VH4-34 (VH4.21) gene expression in the chronic arthritides of childhood: studies of associations with anti-lipid A antibodies, HLA antigens, and clinical features.

OBJECTIVE: To determine if the germ line gene VH4-34 (VH4.21) encodes the antimonophosphoryl lipid A (MPL) polyspecific antibodies found in oligoarticular arthritis of childhood. METHODS: Sera from a range of rheumatic diseases of childhood were assayed for VH4-34 derived antibodies by ELISA using the antiidiotype monoclonal antibody 9G4. Results were compared to assays for anti-MPL antibodies, C4d, and Bb, and for HLA type, joint count, and sedimentation rate. RESULTS: VH4-34 derived antibodies were elevated in all diseases studied except rheumatoid factor positive polyarticular disease. In oligoarticular arthritis, VH4-34 gene expression correlated with C4d concentration, and VH4-34 encoded globulins were more concentrated in synovial fluid than in blood. No association was found with HLA type. An association between VH4-34 expression and IgG anti-MPL was found in sera from patients from Cincinnati but not from Stanford. No other evidence supported a direct association between VH4-34 derived and anti-MPL antibodies in these children. CONCLUSION: The expression of VH4-34 is increased in several rheumatic diseases of childhood, but, as in adults, not in rheumatoid arthritis. VH4-34 expression is not associated with HLA type. The polyspecific autoantibody nature of some VH4-34 derived antibodies may explain the wide range of the unusual antibodies found in oligoarticular arthritis.

Prolactin-producing pituitary carcinoma with pulmonary metastases.

Pituitary adenomas rarely are metastatic. Extracranial visceral metastases of prolactinomas were not previously reported. The authors report a case of a 34-year-old man with a prolactin-producing pituitary carcinoma and histologically proven lung metastases. Pathologic examination of the pulmonary spread included electron microscopy and immunohistochemistry; these confirmed prolactin production by the tumor. The patient's presentation at initial diagnosis, disease recurrence, clinical course, management, and response to therapy (with its theoretic basis) are detailed. Despite the use of dopamine analogues (to tolerance and in combination), there was documented intracranial and extracranial disease progression. Possible future therapeutic maneuvers are discussed.

Pneumocystis carinii: a deadly opportunist.

Pneumocystis carinii pneumonia is a life-threatening complication of diseases and therapies associated with immunosuppression. Approximately 80 percent of patients with acquired immunodeficiency syndrome will develop pneumocystis pneumonia. Diagnosis is important, because effective therapy is available. In most cases, diagnosis can be made by sputum analysis. Bronchoalveolar lavage will yield a diagnosis in 85 to 90 percent of patients with pneumocystis pneumonia, and is used when sputum induction and analysis is unproductive, unavailable or negative. Transbronchial biopsy and, rarely, open lung biopsy will yield the etiology of pneumonia in the remaining patients. Pentamidine or trimethoprim-sulfamethoxazole is the treatment of choice. Toxicity often occurs, including hypoglycemia, nephrotoxicity, neutropenia and rash. Corticosteroids are helpful in moderate to severe disease. Mortality for the first episode of P. carinii pneumonia averages 20 percent. Prophylaxis effectively prevents and reduces the incidence of future episodes.

Ventriculo-bronchial fistula: a rare cause of intermittent massive hemoptysis.

A 44-year-old man presented with massive hemoptysis. Bronchoscopy followed by surgical exploration revealed a left ventriculo-bronchial fistula related to previous ventricular surgery. Repair was successful.

Pulmonary complications of percutaneous nephrostomy and kidney stone extraction.

Percutaneous nephrostomy and percutaneous removal of kidney stones are widely used procedures that obviate the need for open urologic surgery in many patients. In six patients who had percutaneous renal manipulation, pulmonary complications of varying severity developed, including urinothorax, pneumothorax, hemorrhage, pleural effusion, pneumonia, and atelectasis. Patients having percutaneous renal manipulation should be monitored during and after the procedure for pulmonary complications.

Endobronchial eosinophilic granuloma: a rare cause of total lung atelectasis.

A 12-yr-old male presented with left lung atelectasis secondary to a mainstem endobronchial mass. At thoracotomy, a left mainstem bronchial sleeve resection successfully extirpated what later proved to be an eosinophilic granuloma. This is the first case report, to our knowledge, of endobronchial eosinophilic granuloma.

Transtracheal migration of an intravertebral Steinmann pin to the left bronchus.

Foreign body aspiration only rarely occurs by migration into the lung from other sites in the body. This report describes the migration of an intracervical Steinmann pin through the posterior tracheal membrane into the left bronchus and its successful removal via the rigid bronchoscope.

Tumor cell embolism to pulmonary alveolar capillaries. Cause of sudden cor pulmonale.

A 51-year-old woman, with a 13-month history of widely metastatic breast carcinoma treated with radical mastectomy and chemotherapy, developed sudden shortness of breath and chest pain. Rapidly progressive pulmonary hypertension was documented that failed to respond to supportive measures, and the patient died. The lungs at autopsy demonstrated tumor microemboli in the form of noncohesive, individual cells within the capillaries of approximately 40% of the pulmonary alveolar septae. This case is remarkable for widespread involvement of the alveolar septal capillaries as the cause of acute cor pulmonale.

Life-threatening bacterial pneumonia in male homosexuals with laboratory features of the acquired immunodeficiency syndrome.

Two male homosexuals with laboratory features of the acquired immunodeficiency syndrome developed fulminating pneumonia requiring mechanical ventilatory support despite antibiotic therapy. Pathology consistent with bacterial pneumonia without other opportunistic pathogens were found at open lung biopsy. Cultures from the open biopsy grew Hemophilus influenzae in one, and Streptococcus pneumoniae was seen on Gram stain and sputum culture prior to antimicrobial treatment in the other. Each recovered on continued single antibiotic therapy. Life-threatening bacterial pneumonia may be a feature of the acquired immunodeficiency syndrome, possibly due to B cell abnormalities.

Pulmonary venoocclusive disease in pregnancy.

A 23-year-old woman in her 27th week of gestation presented with clinical findings of progressive pulmonary hypertension. After cardiac catheterization she went into labor and was delivered by cesarean section. She died shortly thereafter from right heart failure. Pulmonary venoocclusive disease was found at autopsy. Hemodynamic changes during pregnancy, labor, delivery, and the postpartum period may have contributed to her deterioration and death. This is the first description of pulmonary venoocclusive disease in pregnancy.

Pulmonary veno-occlusive disease associated with microangiopathic hemolytic anemia and chemotherapy of gastric adenocarcinoma.

A 68-year-old man was treated with FAM (5-fluorouracil, doxorubicin [Adriamycin], mitomycin-C) for metastic gastric adenocarcinoma. Twelve months later while in complete clinical remission, pulmonary hypertension and microangiopathic hemolytic anemia were recognized, progressed, and ended in his demise 6 months later. At necropsy, minimal residual cancer and severe pulmonary veno-occlusive disease was found. Pulmonary veno-occlusive disease may occur in association with microangiopathic hemolytic anemia and cancer chemotherapy.

Invasive pulmonary aspergillosis associated with aspergilloma in sarcoidosis.

We have described two patients with corticosteroid-treated sarcoidosis and with autopsy-proven aspergilloma and diffuse invasive pulmonary aspergillosis. In one, growth of the fungus within a cyst was probably followed by progression to diffuse pulmonary infection. In the second, lung necrosis from invasive infection may have led to the formation of the mycetoma found at autopsy.

Combination chemotherapy pneumonitis: a case report of possible synergistic toxicity.

A woman with pancreatic cancer developed acute interstitial infiltrates while receiving modest doses of two potential pulmonary toxins, mitomycin-C and chlorozotocin. Chemotherapy pneumonitis, confirmed by lung biopsy, responded dramatically to prednisone with negligible sequelae. Comparison with the doses in other reported cases suggested additive or synergistic toxicity between these two alkylating agents.

Bronchocentric granulomatosis: roentgenographic manifestations.

The roentgenographic manifestations of bronchocentric granulomatosis were reviewed in 15 patients. Nine patients had mass lesions. Four had alveolar infiltrates and 2 presented with reticulonodular infiltrates . Correlation of these radiographic patterns with the pathologic findings helps explain the varied morphologic appearance of this disease on the chest radiographs. Upper lobe involvement and unilateral disease were predominant. Seven of 15 patients (47%) presented with solitary mass lesions. Pleural reaction was occasionally noted. Hilar adenopathy and cavitation were infrequent. There was little correlation between radiographic pattern and clinical presentation.

Parathyroid adenoma with coexistent sarcoid granulomas. A hypercalcemic patient.

A 56-year-old woman had hypercalcemia. Clinical symptoms and chest roentgenograms were suggestive of sarcoidosis. Results of laboratory studies, however, were compatible with primary hyperparathyroidism. At surgical exploration of the neck, a parathyroid adenoma was found. Microscopically, sarcoid-like granulomas were present within the adenoma. The association between hypercalcemic sarcoidosis and primary hyperparathyroidism is reviewed, and the importance of this finding is discussed.

Radiographic signs of pulmonary embolism.

Contrary to popular belief, the chest x-ray is abnormal in most patients with documented pulmonary emboli. Radiographic signs include elevation of the diaphragm, infiltrate without infarction, pulmonary infarction, atelectasis, pleural effusion, pulmonary edema, signs of reduced blood flow and changes in the pulmonary vasculature. Recognition of these nonspecific signs can alert the physician to the need for definitive testing by lung scanning and angiography.