involvement.

Microscopic polyangiitis (MPA) is a non-granulomatous, systemic and small vessel vasculitis accompanied by segmental necrotizing glomerulonephritis with no evidence of other small vessel disease. We report a patient with weakness, fever, and arthralgia whose CXR and thoracic CT showed widespread nodular infiltration. His proteinase-3 anti-neutrophilic cytoplasmic antibody (c-ANCA) was positive. The serum creatinine was increased and haematuria subsequently developed. Renal biopsy revealed a focal segmental necrotizing glomerulonephritis which was compatible with MPA. He was treated with high-dose corticosteroid and cyclophosphamide. Because of a worsening CXR and hypoxaemia, mechanical ventilation was applied. Despite this he died of respiratory failure following 20 days of treatment. Nodular infiltration is an unusual radiological pattern in patients with MPA and is the reason for this report.

Sarcoidosis caused by interferon therapy.

Interferon alpha (IFN-alpha) is an immunomodulator that is used as an antiviral agent in active chronic viral hepatitis C. IFN therapy can cause an induction or exacerbation of sarcoidosis. Although several reports in the gastroenterology literature have suggested an association between IFN therapy and sarcoidosis, this association has rarely been described elsewhere. A 47-year-old woman developed sarcoidosis after cessation of treatment with IFN and ribavirin for chronic hepatitis C. Her sarcoidosis showed liver, pulmonary and skin involvement. She continues to be monitored regularly in the Department of Pulmonary Diseases without steroid therapy. Her sarcoidosis improved spontaneously. We conclude that patients should be monitored for sarcoidosis during and after IFN therapy.

Exercise capacity in sarcoidosis. Study of 29 patients.

BACKGROUND AND OBJECTIVE: Sarcoidosis is a systemic granulomatous disease of unknown etiology. Aims of this prospective study are to evaluate degree of impairment in pulmonary function tests (PFT), arterial blood gas analysis (ABG), respiratory muscle strength, exercise capacity and correlation of these parameters with radiological stages; to further evaluate the use of cardiopulmonary exercise testing in assessment of extent of pulmonary disease; and to discuss the pathophysiologic mechanisms of limitation in exercise capacity in patients with sarcoidosis. PATIENTS AND METHOD: 29 patients with sarcoidosis were grouped according to their radiological stages (stage I: group 1; stage II, group 2; stage III, group 3). Groups 1, 2 and 3 included 11, 13 and 5 patients, respectively. PFT, cardiopulmonary exercise testing and ABG were performed for each patient. RESULTS: Evaluation of all patients showed a significant decrement in exercise capacity. Patients in stage III had decreased diffusing capacity and exercise capacity. There was limitation in exercise capacity in stage I patients who had completely normal spirometry and diffusing capacity. We also found a correlation between radiological stages of the disease and exercise capacity, diffusing capacity and ABG. CONCLUSIONS: Exercise capacity is impaired also in early stages of sarcoidosis and it was found to be the earliest impaired physiological parameter in sarcoid patients. Exercise intolerance, having mutifactorial basis, is correlated with radiological stages. Circulatory impairment and impaired heart rate response to exercise have effects on limitation in exercise capacity. Especially in advanced radiological stages of disease, ventilatory and gas exchange impairment also seems to be effective on limiting exercise in patients with sarcoidosis.

Exercise capacity in sarcoidosis. Study of 29 patients

FUNDAMENTO Y OBJETIVO: La sarcoidosis es una enfermedad sistémica granulomatosa de etiología desconocida. Los objetivos de este estudio prospectivo fueron evaluar el grado de deterioro delas pruebas funcionales respiratorias, la gasometría arterial, la fuerza de la musculatura respiratoria y la capacidad de ejercicio, así como la correlación de estos parámetros con los estadios radiológicos. Además, se decidió evaluar la utilización de las pruebas de ejercicio cardiorrespiratorio en la valoración de la extensión de la enfermedad pulmonar y, por último, discutir los mecanismos fisiopatológicos de limitación de la capacidad de ejercicio en los pacientes con sarcoidosis. PACIENTES Y MÉTODO: Se incluyó a 29 pacientes, que fueron agrupados según sus estadios radiológicos(estadio I, grupo 1; estadio II, grupo 2; estadio III, grupo 3). Los grupos 1, 2 y 3 incluyeron11, 13 y 5 pacientes, respectivamente. En cada paciente se realizaron pruebas de funcionalismo respiratorio, pruebas de ejercicio cardiorrespiratorio y gasometría arterial. RESULTADOS: Se observó una disminución significativa de la capacidad de ejercicio. Los pacientes del estadio III tenían una disminución de las capacidades de difusión y de ejercicio. Se observó una limitación de la capacidad de ejercicio en los pacientes del estadio I que, por otra parte, mostraron una capacidad de difusión y espirometría normales. También observamos una correlación entre los estadios radiológicos de la enfermedad y la capacidad de ejercicio, capacidad de difusión y gasometría arterial. CONCLUSIONES: La capacidad de ejercicio está deteriorada en los estadios iniciales de la sarcoidosis y éste fue el parámetro fisiológico más precozmente deteriorado en estos pacientes. La intolerancia al ejercicio, con una base multifactorial, se correlaciona con los estadios radiológicos. La afección circulatoria y la respuesta alterada de la frecuencia cardíaca al ejercicio tienen efectos sobre la limitación de la capacidad de ejercicio. Especialmente en los estadios radiológicos avanzados de la enfermedad, el deterioro ventilatorio y del intercambio gaseoso parecen tener asimismo influencia sobre la limitación del ejercicio en pacientes con sarcoidosis

BACKGROUND AND OBJECTIVE: Sarcoidosis is a systemic granulomatous disease of unknown etiology. Aims of this prospective study are to evaluate degree of impairment in pulmonary function tests (PFT), arterial blood gas analysis (ABG), respiratory muscle strength, exercise capacity and correlation of these parameters with radiological stages; to further evaluate the use of cardiopulmonary exercise testing in assessment of extent of pulmonary disease; and to discuss the pathophysiologic mechanisms of limitation in exercise capacity in patients with sarcoidosis. PATIENTS AND METHOD: 29 patients with sarcoidosis were grouped according to their radiological stages (stage I: group 1; stage II, group 2; stage III, group 3). Groups 1, 2 and 3 included 11,13 and 5 patients, respectively. PFT, cardiopulmonary exercise testing and ABG were performed or each patient. RESULTS: Evaluation of all patients showed a significant decrement in exercise capacity. Patients in stage III had decreased diffusing capacity and exercise capacity. There was limitation in exercise capacity in stage I patients who had completely normal spirometry and diffusing capacity. We also found a correlation between radiological stages of the disease and exercise capacity, diffusing capacity and ABG. CONCLUSIONS: Exercise capacity is impaired also in early stages of sarcoidosis and it was found to be the earliest impaired physiological parameter in sarcoid patients. Exercise intolerance, having mutifactorial basis, is correlated with radiological stages. Circulatory impairment and impaired heart rate response to exercise have effects on limitation in exercise capacity. Especially in advanced radiological stages of disease, ventilatory and gas exchange impairment also seems to be effective on limiting exercise in patients with sarcoidosis

Sarcoidosis in Turkey: 1954-2000.

BACKGROUND: Sarcoidosis is a multisystemic disease of unknown etiology. The presentation and frequency of different organ involvement can vary according to race, geographical location and gender. Because of the multiorgan involvement and its mimicking nature, the diagnosis is usually a challenge, even to specialists. Therefore, knowledge of the epidemiologic features of the disease is important. METHODS: The first case report of sarcoidosis in Turkey was published in 1954. We obtained data from case series by hand searching of journals and congress abstract books on pulmonary medicine between 1954 and 2000. Series of 5 or more cases were included in our compilation of data. RESULTS: Data for 1327 patients with the diagnosis of sarcoidosis were obtained from 29 reports. There were nearly twice as many females as males with the disease in these case series. Most of the patients were at stage 1 or 2 at the time of diagnosis. Peripheral lymph node enlargement was reported in 119 patients, skin involvement in 22 and nervous system involvement in 12 patients. Erythema nodosum was reported in 137 patients. Serum angiotensin-converting enzyme was elevated in 52% and the tuberculin skin test was positive in 24% of patients. Organ biopsies seemed to be the preferred diagnostic method in the initial papers while recent papers revealed the value of obtaining a bronchoscopic biopsy. CONCLUSION: Despite several limitations of our study, this is the first compilation of 46 years of data on sarcoidosis in Turkey. Further studies on the geographical distribution and incidence and prevalence are needed for our country.

Endobronchial lipoma: a case report.

Endobronchial lipoma (EL) is a rare benign neoplasm that may cause irreversible pulmonary damage distally, and may be misdiagnosed clinically as a bronchial carcinoid or malignant tumor. They simulate malignant tumors, because of the age, sex and smoking history of the patients in whom they are found. Proper management is a "must" in order to avoid serious and unnecessary complications of the lung. We present a case of endobronchial lipoma, which is located in the right upper lobe bronchus, and diagnosed and treated by surgery.

Multiple endobronchial mass lesions due to sarcoidosis.

Sarcoidosis is a systemic granulomatous disease that primarily affects the lung and lymphatic systems of the body. The lungs are affected in over 90% of sarcoid patients. Although paranchymal lung disease is more common, the airways may also be involved. Bronchial mucosa is often affected in sarcoidosis, but endobronchial mass lesions are very rare. We present a case with multiple endobronchial mass lesions due to sarcoidosis.

Prognostic significance of blood group antigen expression of tumor tissue in lung cancer patients.

AIMS AND BACKGROUND: Many prognostic factors have been evaluated both for SCLC and NSCLC. The prognostic significance of blood group antigen expression of tumor tissues has been studied particularly in NSCLC, yielding divergent results. The aim of the present study was to investigate the prognostic value of the tumoral expression of blood group antigens ABH in lung cancer. METHODS: The presence of blood group antigens was assessed immunohistochemically in paraffin-embedded tumor samples from 92 patients diagnosed between 1996 and 1997. Monoclonal antibodies were used to detect blood group antigens. RESULTS: The median survival was longer in NSCLC patients whose tumors were positive for blood group antigen A (P = 0.009). Since the expression of blood group antigen A in tumor cells was limited to patients with type A or AB blood, survival analysis of these patients showed survival to be longer in non-small cell lung cancer patients with blood group antigen A-positive tumors (P= 0.0019). CONCLUSIONS: Expression of blood group antigen A in tumor cells is an important, favorable prognostic factor in patients with non-small cell lung cancer, which could be useful to stratify patients with blood group A or AB according to possible outcome, and to guide therapeutic decision-making. The expression of blood group antigens ABH should be evaluated in larger series of lung cancer patients (including small and non-small cell lung cancer) with all blood types.