Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0409974 (lupus)
22,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Pulmonary haemosiderosis is a rare disease of unknown etiology, mainly affecting children and adolescents. Pulmonary haemosiderosis may occur in association with several respiratory or other disease (Lupus erythematosus, Goodpastures syndrome). Delay in diagnosing can lead to fatal complications. BAL appears to be the method of choice to detect haemosiderin-laden macrophages. No reference values are available for children to date. 64 bronchoalveolar lavages were performed to establish reference values of haemosiderin-laden macrophages in children. Less haemosiderin was found in children compared to adults, and hence a new haemosiderin score was established for paediatric patients. Compared to healthy children, no elevated haemosiderine levels were found in children with chronic pulmonary disease such as bronchiectasis or chronic aspiration caused by gastro-oesophageal reflux. Therefore even a mildly increased amount of haemosiderin-laden macrophages in children requires medical attention.
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PMID:[Reference values of hemosiderin-laden alveolar macrophages in bronchoalveolar lavage in children]. 1041 43

A 41-year-old man with fever, diarrhea and skin rash received a diagnosis of drug-induced lupus. He was given corticosteroids for 3 months and was subsequently admitted to a local hospital due to dyspnea. Pneumonia was then diagnosed and he was given a new quinolone antibacterial agent. Despite this treatment, his symptoms and signs gradually worsened and he was referred to our hospital. High resolution CT (HRCT) of the chest showed diffuse ground-glass opacities, reticular shadows, parenchymal abnormalities, traction bronchiectasis, a subpleural curvilinear shadow and septal lines. Serological examinations were positive for anti-myeloperoxidase antineutrophil cytoplasmic antibodies (MPO-ANCA) and subsequent HRCT findings were consistent with ANCA-related lung disease. However, the patient had complications such as previous syphilis infection, oral candidiasis, herpes zoster, hepatitis B virus and cytomegalovirus infection. Additionally, his serum was positive for HIV antibody and HIV-1 RNA, and therefore we diagnosed AIDS. His bronchoalveolar lavage fluid revealed Pneumocystis jirovecii. It is known that HIV infection is associated with many types of autoantibodies including MPO-ANCA. Therefore, in HIV/AIDS patients with interstitial lung diseases, it is important to differentiate opportunistic Pneumocystis pneumonia infection from collagen vascular disease-associated interstitial lung diseases.
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PMID:[A case of AIDS with Pneumocystis jirovecii pneumonia which required differentiation from ANCA-related lung disease]. 2235 55

Coexisting morbidities in CVID include bronchiectasis, autoimmunity and malignancies. The incidence of autoimmune disease in CVID patients may approach 20% of cases. The most common autoimmune disease found in CVID patients is autoimmune cytopenia, but rheumatoid arthritis, lupus, and now primary biliary cirrhosis have also been reported. The coexistence of immunodeficiency and autoimmunity appears paradoxical, since one represents a hypoimmune state and the other a hyperimmune state. However, this paradox may not actually be all that implausible due to the complex nature of immune cells, signaling pathways and their interactions. The cellular alterations in combined variable immunodeficiency include a range of T and B cell abnormalities. Selective immune derangements found in CVID include a downregulation of regulatory T cells (Treg cells), accelerated T cell apoptosis, abnormal cytokine production secondary to cytokine gene polymorphisms and increased autoreactive B cell production. The impact of these abnormalities on T and B cell interaction may not only explain the immunodeficiency but also the development of autoimmunity in select groups of patients with CVID. The variability in the clinical manifestations of CVID as a result of this immune interaction suggests that CVID is not one disease but many. This is important because it follows that the treatment of CVID may not always be the same, but may need to be directed specifically towards each individual patient.
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PMID:Common variable immunodeficiency and autoimmunity--an inconvenient truth. 2474