Diagnosis Of Pulmonary Vasculitis Research Articles

What to do when you suspect your patient suffers from pulmonary vasculitis?

Making a diagnosis of pulmonary vasculitis is challenging. The most common cause of pulmonary vasculitis is small vessel anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Pulmonary involvement in other forms of vasculitis such as large vessel vasculitis is rare. Since correct and timely diagnosis is pivotal to start (immunosuppressive) therapy to avoid vasculitic damage, a complete patient history should be obtained and a physical examination performed. Initial laboratory evaluation should include inflammation markers, renal and liver function tests, and the determination of ANCA. New developments in ANCA testing result in tests with excellent predictive value for the diagnosis of AAV-related pulmonary vasculitis. Consequently, ANCA should be tested with these tests of the so-called second (capture ELISA) or third (anchor ELISA) generation. In patients who are ANCA negative, a simple algorithm is presented based on laboratory evaluation of autoantibodies and 18F-FDG-PET-CT scanning. Such an algorithm may be useful for accelerating the diagnostic process needed to make a diagnosis of pulmonary vasculitis, or alternatively, to quickly exclude such a diagnosis.

Current modalities in the diagnosis of pulmonary vasculitis

This review addresses the pulmonary manifestations of the vasculitides, with a focus on diagnostic modalities. Haemorrhagic presentations (usually associated with nephritis: the pulmonary-renal syndrome) are the most common vasculitic cause of early death. The diagnostic modalities in the pulmonary vasculitides are reviewed, with a focus on primary systemic vasculitis. A literature search of original research and review articles on pulmonary vasculitides was undertaken using the PubMed database. Small-vessel anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, especially granulomatosis with polyangiitis (Wegener's granulomatosis) are the most frequent causes of pulmonary vasculitis and typically present as nodules, alveolar infiltrates (haemorrhagic or not), cavities or tracheobronchial stenosis. Lung involvement is less common in large-vessel vasculitis when pulmonary vascular abnormalities can be seen. No single test is pathogonomonic and diagnosis requires integration of clinical, laboratory, imaging and histological findings. Treatment follows similar regimens to other vasculitic presentations, with glucocorticoids in conjunction with immunosuppressive agents, and management of intercurrent sepsis and the increased risk of cardiovascular and thromboembolic events. Prompt diagnosis and intensive treatment of pulmonary vasculitis is essential to improve early mortality and long-term outcomes.

Pulmonary Vasculitis in the Intensive Care Unit

Pulmonary vasculitis can occur in apparent isolation, as part of a primary systemic vasculitis, or with an underlying systemic inflammatory autoimmune disorder. The presentation of pulmonary vasculitis in the intensive care unit (ICU) can be fulminant and will often overlap with more common disorders that affect the critically ill. Although diffuse alveolar hemorrhage (DAH) is the clinical feature that often initiates the concern for an underlying vasculitis, hemoptysis may not be apparent or its presentation can be mistaken for an alternative disease process. As a result, the diagnosis of pulmonary vasculitis in the ICU may be delayed or be completely unrecognized. A high level of suspicion is essential to obtain a timely diagnosis and for effective therapies to be implemented. There have been significant advances this past decade in diagnostic strategies as well as in the therapeutic options for patients with pulmonary vasculitis. We review here the clinical presentations, diagnostic strategies, and treatment options of the critically ill patients presenting with pulmonary vasculitis. The reader is referred to other resources for a more comprehensive review of specific vasculitic entities.

Pulmonary vasculitis.

The granulomatous vasculitides frequently involve the lung. These syndromes include Wegener's granulomatosis, allergic angiitis and granulomatosis, and the polyangiitis overlap syndrome. Although not a true systemic vasculitis, necrotizing sarcoid granulomatosis also represents a type of pulmonary vasculitis. It is clear that many infectious agents can cause a picture in the lung that can be confused with granulomatous vasculitis and that an infectious process must be ruled out before a diagnosis of pulmonary vasculitis can be established. Pulmonary vasculitis can be associated with the hypersensitivity vasculitides, and pulmonary hemorrhage can be secondary to pulmonary capillaritis. Therapy of the hypersensitivity vasculitides consists of removing the offending antigen and instituting a limited course of corticosteroids. If the vasculitis is secondary to an underlying disease, such as lymphoma, therapy should be directed at the primary disease. Combination therapy with cyclophosphamide and corticosteroids is effective in the systemic vasculitides and the 5-yr survival rate is approximately 90%.

Pulmonary Vasculitis Complicating Childhood Ulcerative Colitis

A case of pulmonary vasculitis complicating ulcerative colitis is presented, and the literature is reviewed. Unexplained pulmonary problems complicating severe ulcerative colitis should prompt a review of rare etiologies to include pulmonary vasculitis, apical pulmonary fibrosis, and salicylazosulfapyridine-induced lung disease. Lung biopsy will establish a diagnosis of pulmonary vasculitis.

Pulmonary abnormalities in the necrotizing vasculitides and their rapid response to steroids.

Abstract Pulmonary disease was studied in 23 patients with periarteritis nodosa, Wegener's granulomatosis, or other forms of necrotizing vasculitis. Primary lung lesions consisting of patchy infiltrates or nodules, caused by pulmonary vasculitis, often develop in such patients. Secondary lesions such as uremic pulmonary edema or pulmonary infarction also occur. The specific radiological diagnosis of pulmonary vasculitis can reliably be made, based upon the character of the lesions, the rarity of superimposed infection, and a rapid and dramatic response to steroids.