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Alveoläres Hämorrhagiesyndrom
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Diffuse alveolar hemorrhage is an acute, life-threatening event, and repeated episodes can lead to organizing pneumonia, collagen deposition in small airways, and, ultimately, fibrosis. Among the many conditions it can accompany are Wegener granulomatosis, microscopic polyangiitis, Goodpasture syndrome, connective tissue disorders, antiphospholipid antibody syndrome, infectious or toxic exposures, and neoplastic conditions. Its many causes and presentations pose an important challenge to the clinician.
A 40-year-old woman was referred to pulmonology after presenting with dyspnoea and self-limiting haemoptysis. Chest CT revealed diffuse ground glass opacities and small thin-walled cysts. Bronchoalveolar lavage cultures were negative and cytology revealed haemosiderin-laden macrophages. Transthoracic echocardiogram was normal. Connective tissue disease and vasculitis work-up were negative. Vascular endothelial growth factor-D level was indeterminate. Lung function was normal. She underwent video-assisted thoracoscopic lung biopsy. In addition to findings consistent with lymphangioleiomyomatosis, histopathological examination identified haemosiderosis without capillaritis, confirming a diagnosis of diffuse alveolar haemorrhage in the context of the associated clinical and radiographic features. Follow-up imaging after 5 months showed resolution of the diffuse ground glass opacities. Pharmacotherapy with sirolimus was not initiated due to absence of deterioration in pulmonary function. Diffuse alveolar haemorrhage in patients with lymphangioleiomyomatosis is a rare but important presentation. The few previously reported cases progressed to respiratory failure requiring mechanical ventilation.
Context.—The most common lung tissue samples seen by pathologists worldwide are obtained with the flexible bronchoscope. Specimens taken for examination of diffuse or multifocal parenchymal lung abnormalities pose special challenges for the general surgical pathologist, and these challenges are often compounded by high clinical expectations for accurate and specific diagnosis.
Objective.—To present and discuss the most common histopathologic patterns and diagnostic entities seen in transbronchial biopsy specimens in the setting of diffuse or multifocal lung disease. Specifically, acute lung injury, eosinophilic pneumonia, diffuse alveolar hemorrhage, chronic cellular infiltrates, organizing pneumonia, alveolar proteinosis, sarcoidosis, Wegener granulomatosis, intravenous drug abuse-related microangiopathy, Langerhans cell histiocytosis, and lymphangioleiomyomatosis are presented. Clinical and radiologic context is provided for the more specific diagnostic entities.
Data Sources.—The published literature and experience from a consultation practice.
Conclusions.—The transbronchial biopsy specimen can provide valuable information for clinical management in the setting of diffuse or multifocal lung disease. Computed tomographic scans are useful for selecting appropriate patients to undergo biopsy and in limiting the differential diagnosis. Knowledge of the clinical context, radiologic distribution of abnormalities, and histopathologic patterns is essential. With this information, the surgical pathologist can substantially influence the diagnostic workup and help guide the clinician to an accurate clinical/radiologic/pathologic diagnosis.
Vasculitides are a heterogeneous group of disorders in which inflammation of blood vessel walls is present at least some time during the course of the disease. Vasculitides can affect any caliber or type of vessel in many anatomic sites; however, the disease can alter more than just vasculature. Given the diversity of vasculitides, in 2012, a revised classification system was proposed to categorize vasculitides by the type of vessel involved including size, function, and structural attributes. In the lung, vasculitis impacts both the pulmonary vessels and parenchyma. Extrapulmonary involvement, particularly with concomitant kidney involvement, is a frequent occurrence. Pulmonary vasculitides often present with hemoptysis, pathologically manifested as diffuse alveolar hemorrhage (DAH) with or without capillaritis and can be life threatening when diffuse throughout the lungs. Etiologies for DAH include both primary and secondary vasculitides, along with collagen-vascular diseases, infection, and drug toxicity. Therefore, diagnosing the specific vasculitic etiology often relies on comprehensive assessment of all clinical, laboratory/serological, imaging, and histopathologic features that may be present. The most common primary pulmonary vasculitides often affect small vessels and are associated with circulating antineutrophilic cytoplasmic antibodies (ANCAs). In the 2012 classification, these include granulomatosis with polyangiitis (formerly Wegener granulomatosis), eosinophilic granulomatosis with polyangiitis (formerly Churg–Strauss' syndrome), and microscopic polyangiitis. Other less frequent vasculitides that are non-ANCA associated or affect medium- to large-sized vessels can have pulmonary involvement. These entities are usually associated with extrapulmonary disease and include polyarteritis nodosa, Takayasu's arteritis, Behçet's disease, and antibasement membrane antibody disease (formerly Goodpasture's syndrome). Although all vasculitides have vessel wall inflammation at some phase in the disease process, their histopathologic findings are as diverse as the group of diseases themselves. The characteristic histologic findings of the pulmonary vasculitides will be reviewed here.
With its award-winning, innovative approach, the new edition of Practical Pulmonary Pathology, by Kevin O. Leslie, MD and Mark R. Wick, MD, provides comprehensive, practical guidance in the accurate identification and interpretation of neoplastic and non-neoplastic diseases of the lungs. Lavishly illustrated in full color throughout, this "one-stop" resource captures key morphologic patterns for a full range of common and rare conditions and assists in the interpretation of complex diagnostic puzzles. An easily accessible format with a unique "visual index" places in-depth diagnostic guidance quickly at your fingertips-in print or online at www.expertconsult.com.
Eine diffuse alveoläre Hämorrhagie bezeichnet eine diffuse intraalveoläre Blutung aus kleinen Blutgefäßen infolge einer schweren Schädigung der alveolokapillären Membran der Lungen. Dies ist ein relativ seltenes, dann jedoch oft lebensbedrohliches Ereignis. Das differenzialdiagnostische Spektrum ist vielfältig und beinhaltet sowohl immunologische, als auch toxische, infektiöse, hämodynamische, neoplastische und physikalische Ursachen. Bei einer diffusen alveolären Hämorrhagie kann es sich um eine Folge sowohl von Systemerkrankungen, als auch von auf die Lunge beschränkten Prozessen handeln. Die häufigsten Ursachen sind die sogen. Kleingefäßvaskulitiden, die mikroskopische Polyangiitis und die Wegener'sche Granulomatose. Seltenere Ursachen sind das Goodpasture-Syndrom und der systemische Lupus erythematodes. Neben autoimmunen Prozessen müssen vor allem Erkrankungen mit eine Steigerung der linksventrikulären Vorlast, Infektionen und Medikamentennebenwirkungen in Betracht gezogen werden. Es wird ein Überblick über das differenzialdiagnostische Spektrum und das diagnostische Vorgehen bei diesen Patienten gegeben. Da eine frühzeitige aggressive Behandlung lebensrettend sein kann, ist eine rasche und gezielte Diagnostik entscheidend.
Diffuse alveolar hemorrhage (DAH) represents a syndrome that can complicate many clinical conditions and may be life-threatening, requiring prompt treatment. It is recognized by the signs of acute- or subacute-onset cough, hemoptysis, diffuse radiographic pulmonary infiltrates, anemia, and hypoxemic respiratory distress. DAH is characterized by the accumulation of intra-alveolar red blood cells originating most frequently from the alveolar capillaries. It must be distinguished from localized pulmonary hemorrhage, which is most commonly due to chronic bronchitis, bronchiectasis, tumor, or localized infection. Hemoptysis, the major sign of DAH, may develop suddenly or over a period of days to weeks; this sign may also be initially absent, in which case diagnostic suspicion is established after sequential bronchoalveolar lavage reveals worsening red blood cell counts. The causes of DAH can be divided into infectious and noninfectious, the latter of which may affect immunocompetent or immunodeficient patients. Pulmonary infections are rarely reported in association with DAH, but they should be considered in the diagnostic workup because of the obvious therapeutic implications. In immunocompromised patients, the main infectious diseases that cause DAH are cytomegalovirus, adenovirus, invasive aspergillosis, Mycoplasma, Legionella, and Strongyloides. In immunocompetent patients, the infectious diseases that most frequently cause DAH are influenza A (H1N1), dengue, leptospirosis, malaria, and Staphylococcus aureus infection. Based on a search of the PubMed and Scopus databases, we review the infectious diseases that may cause DAH in immunocompetent patients.
Diffuse alveolar hemorrhage (DAH) is often a catastrophic clinical syndrome causing respiratory failure. Recognition of DAH often requires BAL as symptoms are nonspecific, hemoptysis is absent in up to one-third of patients, and radiographic imaging is also nonspecific and similar to other acute alveolar filling processes. Once the diagnosis is established, the underlying cause must be established in order to initiate treatment. This review discusses the diagnosis of the underlying histologies and the clinical entities that are responsible for DAH as well as treatment options.
Specialty imaging - HRCT of the lung
- J W Gurney
Biopsy interpretation of the lung
- D I Suster
- S Suster
Differentialdiagnostik der diffusen alveolären Hämorrhagie
- Schreiber