Characteristics and Prognosis of Microscopic Polyangiitis Patients with Diffuse Alveolar Hemorrhage and Interstitial Lung Disease

Objective To evaluate the clinical characteristics and prognostic predictors of patients with diffuse alveolar hemorrhage(DAH)and/or interstitial lung disease(ILD)secondary to microscopic polyangiitis(MPA)in a Chinese general hospital.Methods We retrospectively reviewed the medical records of MPA patients admitted to internal medicine departments between the year 2002 and 2012.The patients were divided into the ILD,DAH,DAH combined with ILD(DAHILD),and no pulmonary involvement(NPI)groups according to pulmonary involvement patterns.The clinical characteristics at diagnosis were analyzed.The risk factors associated with short-term death and long-term death were identified with Logistic regression and Cox analysis.Results Of 193 newly diagnosed MPA patients,181 patients were enrolled in the research,of which 19 had DAH alone,96 had ILD alone,18 had DAH and DAH concurrently,and 48 had NPI.The median of serum creatine level in the DAH group was 449μmol/L,significantly higher than that in the ILD group(123μmol/L,Nemenyi=-35.215,P=0.045)and DAHILD group(359μmol/L,Nemenyi=-43.609,P=0.007).The median follow-up time was 67(range:1-199)months.Patients in the ILD group were older than those in the DAH group(median:69 years vs.57 years,Nemenyi=43.853,P=0.005).The patients with both DAH and ILD had combined features of the two subtypes,and the highest mortality(72.2%at the end of follow-up).The elevated white blood cell count was a risk factor for short-term death(OR=1.103,95%CI:1.008-1.207,P=0.032 for one month;OR=1.103,95%CI:1.026-1.186,P=0.008 for one year).Old age(HR=1.044,95%CI:1.023-1.066,P<0.001),cardiovascular system involvement(HR=2.093,95%CI:1.195-3.665,P=0.010),poor renal function(HR=1.001,95%CI:1.000-1.002,P=0.032)were risk factors for long-term death.Pulmonary infections(38/54)were the leading causes of death,especially for the patients with ILD.Besides,49 patients suffered from pulmonary infections in the first year after diagnosis.Conclusions MPA patients who presented with different pulmonary involvement patterns have completely different clinical features.These subtypes probably have different pathogenesis and should be studied separately.

Diffuse alveolar hemorrhage complicating dengue haemorrhagic fever in a 15-year-old boy:A case report

Rationale:Dengue fever is a viral infection that is spread through the bites of infected female Aedes mosquitos.It can cause life threatening complications,including dengue haemorrhagic fever(DHF)and dengue shock syndrome.Patient concerns:A 15-year-old male presented with fever and petechiae and later developed hemoptysis.Diagnosis:Dengue fever with DHF with diffuse alveolar hemorrhage.Interventions:Invasive ventilation with high positive end expiratory pressure,multiple transfusions of packed red blood cells,fresh frozen plasma,single donor platelets and inotropic support Outcomes:The patient was stabilized and discharged on minimal supplemental oxygen.Lessons:Diffuse alveolar hemorrhage,although very rare,should be considered in a patient with dengue who presents with hemoptysis.The treatment is directed at providing respiratory and circulatory support,and preventing the progression of microcirculation damage.

Diffuse alveolar hemorrhage with histopathologic manifestations of pulmonary capillaritis:Three case reports

BACKGROUND Diffuse alveolar hemorrhage(DAH)is a multicause pulmonary capillary hemorrhage or pulmonary vascular small vessel injury(mainly capillaries,including arteries and veins),causing pulmonary microcirculation blood to accumulate in the alveolar space.DAH is classified by the histological absence or presence of pulmonary capillaritis(PC)and is rarely reported in the literature.CASE SUMMARY This is a report of three girls aged 6-11 years with DAH and PC.Two patients had decreased hemoglobin and one had increased erythrocyte sedimentation rate.High-resolution computed tomography showed bilateral diffuse pulmonary infiltrate,and diagnosis of PC was confirmed by lung biopsy.Immunofluorescence test in one case showed granular IgG and a small amount of granular IgA deposit on the alveolar walls,and was negative in the other two cases,describing isolated pauci-immune PC.Treatment was with glucocorticoid alone or combination with immunosuppressants,and the symptoms resolved in all patients.CONCLUSION PC is classified as isolated and immune-mediated PC associated with systemic disease.It can be controlled in most children with glucocorticoid alone or combined with immunosuppressants.

Granulomatosis with polyangiitis presenting as high fever with diffuse alveolar hemorrhage and otitis media: A case report

BACKGROUND Granulomatosis with polyangiitis is a necrotizing inflammation of small andmedium-sized vessels accompanied by formation of granuloma, involvement ofprimary granulomatous upper and lower respiratory tracts, glomerulonephritis,and vasculitis of small vessels.CASE SUMMARY Herein, we described a case of a 52-year-old man admitted with pulmonarynodules and high fever. Autoantibody workup revealed that the patient waspositive for c-anti-neutrophil cytoplasmic antibodies and proteinase-3 antineutrophilcytoplasmic antibodies. Pulmonary biopsies revealed a localgranulomatous structure. The patient received therapy with methylprednisoloneand intravenous immunoglobulin, and his clinical symptoms improved.CONCLUSION Intravenous immunoglobulin may act on granulomatosis with polyangiitis similarto immunosuppressants.

Alveolar Hemorrhage and Acute Respiratory Distress Syndrome Associated with Pulmonary Cement Following Percutaneous Vertebroplasty with Polymethylmethacrylate

We report a rare complication of diffuse alveolar hemorrhage and respiratory failure following percutaneous vertebroplasty in a patient who has evidence of cement leakage. Cement injection was done two days prior to presentation and covered 2 vertebral levels for osteoporosis induced fractures.