Management of virus-induced systemic vasculitides

The best therapeutic strategy in virus-induced vasculitides should take into account the etiology of the disease and be adapted to the pathogenesis. The combination of antiviral treatments and plasma exchanges has been proven effective in polyarteritis nodosa. In HIV-related vasculitis, this strategy is effective and does not jeopardize the outcome of AIDS, as do cytotoxic agents. In vasculitis related to hepatitis C virus-associated cryoglobulinemia, plasma exchanges improve the outcome, but the poor effectiveness of antiviral drugs usually does not favor a definite recovery of the patients. Relapses are frequent.     

Diffuse alveolar hemorrhage

Diffuse alveolar hemorrhage represents a medical emergency, and clinicians must have an expedient approach to its identification. There are many causes of diffuse alveolar hemorrhage, including vasculitides, immunologic conditions such as Goodpasture's syndrome, collagen vascular disease, and idiopathic conditions. Careful attention to the medical history, physical examination, and targeted laboratory evaluation often suggests the underlying cause. Patients in whom the diagnosis of diffuse alveolar hemorrhage remains uncertain should undergo diagnostic bronchoscopy. In patients with evidence of diffuse alveolar hemorrhage and renal involvement, kidney biopsy should be considered to identify the underlying cause and help direct therapy.     

弥漫性肺泡出血

弥漫性肺泡出血是以咯血、缺铁性贫血和胸片以暂时性弥漫肺泡浸润或实变为特征的临床综合征。此综合征可见于一组各种各样的异质性疾病，其中许多疾病还共有肾小球肾炎的临床表现，并可表现为Good-pasture综合征而增加诊断的复杂性和治疗的难度，本文对弥漫性肺泡出血的分类和Good—pasture综合征诊断的主要问题进行了评述。     

Diffuse alveolar hemorrhage

Diffuse alveolar hemorrhage is a clinical syndrome that can be life threatening if not diagnosed and treated in time. In most cases it occurs largely as a result of small-vessel vasculitis of the lungs. The many different forms can be classified into 3 large groups: a) pauciimmune disease, which generally involves pulmonary capillaritis and is associated with the presence of antineutrophil cytoplasmic antibodies; b) syndromes caused by immune deposits, which can be detected by immunofluorescence; and c) a large miscellaneous group that includes drug reactions, infections, and idiopathic disease. Diagnosis is based on a combination of signs, symptoms, serology, and histology. Biopsy with video-assisted thoracoscopy should be recommended in patients with diffuse alveolar hemorrhage without known cause and with no prior diagnosis of systemic disease, in whom serology studies do not reveal conclusive data, and in general in those patients for whom there is a high level of suspicion of diffuse alveolar hemorrhage. In all such cases, the fresh biopsy material should be sent to the pathology laboratory for preparation of frozen sections to be used for immunofluorescence.     

Lupus nephritis associated with bacterial panperitonitis and lung alveolar hemorrhage.

A 36-year-old woman was admitted to the hospital with the diagnosis of nephrotic syndrome due to lupus nephritis. The patient had panperitonitis caused by Staphylococcus aureus as a complication, and an emergency laparotomy was performed. After the operation, the patient developed a massive lung alveolar hemorrhage. Methylprednisolone pulse therapy showed a marked effect on the lung hemorrhage. It is known that lung alveolar hemorrhages associated with systemic lupus erythematosus have a very high mortality; the present case is relatively rare because of the good response to steroid pulse therapy.     

Diffuse alveolar hemorrhage syndromes

Diffuse alveolar hemorrhage (DAH) is a rare yet serious and frequently life-threatening complication of a variety of conditions. DAH may result from coagulation disorders, inhaled toxins, or infections. Most cases of DAH are caused by capillaritis associated with systemic autoimmune diseases such as antineutrophil cytoplasmic antibodies-associated vasculitis, anti-glomerular basement membrane disease, and systemic lupus erythematosus. Early recognition is crucial, because the prompt institution of supportive measures and immunosuppressive therapy is required for survival. Our understanding of DAH and its management is largely empiric and based on small case series and individual reports, many dating back more than one decade. To provide the practicing specialist with a rational diagnostic and management approach to the patient with DAH, this review summarizes the most recent publications and salient information derived from older publications.     

Subclinical alveolar bleeding in pulmonary vasculitides: correlation with indices of disease activity.

Haemosiderin-laden alveolar macrophages are a common finding in patients with alveolar bleeding. Iron-positive macrophages, suggestive of subclinical alveolar bleeding, were found to be fairly common in bronchoalveolar lavage (BAL) fluid in primary systemic vasculitis but uncommon in collagen vascular diseases (CVDs) and rheumatoid arthritis (RA). To substantiate the impression that subclinical alveolar bleeding may be a feature distinguishing between these disorders, fibreoptic bronchoscopy and BAL were performed in 49 patients with active Wegener's granulomatosis or Churg-Strauss syndrome and 44 patients with CVDs or RA, all of them without clinically manifest alveolar bleeding. The percentage of iron-positive cells was compared with clinical and radiological findings. Only a minority of the CVD and RA patients had iron-positive alveolar macrophages; the 95th percentile of the median number of such cells was 5%. Fifty-three per cent of the patients in the vasculitis group had >5% iron-positive cells, with individual counts ranging up to 95%. Patients with iron-positive macrophages had more extensive disease, more frequent microhaematuria, a higher antineutrophil cytoplasmic antibody titre, a higher myeloperoxidase concentration in the BAL fluid and somewhat more frequent low-attenuation opacities in pulmonary high-resolution computed tomography than the patients with a low iron-positive cell count. In conclusion, subclinical alveolar bleeding was, indeed, a common finding in antineutrophil cytoplasmic antibody-associated vasculitis, which distinguished these disorders from lung disease due to collagen vascular diseases or rheumatoid arthritis. Its association with indices of disease activity, although weak in this cross-sectional study, merits a longitudinal study of its value for the long-term monitoring of vasculitis patients.     

Antiphospholipid antibodies-associated diffuse alveolar hemorrhage

ObjectivesTo describe the clinical features and outcomes of 17 patients with primary antiphospholipid syndrome (PAPS) or antiphospholipid antibodies (aPL) and diffuse alveolar hemorrhage (DAH).MethodsWe reviewed the medical records of all patients diagnosed with PAPS-associated DAH and aPL-associated DAH between January 1, 1997, and December 31, 2013, for clinical features, laboratory and radiographic findings, management, and outcomes.ResultsA total of 17 patients met the criteria for DAH and had aPL and 10 patients met the criteria for PAPS. The mean age at DAH diagnosis was 57.6 years. Secondary causes of DAH were ruled out. Surgical lung biopsy was performed in 6 cases, 5 of whom had bland hemorrhage. Pulmonary capillaritis was present in only 1 case. Four patients (3 with aPLs and 1 with PAPS) achieved complete remission despite receiving no treatment. The majority of patients treated received initial corticosteroids. Additionally, cyclophosphamide (2 cases), rituximab (1 case), plasma exchange (2 cases), methotrexate (1 case), azathioprine (1 case), and hydroxychloroquine (2 cases) were used. In total, 10 patients (59%) achieved complete and sustained remission with a median length of follow-up of 48 months. Four patients (23%) died (2 with PAPS and 2 with aPLs), all from uncontrolled DAH. Three patients (18%) relapsed after achieving complete remission.ConclusionsDAH is a rare complication of PAPS that can also arise de novo in aPL-positive individuals. Lung pathology shows either bland hemorrhage or capillaritis. Recognition of this unusual but known complication is important, since early diagnosis and therapy could potentially affect outcomes.     

Diffuse alveolar hemorrhage following alemtuzumab

This study describes an unusual patient with X-linked Alport syndrome (XLAS) in whom diffuse alveolar hemorrhage (DAH) developed as a complication of alemtuzumab therapy following renal transplantation. A 26-year-old man with XLAS underwent retransplantation with a cadaveric renal allograft. He received alemtuzumab therapy as a part of an immunosuppressive induction protocol, and dyspnea and hemoptysis developed. A chest CT scan showed diffuse alveolar opacities. Bronchoscopy was performed to determine the cause of hemoptysis and hypoxia. BAL showed a characteristic increasingly bloody return in the sequential aliquots. There was no growth of pathogenic bacteria or evidence of opportunistic infection. Clinical improvement occurred with the initiation of steroids, and the patient required short-term mechanical ventilation for acute respiratory failure. To our knowledge, this is the first reported case of DAH associated with use of alemtuzumab therapy, although other pulmonary toxicities have been described. The prevalence of this form of pulmonary toxicity is unclear and requires further systematic study.     

Diffuse alveolar hemorrhage secondary to sarcoidosis

Clinical Rheumatology - Diffuse alveolar hemorrhage (DAH) is a pulmonary condition that can be caused by autoimmune disorders such as lupus, small vessel vasculitis, and antiphospholipid syndrome....     

Angiosarcoma presenting as diffuse alveolar hemorrhage

Angiosarcoma has been reported as occurring in both postirradiation and postradical mastectomy patients. Described is a patient, postmastectomy and irradiation, with alveolar hemorrhage secondary to angiosarcoma. Angiosarcoma, primary or metastatic to lung, should be included in the differential diagnosis of diffuse alveolar hemorrhage in this patient population.     

MPO-ANCA related diffuse alveolar hemorrhage]

The purpose of this study was to elucidate the clinical features characterizing patients with myeloperoxidase specific-antineutrophil cytoplasmic antibody (MPO-ANCA) related diffuse alveolar hemorrhage (DAH). Seventeen MPO-ANCA-positive patients were evaluated. Nine patients (52.9%) had pulmonary involvement; of those, 6 (35.3%) had DAH, and 4 (23.5%) had interstitial pneumonia (1 patient had both pulmonary diseases). Three of the patients with DAH demonstrated only mildly bloody sputum. All patients with DAH had increased peripheral white blood cell counts, high titers of C-reactive protein and MPO-ANCA, and marked microscopic hematuria. DAH was diagnosed in all cases by fiberoptic bronchoscopy with bronchoalveolar lavage. All patients with DAH were treated with three pulses of methylprednisolone, and 5 were treated with cyclophosphamide. Three of the patients with DAH required mechanical ventilation for respiratory insufficiency, but 2 were relieved of that need by immunosuppressive therapy. In spite of intensive care, 1 patient died of respiratory failure and 2 died of complications related to therapy. The prognosis for patients with DAH is poor. We emphasize the importance of prompt and accurate diagnoses and aggressive care, including immunosuppressive therapy, mechanical ventilation, and hemodialysis. In addition, extra precautions should be taken against opportunistic infections such as Pneumocystis carinii pneumonia.     

Churg-Strauss syndrome with alveolar hemorrhage]

A 35 year-old man was admitted to our hospital because of cough, wheezing, and paresthesia of the right upper extremity. He demonstrated marked eosinophilia, bronchial asthma, and mononeuritis multiplex. We diagnosed Churg-Strauss syndrome. Serum MPO-ANCA was elevated to 189 U/ml. Chest high-resolution computed tomography showed panlobular ground-glass attenuation in both lungs. BALF showed bloody fluid, and TBLB revealed findings consistent with eosinophilic pneumonia. Lung biopsy by VATS revealed eosinophilic pneumonia, pulmonary vasculitis, capillaritis, and hemosiderosis. The patient recovered after treatment with prednisolone and cyclophosphamide. We concluded that alveolar hemorrhage due to pulmonary capillaritis could be a complication in cases of Churg-Strauss syndrome.     

The problem of diffuse alveolar hemorrhage syndromes

No matter how little the amount of blood and no matter how infrequent the event, the presence of hemoptysis is a terrifying and infernal experience for the patient. For the physician the evaluation of pulmonary bleeding carries an urgency that requires a logical plan and prompt execution.