Lupus pernio. Radiogallium imaging in a patient with chronic cutaneous sarcoidosis.

A 37-year-old man with a history of chronic skin sarcoidosis had the classic triad of lupus pernio. Each of the lesions (nose, ears, and hands) revealed avid radiogallium accumulation. In addition, both the panda sign and the lambda sign of hilar-mediastinal lymph node uptake of radiogallium were present, despite normal results of chest radiography. Several other lymph node chains were also involved. The case can serve as a baseline to determine if other instances of lupus pernio have such a wide systemic distribution of radiogallium uptake.

Specificity and sensitivity of distinctive chest radiographic and/or 67Ga images in the noninvasive diagnosis of sarcoidosis.

An analysis of gallium-67 (67Ga) uptake in the lacrimal and salivary glands and intrathoracic lymph nodes was made in 162 patients with sarcoidosis, consisting of a large number with chronic "fibrotic" disease, and 167 HIV-positive patients (most of whom have/had AIDS). This study was designed to further assess the diagnostic sensitivity and to fully evaluate the diagnostic specificity of chest radiographic and/or 67Ga uptake findings found to be characteristic of sarcoidosis. A lambda 67Ga uptake image or a panda 67Ga uptake image with associated bilateral, symmetrical hilar lymphadenopathy (BSHL) or bilateral, symmetrical parenchymal infiltration indicative of pulmonary fibrosis on chest radiograph (BSIF) was commonly present in sarcoidosis. These distinctive 67Ga uptake images were frequently observed in patients with normal chest radiographs (stage 0 [33 percent]), as well as in patients with BSHL on chest radiograph whether the disease was in an "early" stage, ie, stages I (74 percent) and II (90 percent) or a chronic "fibrotic" stage, ie, IVa (71 percent). A panda 67Ga uptake image was observed in 8 percent of HIV-positive patients; however, a lambda 67Ga uptake image alone or any of the other chest radiographic and/or 67Ga uptake patterns distinctive for sarcoidosis were not observed in any of 167 HIV-positive patients. We conclude that (1) a lambda 67Ga thoracic image (usually associated with a panda 67Ga uptake image) or a panda 67Ga uptake image together with BSHL or BSIF on chest radiograph represent distinctive patterns that are highly specific and sensitive for the noninvasive diagnosis of the majority of sarcoidosis patients, and (2) the finding of a panda 67Ga uptake image, not associated with BSHL or BSIF on chest radiograph, should suggest, in addition to a limited number of readily diagnosable disorders, the presence of a positive HIV status.

Hypersensitivity pneumonitis associated with a portable home humidifier.

Hypersensitivity pneumonitis (HP) is an immunologically mediated inflammatory disorder of the pulmonary parenchyma which has been traditionally associated with the inhalation of organic dusts (farmer's lung, pigeon breeder's disease) and a variety of simple chemical compounds. More recently, the disease has been reported in association with the inhalation of aerosolized droplets in home and office settings. In Connecticut, the home and office, rather than the farm or factory, are the most common, although frequently unrecognized, environmental settings in which HP develops. We report here a case of a young, healthy woman who developed severe HP associated with the use of a portable home ultrasonic humidifier. This clinical entity should be suspected in the patient with a "recurrent viral illness" or "recurrent pneumonia." A review of the clinical and laboratory findings of humidifier-induced HP are discussed in relation to its pathogenesis, diagnosis, and treatment.

Recognition of distinctive patterns of gallium-67 distribution in sarcoidosis.

Assessment of gallium-67 (67Ga) uptake in the salivary and lacrimal glands and intrathoracic lymph nodes was made in 605 consecutive patients including 65 with sarcoidosis. A distinctive intrathoracic lymph node 67Ga uptake pattern, resembling the Greek letter lambda, was observed only in sarcoidosis (72%). Symmetrical lacrimal gland and parotid gland 67Ga uptake (panda appearance) was noted in 79% of sarcoidosis patients. A simultaneous lambda and panda pattern (62%) or a panda appearance with radiographic bilateral, symmetrical, hilar lymphadenopathy (6%) was present only in sarcoidosis patients. The presence of either of these patterns was particularly prevalent in roentgen Stages I (80%) or II (74%). We conclude that simultaneous (a) lambda and panda images, or (b) a panda image with bilateral symmetrical hilar lymphadenopathy on chest X-ray represent distinctive patterns which are highly specific for sarcoidosis, and may obviate the need for invasive diagnostic procedures.

Extrapulmonary sites of radiogallium accumulation in sarcoidosis.

In an effort to detect extrapulmonary sites of radiogallium accumulation in cases of sarcoidosis, 145 separate Ga-67 citrate studies of 114 patients with biopsy-proven sarcoidosis were examined. The most characteristic extrapulmonary radiogallium uptake pattern was the "panda sign" in 47 patients (41%). The most common site of prominent extrapulmonary radiogallium uptake was the lacrimal glands in 101 patients (88%). Second most common was activity in one or more superficial lymph node regions such as the cervical, axillary, femoral, or inguinal in 19 patients (17%). Other extrapulmonary sites included breast uptake in 6 out of 80 women (8%), prominent splenic and nasal uptake in 9 (8%) patients, periportal accumulation in 7 (6%), and cutaneous/subcutaneous activity in 4 (4%). Because many of these individuals were receiving corticosteroids, the natural (untreated) prevalence of extrapulmonary findings may be even higher. Although the sensitivity and specificity of extrapulmonary radiogallium accumulation has still to be determined, many of the sites may be accessible to biopsy both for diagnostic purposes and to follow the effects of medications. It is therefore suggested that whole-body imaging be performed when radiogallium is administered to patients with suspected or known sarcoidosis.

Bronchocentric granulomatosis and allergic bronchopulmonary aspergillosis.

Considerable clinical and radiologic similarity exists among FB, ABP, MIB, ABPA, and, to a lesser extent, BCG. In the absence of focal obstructive lesions such as tuberculosis or neoplasm, one of the diffuse bronchial hypersecretory diseases, that is, bronchial asthma, acute or chronic bronchitis, asthmatic bronchitis, or cystic fibrosis, is prerequisite to the development of a clinical and radiologic syndrome, resulting from bronchial mucus retention. This hypersecretory bronchial obstruction syndrome characterizes each of the disorders discussed. Thus, the differences existing among these disorders are merely those of size and location of mucus plugs or casts, and there is substantial variation within each individual entity concerning even this aspect. Unfortunately, extensive efforts to elucidate immunopathogenesis exist only for ABPA. Although evidence for the immunologic and morphologic presence of fungi, particularly AF, in ABPA is quite compelling, it is not necessarily conclusive evidence for an etiologic role of AF in the immunopathogenesis of this disorder. In this regard, it is reasonable to speculate that, perhaps, similar immunologic and morphologic information exists for FB, ABP, MIB, and, in some cases, BCG. BCG, because of its unique intrinsic bronchial obstructive and destructive histopathology, is a disorder distinct from the others, though sharing common clinical and radiologic features in some instances. If Aspergillus is involved in the immunopathogenesis of this disease, it cannot be the only etiologic factor, since in many instances no evidence of its presence is found.

Effect of corticosteroids on lung volume-pressure curves in bleomycin-induced lung injury in the rat.

In this study we investigated changes in volume-pressure curves at various stages after bleomycin induced lung injury (6 h, and 3, 7, and 28 days) and tested the ability of steroids to influence these changes. The most significant decreases in volume-pressure curves were observed at 3 days post bleomycin, a time point that correlates to the peak period of inflammation. Steroids as drugs of high antiinflammatory potency were used in order to modulate the inflammatory response and possibly the volume-pressure curve changes observed during the natural course of bleomycin induced lung injury. Dexamethasone treatment significantly inhibited the decreases in volume pressure curves observed at 3 days post bleomycin, whereas methylprednisolone did not. Also both steroids significantly inhibited inflammation as measured by histological evaluation and the presence of cells in the bronchoalveolar lavage fluid.

Correlation of changes in pulmonary surfactant phospholipids with compliance in bleomycin-induced pulmonary fibrosis in the rat.

Qualitative and quantitative analysis was performed on phospholipids isolated from bronchoalveolar lavage fluid during the development of pulmonary fibrosis in the rat. A single transtracheal injection of 2.0 units of bleomycin was administered to rats to induce lung injury. Animals were killed at 0, 3, 7, 14, 30, and 120 days after bleomycin treatment. Total lipid phosphorus in BAL from animals given bleomycin increased from 1.6 mumol/lung in normal animals to 3.2 mumol/lung at 14 and 30 days. The increase in phospholipids was primarily in phosphatidylcholine with minor increases in phosphatidylinositol and phosphatidylethanolamine. These quantitative changes were accompanied by qualitative changes that included an increase in the percentage of total phosphatidylcholine, disaturated phosphatidylcholine, and phosphatidylinositol. In contrast, the percentage of phosphatidylglycerol was significantly reduced. Plasma phospholipid analysis indicated that these alterations were not due to plasma contamination. The functional significance of the phospholipid changes was assessed by comparing air- and saline-filled compliance measurements at similar times after bleomycin. Abnormal compliance measurements were observed at 3, 7, and 14 days after bleomycin. At 3 and 7 days the predominant compliance defect was at the air-liquid interface; however, at 14 days, when phospholipids were significantly elevated, the defect was primarily due to tissue components.(ABSTRACT TRUNCATED AT 250 WORDS)

Surfactant and physiologic alterations in an animal model of adult human lung disease. A preliminary report.

The diffuse obstructive and infiltrative lung diseases (e.g. bronchitis, asthmatic bronchitis, fibrosing alveolitis, sarcoidosis) contribute significantly to human morbidity and mortality. Disturbance of ventilation and perfusion represents a major physiologic defect in these diseases, resulting in pulmonary hypertension, cor pulmonale and right heart failure. Immunologic and surfactant alterations play a prominent role in these diseases. There is a growing body of evidence indicating that surfactant is a most important material in maintaining airway patency. General concepts regarding these functions in human adult lung diseases and results of investigations of surfactant in an animal model of bleomycin-induced lung fibrosis are presented.

Differential cellular analysis of bronchoalveolar lavage fluid obtained at various stages during the development of bleomycin-induced pulmonary fibrosis in the rat.

The purpose of this study was to analyze the cellular components of bronchoalveolar lavage fluid throughout the development of bleomycin-induced pulmonary fibrosis in the rat. Animals were killed and lavaged at various times after the administration of a single intratracheal injection of bleomycin. The results demonstrate that a significant influx of inflammatory cells appear in the lavage fluid as early as Day 1 after bleomycin treatment. Polymorphonuclear leukocytes are the first cells to appear and significant concentrations persist for as long as 1 month after bleomycin treatment. There is a very transient yet significant influx of eosinophils on Day 7 after bleomycin treatment. Lymphocytes are present from 3 to 14 days after bleomycin treatment; greater than 97% are T-cells and less than 3% are B-cells. There is a 1:1 ratio of W3/25+ cells (helper cell activity) to OX8+ cells (suppressor cell activity) comprising the lymphocyte population. The blood and lymphoid tissue of these animals contain a normal 2:1 ratio of these subsets. The data demonstrate that specific T-cell populations are present in the air spaces of the lung in response to bleomycin-induced pulmonary fibrosis in this model.