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Query: UMLS:C0034069 (pulmonary fibrosis)
 7,050 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report the occurrence of the yellow nail syndrome in 3 patients with rheumatoid arthritis. In 2 patients the development of the syndrome was related to severe chest infections, and there was evidence of immunodeficiency, consisting in 1 case of a persistently low serum IgA level and in the other of a generalised marrow suppression. The third patient gave a history of recurrent sinusitis and showed evidence of previous chest infections and pulmonary fibrosis, but has no gross evidence of immunodeficiency. We have found only 1 previous brief mention of a patient with the yellow nail syndrome and rheumatoid arthritis.
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PMID:Yellow nail syndrome in rheumatoid arthritis: report of three cases. 51 48

Pneumocystis carinii pneumonia occurred in 6 of 17 rhesus monkeys infected with simian immunodeficiency virus and was studied by immunohistochemistry and by scanning and transmission electron microscopy. A monoclonal antibody/streptavidin-biotin-peroxidase staining method was highly sensitive for detecting the organisms in small, early lesions and was much more sensitive and specific than traditional silver impregnation methods. Reprocessing of paraffin wax-embedded lung tissue for scanning electron microscopy and use of a video printer to produce a photographic montage of light microscopic lesions allowed the same areas of tissue to be examined and compared by both methods. The ultrastructural morphology of P. carinii in the rhesus monkey was identical to that in man, as were the histological and electron microscopic lesions, including pulmonary fibrosis. Trophozoites were seen attached to alveolar type I epithelium mainly by intimate apposition to the plasma membrane, but scanning electron microscopy also showed attachment by elongated filopodia. Few macrophages were present in infected alveoli, and though phagocytosis followed by digestion of P. carinii trophozoites was observed, it appeared to occur at a very low level.
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PMID:Pneumocystis carinii pneumonia in simian immunodeficiency virus infection: immunohistological and scanning and transmission electron microscopical studies. 207 17

Cutaneous histiocytosis may take two principal forms. It is either a benign proliferative process or a relentless, progressive process with a poor prognosis. In histiocytic medullary reticulosis, histiocytes demonstrate nuclear atypia and the outcome is uniformly fatal. Benign cephalic histiocytosis X causes lesions similar to those of histiocytosis X, but Langerhans' cells are absent. In congenital self-healing histiocytosis X, the Letterer-Siwe-like cutaneous infiltrate contains Langerhans' cells, but the lesions heal spontaneously without treatment. The nodular cutaneous lesions of juvenile xanthogranuloma appear in infancy and resolve without treatment; however, the higher percentage (10%) of associated ocular lesions may lead to glaucoma and blindness. In histiocytosis X, the cutaneous lesions show a marked proliferation of Langerhans' cells, with prognosis dependent on the patient's age and the extent of organ dysfunction. Patients who survive the acute form of the disease may develop diabetes insipidus, growth retardation, pulmonary fibrosis, and biliary cirrhosis. A subtle immunologic defect has been identified in patients with histiocytosis X, yet the pathogenesis of the disease is still speculative. Familial disease occurring in early infancy should be differentiated from complete or partial immunodeficiency syndromes. Guidelines for evaluating patients with cutaneous histiocytosis are reviewed.
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PMID:Cutaneous histiocytosis syndromes. 299 40

A male infant in whom multiple recurrent multiorgan infections developed during the first six months of life was found to have combined immunodeficiency. Progressive pulmonary disease developed at age two years; cytomegalovirus (CMV) was isolated from the respiratory tract and urine. Three separate intramuscular grafts of cultured thymus fragments did not produce change in the course of the illness. Soon after age three years, IgG lambda appeared in the serum as an M-component. The patient died at age three and one-half years, with respiratory insufficiency due to pulmonary fibrosis. At autopsy, a malignant plasma cell infiltrate was limited to the retroperitoneum. The infiltrate replaced lymph node structures and surrounded nerve fascicles, which appeared necrotic, and contained CMV inclusions in ganglion cell nuclei. The plasma cells showed strong monoclonal staining for IgG lambda. Also noted was positive staining for J-chain, which has been reported previously in malignant plasma cells producing IgG. CMV could be responsible for abnormal B-cell proliferation in patients with defective immunoregulation who receive immunotherapy, as in lymphoid abnormalities associated with Epstein-Barr virus.
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PMID:Abnormal B-cell proliferation associated with combined immunodeficiency, cytomegalovirus, and cultured thymus grafts. 608 43

Pulmonary aspergillosis has recently been described as an emerging infection in patients with acquired immune deficiency syndrome (AIDS), but the pathological changes have not been well documented. In this autopsy study, 17 cases of AIDS-related pulmonary aspergillosis were identified from the files of two institutions. With the exception of hypersensitivity reactions, the entire spectrum of pulmonary aspergillosis was represented. Thirteen patients exhibited acute invasive aspergillosis, and seven patients had evidence of subacute or chronic invasive infection, four of whom also had areas of acute invasion. One patient had necrotizing bronchial aspergillosis as well as acute invasive infection, and one individual had saprophytic colonization of a cavity caused by previous Pneumocystis carinii pneumonia (PCP) without evidence of invasive aspergillosis. The same conditions known to predispose immunocompromised individuals without human immunodeficiency virus (HIV) infection to invasive pulmonary aspergillosis were also identified in these patients with AIDS and included neutropenia, steroid therapy, and underlying lung disease. Additional pulmonary conditions were identified in all but one case and consisted mainly of infection or some form of chronic lung disease. In particular, half of the cases were associated with pulmonary fibrosis related to prior PCP. All cases occurred in or after 1990, confirming the perception of the recent emergence of aspergillosis in AIDS. As suggested by this study, one reason for this may be that patients with AIDS are now living long enough to develop one or more of the predisposing conditions for pulmonary aspergillosis.
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PMID:Pulmonary aspergillosis in acquired immune deficiency syndrome: autopsy study of an emerging pulmonary complication of human immunodeficiency virus infection. 938 32

The occurrence of sarcoidosis in combination with common variable immunodeficiency (CVID) has been described in a small number of patients. In these patients, sarcoidosis consisted of lymphadenopathy, mild to moderate pulmonary involvement and hepatosplenomegaly. However, severe and rapidly progressive pulmonary fibrosis in combination with a severe defect of the cellular and humoral immune system has not been described yet. In our patient, defects of the T and B cell system resulted in severe immunodeficiency. The defect of the humoral immune system was characterized by the impairment of specific antibody production in vivo. In addition, hypogammaglobulinemia with missing IgA and IgE along with a marked defect in IgM and IgG production was noted. There was a progressively reduced lymphocyte proliferation in response to T cell mitogens, while proliferation after specific IL-2 stimulation was normal. A Th1 lymphocyte-subset-like profile might thus play a role in the pathogenesis and might form the connecting link between sarcoidosis and CVID. This is the report of a so far new and unique combination of severe immunodeficiency and sarcoidosis also associated with a congenital dysmorphia consisting of a palatal cleft. The findings of the 40 patients with CVID and sarcoidosis reported so far are discussed in order to point out the typical features of patients with this uncommon syndrome.
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PMID:Sarcoidosis and common variable immunodeficiency. A case of a malignant course of sarcoidosis in conjunction with severe impairment of the cellular and humoral immune system. 1085 23

Common variable immunodeficiency is a disorder characterised by hypogammaglobulinemia with B-lymphocytes in peripheral blood and repeated infections. We report a child with a diagnosis of diabetes mellitus and celiac disease during lactation, and in whom common variable immunodeficiency was diagnosed at the age of 5. During evolution of the disease he presented multiple respiratory infections in spite of substitution therapy with gamma globulins. He presented pulmonary fibrosis with a pulmonary volume reduced, and a spirometric restrictive patron. Immunologically, he presents reduction in CD4 lymphoid population. He expresses the alleles DQ2 A1 0501 and B1 which are strongly associated with susceptibility to insulin-dependent diabetes mellitus and celiac disease, but don't express antigens HLA class II DR3 and DR4 that are more frequent in these entities. The main disease and all the complications had affected his curve pondostatural.
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PMID:Common variable immunodeficiency, insulin-dependent diabetes mellitus and celiac disease. 1126

Particular attention has been recently dedicated to the development of a sarcoidosis-like form in patients with common variable immunodeficiency (CVI). In sarcoidosis a 5-fold increase in the number of cells in the bronchoalveolar lavage compared with normal controls has been described and the predominance of CD4+ T cells is a common feature. In contrast to patients affected by sarcoidosis or CVI, patients with co-existence of the two diseases had a more favorable prognosis, as progressive pulmonary fibrosis occurred only in a few cases even when persistent alveolar lymphocytosis was present. Moreover, patients presenting with concomitant sarcoidosis and CVI, showed a lower rate of respiratory infections and of the evolution towards bronchiectasies in comparison with patients affected by CVI alone. The clinical evolution observed in our patients was similar with that reported in other studies and confirms their clinical features. One may speculate that the increase in the number of macrophages and T lymphocytes, as suggested by the increased cellularity in the bronchoalveolar lavage of these patients, can produce a peculiar microenvironment characterized by high levels of soluble factors (cytokines). In turn, the latter could be responsible for a more efficacious effector phase of the immune response. This may explain the reduced rate of infectious diseases and consequently of bronchiectasies observed in CVI patients.
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PMID:["Sarcoidosis-like" granulomatous disease in patients with common variable hypogammaglobulinemia]. 1215 44

This case illustrates the reopening of foramen ovale in a young patient with chronic pulmonary hypertension caused by bronchiectasis and chronic pulmonary fibrosis, which resulted in a prominent right-to-left shunt and severe hypoxia. Her clinically unsuspected right-to-left shunt was discovered during ventilation-perfusion scan, which was performed for the evaluation of pulmonary embolism. She had common variable immune deficiency, a primary immunodeficiency disease in which B-lymphocytes produce few or no antibodies. Most patients with this syndrome have an intrinsic defect in their B-lymphocytes that results in reduced immunoglobulin production. In these patients, recurrent respiratory tract infections are common and may result in chronic lung disease, fibrosis, particularly bronchiectasis (20-30%) and even cor pulmonale as happened in our patient [J. Clin. Immunol. 9 (1989) 22-33.].
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PMID:Lung scan in the diagnosis and management of patent foramen ovale pulmonary embolism, paradoxical embolism. 1221 71

Pneumonia is a major complication of human immunodeficiency virus (HIV) pathogenesis but it develops only after prolonged infection. We used the macaque model to explore a hypothesis that the disease is a two-stage process, the first stage being establishment of the viral infection in the lung and the second being amplification of virus replication by host factors induced by chemical agents or opportunistic pathogens in the lung. Bleomycin, a chemical known to induce diffuse alveolar damage and pulmonary fibrosis with accumulation of macrophages and a rich T helper type 2 (Th2) cytokine environment, was inoculated intratracheally into five of eight SHIV 89.6P-infected macaques and into one uninfected macaque. Three additional simian HIV (SHIV)-infected macaques without bleomycin treatment served as untreated virus controls. Although none of the animals became clinically ill, bleomycin induced classical host responses in the lungs of all the treated, virus-infected macaques. There was enhanced production of the chemokine, monocyte chemotactic protein-1 (MCP-1), that had previously been shown to cause enhanced replication of the virus. Four of the five treated animals developed more productive SHIV infection in the lungs compared with the infected untreated animals. Enhanced virus replication was found primarily in infiltrating macrophages. Enhanced replication of the virus in the lungs was associated with host factors induced by the drug and supported the hypothesis for a two-stage process of pulmonary pathogenesis.
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PMID:Bleomycin treatment causes enhancement of virus replication in the lungs of SHIV-infected macaques. 1722 Mar 71


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