Critical Care Medicine

Immunologic lung disease

Immunological lung disease

Synonyms

Connective tissue disease-associated lung diseases, collagen vascular diseases, vasculitides,idiopathic interstitial pneumonias

Related conditions

Acute interstitial pneumonitis (AIP), acute idiopathic eosinophilic pneumonia (EAP), acute exacerbation of interstitial lung disease (AE-ILD), acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF), systemic lupus erythematosus (SLE), bone marrow transplantation (BMT), associated idiopathic pulmonary syndrome, diffuse alveolar hemorrhage (DAH), vasculitis, ANCA-associated vasculitis, Wegener's granulomatosis (WG), microscopic polyangiitis (MPA), Churg-Strauss vasculitis (CSV), idiopathic Pauci-immune pulmonary capillaritis (IPIPC), Goodpasture's syndrome (GS), Henoch-Schonlein purpura (HSP), rheumatoid arthritis (RA), scleroderma (SSc), polymyositis/anti-synthetase syndromes

1. Description of the problem

Immunologic lung diseases are not a disease per se, but rather a collection of disorders clinically grouped together based upon (i) an autoimmune or idiopathic pathophysiologic mechanism and (ii) similar clinical-radiologic-pathologic presentations.

While each of these individual entities occur with very low frequency, identifying these disorders and distinguishing them from more common entities with similar presentations is critical. Often the clinical management of these disorders may be markedly different from competing diagnostic considerations such as pneumonia, ARDS or aspiration.

As many of these diseases are often thought of as having primarily a chronic, progressive course, critical care physicians may not necessarily be as familiar with the underlying disorders as their rheumatology or interstitial lung disease (ILD) colleagues. Conversely, rheumatologists or ILD specialists that provide these patients longitudinal outpatient care may not be intimately familiar with the acute, life-threatening presentations that can be associated with these disorders.

Immunologic lung disease as a mimic of ARDS

Patients may present with acute onset (hours, days or weeks) shortness of breath, hypoxemia and pulmonary infiltrates. Indeed, patients may fulfill the American-European Consensus Conference criteria for ARDS; however, patients with acute eosinophilic pneumonia (AEP) or diffuse alveolar hemorrhage (DAH) obviously do not have a pathologic correlation of diffuse alveolar damage (DAD).

Similarly, patients with acute interstitial pneumonitis (AIP), an acute exacerbation of ILD (AE-ILD), hematopoietic stem cell transplantation (HSCT) associated idiopathic pneumonia syndrome (HSCT-IPS) or acute lupus pneumonitis (ALP) may have a pathologic finding of diffuse alveolar damage but are generally not considered to have ARDS due to the pathophysiologic mechanism of their disorder. Patients who present in this fashion may also complain of fever, fatigue, malaise, cough and hemoptysis. as well as the more pronounced features of dyspnea and hypoxemia.

Immunologic lung disease as a cause of diffuse alveolar hemorrhage

As outlined the in chapter on alveolar hemorrhage syndromes, patients with DAH will have acute onset cough, dyspnea, fever, hypoxemia and patchy alveolar infiltrates. Only 50-67% will have hemoptysis Patients with DAH from immunologic lung disease will be defined by a pathologic lesion of capillaritis.

Infectious complications in immunologic lung diseases

Infection is an extremely common complication of immunologic lung disease and a leading cause of morbidity and mortality in disorders such as vasculitis, SLE and bone marrow transplantation. Patients may present with routine or atypical clinical presentations and/or routine or atypical organisms (bacterial, viral, fungal, mycobacterial, Nocardia sp, Pneumocystis jiroveci, etc).

The importance of assessing for concomitant infection cannot be overemphasized. Infections may commonly be missed due to the overlap of signs and symptoms with disease activity and/or drug toxicity, the immunocompromised nature of the host and the high frequency of co-morbid infections in the setting of other confounding acute and chronic disease processes.

To illustrate this point, it should be noted that 13-26% of vasculitis related deaths are secondary to infection, and 50% of patients with acute lupus pneumonitis have a concomitant infection.

Drug toxicity in immunologic lung diseases

Many medications used to treat immunologic lung diseases carry their own significant risks of toxicities and adverse side effects. Methotrexate-induced pneumonitis occurs in 2-8% of patients maintained on chronic therapy. Cyclophosphamide has been associated with a sub-acute pneumonitis and pulmonary fibrosis, albeit at very low frequencies (<1%).

Moreover, the use of immunosuppressive and cytotoxic agents increases the risk for infectious complications. Regular, protocolized screening for adverse side effects and toxicities of medications such as cyclophosphamide, azathioprine, mycophenolate mofetil, methotrexate, TNF antagonists and corticosteroids should be a regular component of the longitudinal care of the immunologic lung disease patient.

Immunologic lung diseases as a cause of airways disease

Primary connective tissue disorders may present with bronchiectasis and/or bronchiolitis. Asthma is one of the three legs of the classic triad of Churg-Strauss vasculitis (asthma, eosinophilia and vasculitis), and patients will often have severe persistent and/or steroid-dependent asthma. Status asthmaticus is a significant contributor to Churg-Strauss associated morbidity and mortality.

Other airway complications that may be seen in the immunologic lung diseases include subglottic, tracheal or bronchial stenosis, bronchomalacia, hemorrhage, and segmental or lobar collapse from airway narrowing/occlusion.

Immunologic lung diseases as a cause of pleural disease

Pleural disease is extremely common in SLE, affecting 50-70% of patients over the course of their illness. Other immunologic lung diseases may present with pleural effusions, pleuritis and rarely pleural fibrosis. New effusions in the setting of immunologic lung disease require further evaluation as, while they may represent an inflammatory effusion, infection, heart failure and pulmonary embolus are also possibilities.

Aspiration

Patients with systemic autoimmune diseases in general, and scleroderma/CREST syndrome in particular, are at increased risk of aspiration events

Venous thromboembolic disease

Venous thromboembolic disease presenting as either a pulmonary embolism or deep venous thrombosis is common in autoimmune diseases in general, and in SLE, anti-phospholipid antibody syndrome and Wegener's Granulomatosis in particular.

For example, the Wegener's Clinical Occurence of Thrombosis study demonstrated a rate of VTE of 7.0/100 persons-years in patients with Wegener's granulomatosis, a rate comparable to patients with known prior VTE events. While not traditionally thought of as an immunologic lung disease, the high frequency of events and the morbidity and mortality associated with these events elevate VTE as a significant concern in any patient with any underlying autoimmune disease and new chest symptoms.

Cardiac disease

Pulmonary edema/heart failure may occur as a result of an underlying immunologic disease. As such, the heart as well as the lung must be evaluated in a patient with respiratory distress and an underlying autoimmune process. Churg-Strauss vasculitis and Wegener's granulomatosis in particular may manifest with a cardiomyopathy, coronary arteritis, conduction delay, arrhythmia or other cardiac disorder. SLE more commonly leads to valvular heart disease or pericarditis.

2. Emergency Management

As with all critical illness, the initial stabilization of the patient and addressing the "ABCs" are critical first steps. Patients with impending respiratory failure, whether from DAH, AIP, ALP or pneumonia should be placed on ventilatory support. Patients with shock require hemodynamic support with intravenous fluids, blood products and/or vasopressors.

Only once the patient has been stabilized can the clinician consider more targeted interventions such as plasma exchange or corticosteroids. That said, rapid implementation of disease-specific therapies, such as plasma exchange for diffuse alveolar hemorrhage from ANCA-associated vasculitis or Goodpasture's syndrome is critical to achieving a good outcome.

Therefore, the ICU clinician caring for these patients should focus not only on optimizing the supportive care elements, but also achieving a rapid and correct diagnosis that permits optimal disease-specific therapy as well. In some instances, life-saving therapies, such as our plasma exchange example above, may need to be initiated based upon a strong clinical suspicion, even as confirmatory diagnostic studies remain pending.

3. Diagnosis

The diagnosis of immunologic lung diseases often requires the clinician to integrate clinical, radiologic, laboratory and pathologic information to decide whether or not the preponderance of the evidence supports a diagnosis of a vasculitis, a connective tissue disease, an idiopathic interstitial pneumonia or other entity classified under the immunologic lung disease heading.

While the presence of a pre-existing fibrotic lung disease or autoimmune disease is extremely helpful in generating an appropriate differential diagnosis, the absence of a pre-existing disorder does not exclude the possibility of an immunologic lung disease. Clinicians need to recognize when a presentation of pneumonia or ARDS does not follow the expected clinical course.

For example, patients ultimately diagnosed with ARDS should have an identified inciting injury such as sepsis, pneumonia, pancreatitis, aspiration or trauma. The absence of an appropriate antecedent history suggests the possibility of an alternative diagnosis, such as an immunologic lung disease, and strong consideration should be given to bronchoscopy with bronchoalveolar lavage, additional laboratory studies (e.g. connective tissue disease serologies), and/or additional imaging studies such as high resolution computed tomography of the chest (HRCT).

Similarly, patients who present with bilateral radiographic infiltrates but no significant fever, leukocytosis or sputum production cannot be assumed to have "atypical" pneumonia but rather the clinician should recognize that this presentation carries an extended differential diagnosis deserving of further investigation.

A comprehensive evaluation of patients with possible immunologic lung disease includes some combination of the following elements as clinical circumstances dictate:

  • Detailed history and review of systems.

  • Full medication, occupational, social, family, environmental and travel histories.

  • A thorough and detailed physical examination (especially looking for evidence of extra-pulmonary disease).

  • Complete blood count.

  • Liver and renal function testing.

  • Connective tissue disease serologies (anti-nuclear antibodies, rheumatoid factor, anti-cyclic citrullinated peptide antibodies, anti-topoisomerase antibodies, anti-SS-A and SS-B antibodies, hepatitis serologies, anti-neutrophil cytoplasmic antibody testing, anti-proteinase-3 and anti-myeloperoxidase ELISA testing, anti-glomerular basement membrane antibodies, anti-phospholipid antibodies).

  • Electrocardiogram.

  • Chest imaging studies (chest radiograph and/or HRCT).

  • Bronchoscopy with bronchoalveolar lavage.

And, if needed:

  • Surgical lung biopsy or biopsy of other target organs (sinus, kidney or skin).

Acute interstitial pneumonitis

AIP occurs either as an idiopathic interstitial pneumonia or in the setting of a connective tissue disease such as rheumatoid arthritis. Known causes of ARDS must be excluded to make a diagnosis of AIP (i.e. exclude a precipitating episode of sepsis, pneumonia, aspiration, trauma or pancreatitis).

AIP presents with acute-onset hypoxemia and respiratory distress, often preceded by a 1-2 week prodromal illness characterized by fatigue, malaise, low-grade fevers, cough, and dyspnea. Mean age of onset is 40-60 years. Laboratory data is non-specific and chest radiograph demonstrates bilateral airspace disease.

HRCT will generally demonstrate bilateral, heterogenous ground glass opacities and/or areas of air space consolidation with or without modest degrees of traction bronchiectasis and/or honeycombing. Should the patient undergo biopsy, histopathology demonstrates an organizing diffuse alveolar damage pattern (DAD). Mortality is greater than 50% and recurrence is common in survivors.

Acute exacerbation of interstitial lung disease

Patients with AE-ILD should fulfill the following criteria: (i) a previous or concurrent diagnosis of a fibrosing interstitial pneumonia, (ii) unexplained worsening or development of dyspnea in 30 days or less, (iii) new ground glass or consolidative changes on HRCT imaging superimposed on fibrotic changes, (iv) no evidence of pulmonary infection, and (v) the exclusion of other causes of acute lung injury.

AE-ILD was first described in patients with idiopathic pulmonary fibrosis (AE-IPF), and this remains the most common underlying disease state; however, AE-ILD has been described in patients with connective tissue disease-associated ILD, chronic fibrotic hypersensitivity pneumonitis, drug-induced interstitial lung disease and non-specific interstitial pneumonitis.

Patients commonly have new hypoxia or increased oxygen requirements associated with their dyspnea and infiltrates, and patients may report cough, hemoptysis, fatigue, malaise, fever, anorexia, nausea, vomiting, myalgias or other symptoms.

Laboratory findings are non-specific. Surgical lung biopsy will commonly demonstrate an organizing diffuse alveolar damage superimposed on an underlying fibrosing interstitial pneumonia, but a minority will have organizing pneumonia that may respond to corticosteroid therapy. In-hospital and 28-day mortality range from 50-70%, and survivors have a relatively poor prognosis.

Acute lupus pneumonitis

Patients present with acute onset cough, dyspnea, fever, fatigue, malaise and hypoxemia. ALP will not infrequently progress to respiratory failure. ALP may be an initial presentation of SLE in up to 50% of patients and affects 1-4% of patients with SLE. Concomitant infection occurs in 50% of patients. Chest imaging demonstrates patchy alveolar infiltrates and pathology demonstrates DAD. ALP has a reported mortality rate of up to 50%.

Bone marrow transplantation associated idiopathic pulmonary syndrome

Patients present 1-3 months after bone marrow transplantation with cough, dyspnea, fever, anorexia, fatigue, malaise and hypoxemia. Respiratory failure occurs in 60-80% of patients. Hypoxemic respiratory failure/distress in the clinical context of recent BMT generates the following differential diagnosis: bacterial or fungal infection, cytomegalovirus pneumonitis, alveolar hemorrhage, graft versus host disease/bronchiolitis obliterans and idiopathic pulmonary syndrome.

BMT associated idiopathic pulmonary syndrome occurs in both autologous and allogeneic marrow recipients with an incidence of approximately 5-8%. Chest imaging demonstrates patchy alveolar infiltrates and pathology demonstrates DAD. As with ALP, concomitant infection occurs relatively frequently, on the order of 25-50%. Mortality of idiopathic pulmonary syndrome is 50-90%.

Acute eosinophilic pneumonia

Patients are commonly young (age 20-50) and present with acute onset (<7 days) dyspnea and hypoxemia, often accompanied by cough, fever, fatigue, malaise and chest pain. The disease progresses rapidly and commonly culminates in respiratory distress/failure. Chest radiographs show bilateral reticular, reticulonodular, and/or alveolar infiltrates, and similarly, HRCT demonstrates bilateral, patchy heterogeneous ground glass attenuation and/or airspace consolidation.

While peripheral eosinophilia is often absent at the time of initial presentation (in stark contrast to chronic eosinophilic pneumonia), eosinophilia on bronchoalveolar lavage (>25% eosinophils on differential cell count) is diagnostic of an eosinophilic pneumonia. In those patients who undergo biopsy, histopathology will reveal pathologic eosinophilic pneumonia often accompanied by a superimposed component of DAD. Some cases of acute eosinophilic pneumonia may be due to drugs/medications (e.g. trazodone, venlafaxine, daptomycin).

Diffuse alveolar hemorrhage syndromes

Patients with DAH present with dyspnea and hypoxemia. While hemoptysis, anemia and diffuse alveolar infiltrates are considered the "classic triad" of DAH, only 2/3 of patients will complain of hemoptysis, and anemia is a non-specific finding in the critically-ill patient population.

The diagnosis of diffuse alveolar hemorrhage is made at the time of bronchoscopy with bronchoalveolar lavage. Aliquots of saline are serially instilled and withdrawn with the bronchoscope in wedge position. The diagnosis of DAH is made if and when the serial lavage fluid demonstrates a hemorrhagic return that does not clear or paradoxically worsens with serial lavage.

The differential diagnosis of DAH includes those entities characterized by a pathologic lesion of capillaritis (ANCA-associated vasculitis - Wegener's granulomatosis (WG), microscopic polyangiitis (MPA), Churg-Strauss vasculitis (CSV) and idiopathic pauci-immune pulmonary capillaritis (IPIPC); immune complex mediated diseases - Goodpasture's syndrome (GS), Henoch-Schonlein purpura (HSP), post-streptococcal disease and cryoglobulinemia; secondary vasculitis - SLE, anti-phospholipid antibody syndrome; and drug-induced disease), and those characterized by bland hemorrhage (coagulopathy, mitral stenosis, idiopathic pulmonary hemosiderosis).

See section entitled "DAH syndromes" for a further discussion of DAH and the pulmonary vasculitides. The combination of DAH with a concomitant finding of glomerulonephritis is diagnostic of a "pulmonary-renal syndrome" and narrows the differential to small vessel vasculitis (WG, MPA, CSV, IPIPC), Goodpasture's syndrome, cryoglobulinemia and post-streptococcal disease.

4. Specific Treatment

A cross-specialty, multi-disciplinary approach involving pulmonologists, rheumatologists, pharmacists and critical care physicians is recommended for the management of these complex diseases and their equally complex treatment regimens. Patients will demonstrate a broad range of disease severity and often will have multiple and heterogeneous manifestations and/or co-morbidities of the underlying autoimmune or idiopathic disease process.

Moreover, aggressive and/or prolonged immunosuppression titrated to the disease severity may be associated with significant adverse effects and requires close and careful longitudinal monitoring, ideally with one or more practitioners experienced in the administration and monitoring of these agents.

Acute interstitial pneumonitis

Patients with AIP are treated with supportive care. While it remains unclear whether or not corticosteroids provide significant benefit, most clinicians attempt a trial of therapy with high dose, intravenous corticosteroids (60-250 mg intravenous methylprednisolone every 6 hours for 3 days that is then tapered as dictated by clinical response). Patients with positive clinical responses are generally treated with prolonged courses of oral corticosteroids and/or cytotoxic steroid-sparing agents such as azathioprine, mycophenolate mofetil or cyclophosphamide.

Acute exacerbation of interstitial lung disease

Patients generally receive broad-spectrum anti-microbial therapy and high dose intravenous corticosteroids (along the lines outlined above); however, it is unclear whether or not this regimen provides significant benefit. In those patients who respond to intravenous corticosteroid therapy, most clinicians recommend transitioning to oral corticosteroids and then introducing a steroid-sparing, cytotoxic agent such as azathioprine or mycophenolate mofetil.

A small clinical trial suggested that patients maintained on anti-coagulation might have lower rates of AE-ILD, and this hypothesis is currently being tested in the prospective, randomized, placebo-controlled ACE-IPF clinical trial (Anticoagulant Effectiveness in Idiopathic Pulmonary Fibrosis) sponsored by the National Institutes of Health.

Acute lupus pneumonitis

Acute lupus pneumonitis is treated with high dose intravenous corticosteroids. Given the high incidence of concomitant infection, patients frequently also receive broad-spectrum anti-microbial therapy. As patients recover from the acute illness, the intravenous steroids are tapered over days/weeks and transitioned to oral corticosteroids with or without the addition of a concomitant cytotoxic, steroid-sparing agent such as azathioprine, methotrexate, cyclophosphamide or mycophenolate mofetil.

Acute eosinophilic pneumonia.

Intravenous corticosteroids result in rapid improvement. Dosing regimens may vary but, similar to other immunologic lung diseases, therapy with high dose intravenous steroids is recommended. Steroid therapy is then tapered according to the clinical response. Unlike the other immunologic lung diseases, the corticosteroids may be tapered off over approximately 2-8 weeks, and resolution without recurrence is a hallmark of idiopathic AEP.

Bone marrow transplantation associated idiopathic pulmonary syndrome

At present, corticosteroids initiated at a dose of 2 mg/kg/day (with tapering as dictated by clinical response) and supportive care represent standard therapy. There is interest, however, in exploring a role for TNF antagonists, and the National Institutes of Health/NHLBI is conducting a phase III, prospective, randomized, placebo-controlled study evaluating the effectiveness of etanercept for the treatment of idiopathic pneumonia syndrome following stem cell transplantation

Diffuse alveolar hemorrhage (DAH) syndromes

Diffuse alveolar hemorrhage is generally classified as a life-threatening disease. As such, those ANCA-associated vasculitides that present with DAH are treated with combination therapy that includes intravenous corticosteriods, plasma exchange and cyclophosphamide. Practically, the corticosteroids and plasma exchange are initiated as early as possible. Plasma exchange is performed for 5-7 days and tailored to clinical response.

The initial recommended dose of corticosteroids is 250 mg IV methylprednisolone every 6 hours for 3 days. Intravenous corticosteroid dosage is then carefully de-escalated over a period of 1-2 weeks (e.g. 125 mg IV every 6 hours x 3 days then 60 mg IV every 6 hours x 3-7 days) until patients can safely be transitioned to high dose oral corticosteroids (1 mg/kg/day of prednisone or equivalent). Ultimately, steroid dosage must be tailored to clinical circumstances.

The timing of induction therapy with cyclophosphamide remains controversial and at the discretion of the physician providing direct patient care. The risk of adverse side effects and complications associated with the use of cyclophosphamide in critically ill patients must be weighed carefully against the risk of delaying cytotoxic therapy.

Additionally, the recently published Rituximab versus Cyclophosphamide for ANCA-associated Vasculitis (RAVE) trial demonstrated that rituximab was as effective as cyclophosphamide in the treatment of patients with major renal disease and alveolar hemorrhage. Therefore, some clinicians will now deploy rituximab in lieu of cyclophosphamide for antibody positive, ANCA-associated vasculitis presenting with alveolar hemorrhage.

Finally, activated factor VII has been used in a handful of patients with DAH where the hemorrhage is refractory to intravenous corticosteroids, plasma exchange, cytotoxic therapy and best supportive care with good results at the case report level.

5. Disease monitoring, follow-up and disposition

Patients found to have an immunologic lung disease or complications thereof benefit from a comprehensive, multi-disciplinary approach to care. Appropriate sub-specialty consultation (rheumatology, pulmonology, immunology and/or nephrology) should be obtained early in the clinical course and comprehensive longitudinal care assured.

Pathophysiology

The disorders characterized under the immunologic lung disease heading all have either an idiopathic or autoimmune etiology. The reader is directed to the specific chapters dealing with each of these disorders within Decision Support to learn more about the pathophysiology underlying each of the individual disorders.

Epidemiology

The disorders characterized under the immunologic lung disease heading are heterogeneous and have varying epidemiology. The reader is directed to the specific chapters dealing with each of these disorders within Decision Support to learn more about the epidemiology of the individual disorders.

Prognosis

Prognosis is determined by the specific pulmonary manifestation and severity of the presentation, the underlying idiopathic or autoimmune disorder and the additional extra-pulmonary manifestations and/or co-morbidities associated with each individual patient presentation.

For example, the idiopathic AEP has an excellent prognosis whereas BMT associated idiopathic pulmonary syndrome and AE-IPF have very poor prognoses. Still, the identification of the correct underlying diagnosis and assessing for concomitant infection, providing the best possible supportive care and instituting correct disease-specific therapies, can greatly improve prognosis.

Special considerations for nursing and allied health professionals.

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What's the evidence?

Description of the problem

(An autopsy study demonstrating that the clinical definition of ARDS is only 75% sensitivity and 84% specific. Entities such as DAH may be mis-diagnosed as ARDS.)

(A review of autopsy results in patients who died with ARDS demonstrated that 23% of patients had major "unexpected" findings.)

(Retrospective cohort study examining frequency and causes of ARDS in patients with SLE. 47% of deaths were due to sepsis or bacteremia, 11% to fungal pneumonia, 11% to alveolar hemorrhage and 5% to acute lupus pneumonitis.)

Emergency management

Diagnosis

(Largest reported case series [13] of patients with AIP.)

(Detailed review of AIP and AE-IPF.)

(Idiopathic Pulmonary Fibrosis Clinical Network perspective on acute exacerbations of IPF including case definition.)

(Clinical and radiographic characterization of AE-IPF with a detailed analysis of the radiographic findings.)

(Clinical characterization of 27 patients hospitalized with AE-ILD with detailed results of a comprehensive diagnostic evaluation.)

(Excellent review of acute, life-threatening manifestations of systemic lupus erythematosus.)

(Largest single case series of acute lupus pneumonitis reported in the literature.)

Acute eosinophilic pneumonia (AEP)

Li, H., Groshong, S.D., Lynch, D.A., Brown, K.K., Frankel, S.K.. "Eosinophilic Lung Diseases". Clinical Pulmonary Medicine. vol. 17. 2010. pp. 66-74.

(Detailed current review of the eosinophilic lung diseases.)

(Large case series (15) of patients with acute eosinophilic pneumonia with detailed clinical characterization and review of the literature.)

(Radiographic characterization of an AEP patient cohort.)

(Histopathologic characterization of an AEP patient cohort.)

(NHBI workshop summary on idiopathic pneumonia syndrome following bone marrow transplantation.)

(Clinical review of idiopathic pulmonary syndrome after bone marrow transplantation.)

(Clinical review of idiopathic pulmonary syndrome after bone marrow transplantation.)

(Excellent clinical review of diffuse alveolar hemorrhage syndromes.)

(Detailed current review of the pulmonary vasculitides.)

Specific treatment

(Small, prospective trial demonstrating that patients receiving prednisolone plus anti-coagulant therapy had improved survival compared with patients treated with prednisolone alone. This study informs the design of the ACE-IPF trial.)

(Results of a small, open-label trial of etanercept plus corticosteroids for the treatment of BMT associated idiopathic pulmonary syndrome.)

(The Randomized Trial of Plasma Exchange or High-Dosage Methylprednisolone as Adjunctive Therapy for Severe Renal Vasculitis (MEPEX) trial demonstrates that plasma exchange is superior to high-dose intravenous corticosteroids for the treatment of severe renal vasculitis.)

(A 20 patient case series demonstrating that plasma exchange represents effective therapy for diffuse alveolar hemorrhage.)

(The Rituximab versus Cyclophosphamide for ANCA-Associated Vascultis (RAVE) trial demonstrates that rituximab (anti-CD19 monoclonal antibody) was as effective as cyclophosphamide in the treatment of patients with major renal disease and alveolar hemorrhage as cyclophosphamide.)

(The EUVAS sponsored Rituximab versus Cyclophosphamide in ANCA-associated Renal Vasculitis trial found similar rates of sustained remission in the rituximab and cyclophosphamide groups.)

(Prospective, randomized, controlled trial of pulse versus daily oral cyclophosphamide for induction of remission in antineutrophil cytoplasmic antibody associated vasculitis that shows no differences in time to remission but a lower complication rate in the intravenous cyclophosphamide arm.)

Disease monitoring, follow-up and disposition

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