Objective
Diffuse alveolar hemorrhage (DAH) in lupus patients confers >50% mortality, and the cause is unknown. We undertook this study to examine the pathogenesis of DAH in C57BL/6 mice with pristane-induced lupus, a model of human lupus-associated DAH.

Methods
Clinical/pathologic and immunologic manifestations of DAH in pristane-induced lupus were compared with those of DAH in humans. Tissue distribution of pristane was examined by mass spectrometry. Cell types responsible for disease were determined by in vivo depletion using clodronate liposomes and antineutrophil monoclonal antibodies (anti–Ly-6G). The effect of complement depletion with cobra venom factor (CVF) was examined.

Results
After intraperitoneal injection, pristane migrated to the lung, causing cell death, small vessel vasculitis, and alveolar hemorrhage similar to that seen in DAH in humans. B cell–deficient mice were resistant to induction of DAH, but susceptibility was restored by infusing IgM. C3−/− and CD18−/− mice were also resistant, and DAH was prevented in wild-type mice by CVF. Induction of DAH was independent of Toll-like receptors, inflammasomes, and inducible nitric oxide. Mortality was increased in interleukin-10 (IL-10)–deficient mice, and pristane treatment decreased IL-10 receptor expression in monocytes and STAT-3 phosphorylation in lung macrophages. In vivo neutrophil depletion was not protective, while treatment with clodronate liposomes prevented DAH, which suggests that macrophage activation is central to DAH pathogenesis.

Conclusion
The pathogenesis of DAH involves opsonization of dead cells by natural IgM and complement followed by complement receptor–mediated lung inflammation. The disease is macrophage dependent, and IL-10 is protective. Complement inhibition and/or macrophage-targeted therapies may reduce mortality in lupus-associated DAH.

/pdf/pathogenesis-of-diffuse-alveolar-hemorrhage-in-murine-lupus-1fpodlw5zf.pdf

Pathogenesis of Diffuse Alveolar Hemorrhage in Murine Lupus

Sarcoidosis is a complex granulomatous disease that affects virtually every organ and tissue, with a prevalence that varies significantly among the sites involved. The role of conventional imaging, such as computed tomography and magnetic resonance imaging, in the assessment of hepatosplenic sarcoidosis is well established by revealing organ enlargement, multiple discrete nodules, and lymphadenopathy. In this review, we aim to describe contrast-enhanced ultrasound (CEUS) findings in liver and spleen involvement by sarcoidosis, reporting evidence from the literature and cases from our experience, after a brief update on safety profile, cost-effectiveness, and clinical indications of this novel technique. Furthermore, we highlight potential advantages of CEUS in assessing hepatosplenic sarcoidosis that may be useful in the clinical practice.

/pdf/hepatosplenic-sarcoidosis-contrast-enhanced-ultrasound-41nijci0ew.pdf

Hepatosplenic Sarcoidosis: Contrast-Enhanced Ultrasound Findings and Implications for Clinical Practice

Lung diseases associated with hydrocarbon exposure

Background Exogenous lipoid pneumonia is a rare entity, the diagnosis is often missed or delayed. Objective To investigate the clinical characteristics of exogenous lipid pneumonia in elderly patients, improve the efficiency of early diagnosis and treatment of this disease. Methods Retrospectively analyzed the clinical information and imaging data of these 2 exogenous lipid pneumonia patients, reviewed related literature. Results Both of the 2 patients were over 60 with a history of gastrointestinal diseases. The clinical presentation is nonspecific. Chest CT showed many different manifestations. Histopathology analysis revealed the accumulation of lipid carrying macrophages, vacuolar cells and inflammation. A literature review indicated that 25 previous cases of exogenous lipid pneumonia were caused by aspiration. The most common site of the lesion was located in right lung, while mineral oil was the most common substances. We sought to discuss the patient's treatment and the corresponding outcomes. Conclusion The diagnosis of lipid pneumonia's mainly depends on medical history and histopathological morphology. The standard treatment for exogenous lipid pneumonia was composed of antibiotics, steroid, bronchoscopic lavage and a regular follow-up.

Exogenous lipid pneumonia in old people caused by aspiration: Two case reports and literature review.

Exogenous lipoid pneumonia (ELP) is a rare type of inflammatory lung disease caused by aspiration and/or inhalation of fatty substances and characterized by a chronic foreign body-type reaction to intra-alveolar lipid deposits. The usual clinical presentation occurs with insidious onset of nonspecific respiratory symptoms and radiographic findings that can mimic other pulmonary diseases. Diagnosis of ELP is often missed or delayed as it requires a high index of suspicion and familiarity with the constellation of appropriate history and radiologic and pathologic features. We herein report a case of occupational exposure to tabletop “Teppanyaki” entertainment cooking as a cause of ELP, confirmed by surgical lung biopsies in a 63-year-old Asian woman who worked as a Hibachi-Teppanyaki chef for 25 years.

https://downloads.hindawi.com/journals/cripu/2016/1035601.pdf

Teppanyaki/Hibachi Pneumonitis: An Exotic Cause of Exogenous Lipoid Pneumonia.

BACKGROUND: Exogenous lipoid pneumonia is an uncommon condition caused by inhalation or aspiration of a fatty substance. It usually presents as chronic respiratory illness mimicking interstitial lung diseases. Acute exogenous lipoid pneumonia is uncommon and typically is caused by an episode of aspiration of a large quantity of a petroleum-based product. Radiological findings vary and may imitate many other diseases. CASE REPORT: We present a rare case of acute exogenous lipoid pneumonia in a fire-eater who aspirated liquid paraffin during his flame-blowing show (fire-eater's lung). He was admitted to the hospital with productive cough, fever, hemoptysis, chest pain and dyspnea. Diagnosis was made on the basis of history of exposure to fatty substance, characteristic findings in CT examination and presence of lipid-laden macrophages in bronchoalveolar lavage fluid. CONCLUSIONS: Acute exogenous lipoid pneumonia is a very rare disease that typically occurs in fire-eaters and is called a fire-eater's lung. The diagnosis is made on the basis of typical history and radiological, as well as histopathological findings. Language: en

/pdf/exogenous-lipoid-pneumonia-a-case-report-of-a-fire-eater-3ub0pbuaog.pdf

Bożena Homola-Piekarska

Papers

Exogenous lipoid pneumonia - a case report of a fire-eater.