Pulmonary infarction caused by sarcoidosis vascular involvement: A case report

Sarcoidosis is a systemic granulomatous disease. In pulmonary sarcoidosis, granulomatous vascular involvement is a common feature that occurs in all types of vessels, including large elastic arteries to venules, but sarcoidosis complicated with pulmonary infarction has not been reported. We report a case of a 60 years old female, who was operated on a clinical diagnosis of lung cancer, and histological examination revealed a pulmonary infarction and sarcoidosis. In the pulmonary elastic arteries, granulomas infiltrated the adventitia and media, and caused elastic fiber collapse and destruction. Arterial occlusion by granulomas was observed in the edge of the infarcted area. It was considered that the arterial sarcoidosis granuloma involvement was the cause of pulmonary infarction. Sarcoidosis is a significant risk factor for cardiovascular events. However, pulmonary infarction is an extremely rare complication of sarcoidosis. Our case suggests that sarcoidosis may cause vascular events in the lungs.     

Pulmonary vascular involvement in sarcoidosis: granulomatous angiitis and microangiopathy in transbronchial lung biopsies

Summary To evaluate the occurrence of granulomatous angiitis and microangiopathy in the lung with sarcoidosis, transbronchial lung biopsy specimens were examined from 174 cases with sarcoidosis. Granulomatous angiitis was seen in 72 cases, which corresponded to 53% of the cases with granulomata. Granulomatous angiitis showed venous involvement (65%), both venous and arterial involvement (24%) or arterial involvement only (11%). There was no significant difference in occurrence of granulomatous angiitis between upper and lower lobes. The cases with granulomatous angiitis in the lung had a higher frequency of ophthalmic symptoms and elevated serum angiotensin converting enzyme level. Basal lamina layering in the microvasculature was more often observed in the bronchial mucosa than in the alveolar walls and is not exclusively related to granulomata. Endothelial proliferation and basal lamina alterations in granulomatous angiitis may be closely associated with granulomas. The present study revealed coexistence of granulomatous angiitis and microangiopathy in the lung with sarcoidosis and suggests that both may participate in the development of pulmonary sarcoidosis.     

Hepatic cytomegalovirus involvement in autopsy cases.

Hepatic involvement of cytomegalovirus (CMV) was studied in 15 autopsy cases with disseminated or solitary CMV infection. Formalin-fixed, paraffin-embedded tissue sections were examined by immunohistochemistry (IHC) using a monoclonal antibody against early and late CMV antigens, and by in situ hybridization (ISH) using biotinylated CMV DNA probes. Three cases showed cytomegalic cells in liver sections by conventional staining, five showed hybridization with CMV DNA probes and seven showed reaction with the monoclonal antibody in the liver. CMV infection was detected not only in cytomegalic cells but also in many non-cytomegalic cells by IHC and ISH, proving these techniques to be superior to routine histological examination. The inflammatory reaction in the liver was not prominent. The reason for the weak inflammatory response in the liver of our present cases, and the possible availability of IHC and ISH for analysis of liver biopsy and bronchoalveolar lavage specimens from immunocompromised hosts were discussed.     

Cardiac sarcoidosis: two cases with autopsy findings

Cardiac sarcoidosis is a disease of young adults. In most cases it presents with sudden death, arrhythmias, conduction disorders, heart failure or cardiomyopathy. The authors describe two cases of myocardial involvement by sarcoidosis that lead to death of the patients. Case one was a 26-year-old Indian man who was previously well and presented with sudden death. Autopsy showed nodules of sarcoid granuloma involving the heart, lungs and lymph nodes. Case two was a 47-year-old Indian lady who complained of reduced effort tolerance. Echocardiography showed that she had restrictive hypertrophic cardiomyopathy with heart failure. Seven months after initial presentation, she developed worsening of heart failure and died. Autopsy revealed involvement of the heart, lungs and liver by sarcoidosis.     

Pulmonary involvement by Niemann-Pick disease. A report of six cases

AIMS: Although pulmonary involvement is a known cause of morbidity in Niemann-Pick disease, histological features in the lung are not well characterized. The purpose of this study is to document the histological features seen in pulmonary involvement by types B and C Niemann-Pick disease and to correlate them with clinical and imaging data. METHODS AND RESULTS: Surgical lung biopsies from six patients (four with type B and two with type C disease) were reviewed and all showed diffuse endogenous lipid pneumonia, with lesser involvement of the interstitium by fibrosis and foamy macrophage accumulation. In type B disease only, there was also fine cytoplasmic vacuolation within the cytoplasm of ciliated epithelial cells. Neither disease showed foamy changes within pneumocytes. One patient had a bronchial cast removed on whole lung lavage. Electron microscopy showed abnormal lamellar inclusions within lysosomes of affected cells in type B disease. In patients with type C disease, biopsies were undertaken as part of investigations into acute respiratory failure in the context of multiorgan systemic presentation. Three patients with type B disease had clinical disease limited to the lung, all adults (mean age of 40 years) with unexplained diffuse parenchymal lung disease and mainly ground-glass shadowing on high-resolution computed tomography. CONCLUSIONS: Niemann-Pick disease should be considered for any patient with unexplained diffuse endogenous lipid pneumonia, even when disease is limited to the lungs and presentation is during adulthood.     

Pulmonary thromboembolism: frequency, site, and vascular change in 103 autopsy cases

For investigation of the incidence of pulmonary thromboembolism, the sites of thromboemboli, pulmonary vascular changes, detailed clinical and autopsy records, and histological sections of a series of 103 autopsied adults were reviewed. Pulmonary thromboembolism was observed in 38.8% of these cases. Pulmonary thromboembolism tended to be more frequent in older, younger, female, and cancer patients. Thromboembolism was found most frequently in arterioles, followed by muscular arteries. A significant correlation (P less than 0.02) was found between the incidence of pulmonary thromboembolism and the histological changes of endothelial protrusion and vacuolization of the intima in these small pulmonary arteries, but no correlation was found between that of pulmonary thromboembolism and constriction figures. These endothelial changes probably represent previous vasoconstriction. The present observations suggest a significant relationship between pulmonary thromboembolism and vasoconstriction of small pulmonary arteries and suggest that widespread vasoconstriction of pulmonary arteries is important in the pathogenesis and pathophysiology of this condition.     

Pulmonary meningiomas: a report of two cases.

Two intraparenchymal lung tumors exhibiting the histopathologic and immunophenotypic characteristics of an intracranial meningioma are presented. The meningiomas presented as solitary asymptomatic nodules in elderly individuals. Both patients survived longer than 3 years following resection, and neither displayed clinical or radiographic evidence of a central nervous system tumor, suggesting that these are primary lung tumors. Review of the literature and discussion of other lesions in the differential diagnosis of this rare intrapulmonary neoplasm are presented.     

Multiple organ involvement in eosinophilic polymyositis: an autopsy report

This report describes autopsy findings of eosinophilic polymyositis in a 29-year-old woman. She complained of muscle pain and tenderness. A serum examination disclosed elevated creatine kinase, and echocardiography showed lowered cardiac function. Electromyography demonstrated myopathic potentials, and the skeletal muscle biopsy revealed degeneration of muscle fibers accompanying marked eosinophilic infiltration. Peripheral eosinophilia was not prominent. A systemic examination excluded possible etiologic factors, such as hypereosinophilic syndrome, collagen vascular diseases, or parasites. The patient died of heart failure after a 4-month clinical course. The autopsy disclosed a set of histological changes indicating tissue injuries, that is, loss of muscle fibers, interstitial fibrosis, and/or infiltrates of lymphocytes not only in the skeletal muscles and heart but also in various visceral organs. Eosinophils were sparsely scattered. These results indicate that the overrun of eosinophils underlying eosinophilic polymyositis, regardless of the presence of hypereosinophilic syndrome or other systemic disorders, could involve wide-ranging visceral organs.     

Pulmonary lesions in Wegener's granulomatosis: a clinicopathologic study of 22 autopsy cases

Pulmonary lesions in 22 autopsy cases of Wegener's granulomatosis (WG) were studied clinicopathologically, with special emphasis on the relation of the lesions to systemic vasculitis, glomerular changes, and clinical manifestations. The pulmonary lesions were divided, on the basis of morphologic characteristics, into four types: 1) the fulminant type, which was characterized by diffusely distributed acute exudative and proliferative alveolitis with prominent small vessel vasculitis (three cases); 2) the granulomatous type, the classic granulomatous lesion (seven cases); 3) the fibrous scar type (five cases); and 4) the mixed type, which had features of both acute exudative and scar-type lesions (seven cases). Clinically, lesions of the fulminant type had rapidly deteriorating courses, terminating in respiratory failure immediately following the onset of the pulmonary manifestations, whereas those of the fibrous scar type had protracted courses. The systemic vasculitis and glomerular lesions in the cases of the fulminant type were almost exclusively fresh lesions. In contrast, the systemic lesions in the cases of the fibrous scar type were nearly always cicatricial. Thus, the morphologic features of the systemic lesions and the clinical profile in each case were well correlated with the pathologic classification of the pulmonary lesions. This correlation may support the assumption that the respiratory tract lesion plays an important role in the progression of systemic involvement in the disease. A comparative study of vascular lesions in various organs revealed some histologic differences between vasculitis in the lungs and that observed outside the respiratory tract; pathogenetic heterogeneity was thus suggested between lesions in the two locations.     

Pulmonary malakoplakia: a report of two cases

Malakoplakia of lung is an unusual condition that has been reported to occur in association with immunocompromised state, particularly in those with acquired immunodeficiency syndrome. We present two cases of pulmonary malakoplakia in immunocompetent individuals. The diagnosis was made on histopathological examination of surgically resected specimen.     

Fetal ascites. A report of 3 autopsy cases

Three rare autopsy cases of fetal ascites were presented and the etiology of each case was described. Case 1 was a male neonate, delivered by cesarean section at 32 weeks' gestation, and died of respiratory failure. The abdomen was remarkably distended with 1020 ml of ascites. The etiology of Case 1 remained unknown even after macroscopic and microscopic examinations. We considered this as "idiopathic" fetal ascites. Case 2 was a female neonate, delivered at 31 weeks' gestation, with marked abdominal distension and cyanosis. Autopsy revealed 435 ml of ascites, and she was considered to have had "polysplenia syndrome" with cardiovascular malformations. Intrauterine heart failure due to cardiac anomalies was thought to be the cause of this ascites. In case 3 embryotomy was carried out under the diagnosis of fetal ascites by ultrasound examination at 22 weeks' gestation. An urachal cyst connected to the dilated urinary bladder and deficiency of musculature of the abdominal wall composed of loose connective tissue with calcification were observed. The abdominal wall was ruptured and 1,960 ml of ascites was measured. Polycystic kidney with renal dysplasia was also found. Case 3 showed "Prune-Berry syndrome" and fetal ascites may have arisen from these anomalies.     

Pulmonary fibroleiomyomatous hamartomas: report of two cases.

The authors report two cases of rare type of hamartoma in the lungs of men (fibroleiomyomatous hamartoma) submitted to a successful operation. The changes were located in the region of large bronchi, causing their narrowing. The authors believe that the muscular coat of the wall of the large blood vessels or bronchi is a probable starting point for proliferation.     

Pulmonary capillary hemangiomatosis-like foci. An autopsy study of 8 cases

Pulmonary capillary hemangiomatosis (PCH) typically occurs in young patients who have signs and symptoms of pulmonary hypertension. It commonly is misdiagnosed in life as pulmonary veno-occlusive disease, and the correct diagnosis usually is not made until autopsy. Autopsy records, including reports, gross photographs, histologic slides, clinical histories, and radiographic images, were reviewed to identify cases with morphologic changes characteristic of PCH. The previous case reports describe PCH as a diffuse process throughout both lung fields. All patients were symptomatic, and most died of the disease. This article details 8 cases of PCH-like foci that were incidental findings at autopsy in which the patients did not have symptoms of pulmonary hypertension nor did PCH contribute in any way to death. This is the first case series that describes pathologic changes of PCH occurring in this setting, and we hope to provide more interest in PCH and its natural history.     

Pulmonary sarcoidosis: A clinico-pathological study.

The light and electron microscopic changes in biopsy tissue from the lung of a 30-year-old housewife severeley incapacitated by diffuse pulmonary sarcoidosis with pulmonary hypertension are presented. The lung tissue was distorted by numerous granulomas in the interstitial tissues and within alveoli. Many pulmonary blood vessels including arteries were damaged by the granulomas. The ultrastructural features of the epithelioid cells were found to be distinctive and probably specific. The giant cells which accompanied the epithelioid cells contained two types of inclusion body: one appeared to be related to the Schaumann body but the nature and origin of the second type was not clear. Many of the granulomas were surrounded by avascular fibrous tissue which contained, in addition to mature fibroblasts, myofibroblasts and a primitive form of cell that appeared to be a fibroblast precursor. It was conjectured that the myofibroblasts, through their contractile powers, might increase the distortion of the lung architecture and thereby the patient's disability. The alveolar walls were thickened by a diffuse infiltrate of macrophages and epithelioid cells but there was no excess of collagen and elastic fibres. The evidence suggested that the epithelioid cells developed from macrophages. From the cellular nature of the diffuse infiltration of the alveolar walls and the absence of fibrosis it seemed that the disease was still at an early and active stage, a conclusion strengthened by the fact that treatment with corticosteroids led to marked and sustained clinical improvement.     

Pulmonary sarcoidosis: management

During the last two decades many advances have been made in the field of sarcoidosis. The disease is now recognised as a multisystem disorder occurring in patients with a genetic predisposition and an exposure to yet unknown transmissible environmental agent/s. The diagnosis is based on a compatible clinical and/or radiological picture, histological evidence of non-caseating granuloma and exclusion of other diseases capable of producing a similar clinical or histological picture. Treatment primarily consists of administration of corticosteroids, although there are valuable alternative drugs. Treatment should be considered in symptomatic patients with evidence of radiologic or lung function deterioration. The patients with extra-pulmonary involvement particularly with ocular, myocardial, and neuro-sarcoidosis almost always need treatment. For asymptomatic pulmonary sarcoidosis patients no therapy is needed.     

Bone marrow involvement in NPM-ALK-positive lymphoma: report of two cases.

Two cases of NPM-ALK-positive anaplastic large cell lymphoma (ALCL) with bone marrow involvement are reported. These cases were recognized within a group of NPM-ALK-positive ALCLs (n = 6) by using immunohistochemistry with the ALK1 monoclonal antibody. In case 1, the bone marrow showed diffuse infiltration of round to spindle-shaped lymphoma cells with moderate fibrosis. In case 2, lymphoma cells intermingling with hematopoietic cells could only be identified by immunohistochemical staining. In contrast to the four NPM-ALK-positive ALCL cases, which showed a cohesive growth pattern in the lymph nodes, the two cases reported here displayed lymphoma cells of smaller size, and they were classified as lymphohistiocytic variants histologically. ALK1 stained small-sized components more clearly than did CD30 (HRS-4). These results suggest that bone marrow involvement of NPM-ALK-positive ALCL may be frequently associated with a histological variant showing a small-sized cell component, and that ALK1 immunostaining is a useful tool to investigate lymphomas for bone marrow involvement.     

Pulmonary veno-occlusive disease in a patient with HIV infection. A case report with autopsy findings

We relate the autopsy findings of a case of pulmonary veno-occlusive disease which occurred in an HIV-infected intravenous drug abuser. This exceedingly rare disease, of unknown cause, is responsible for 10% of primary pulmonary hypertension. Histologically, the disease is characterized by a fibrous intimal thickening of small and medium sized pulmonary veins associated with congestive and dilated capillary network and alveolar haemorrhage. The occurrence of primary pulmonary hypertension in HIV positive patients is 25 times more frequent than in the general population. This is the third reported case of pulmonary veno-occlusive disease occurring in a HIV positive patient. It suggests the role of HIV in the pathogenesis of these vascular lesions.