Subject(s)
Humans , Precancerous Conditions/pathology , Precancerous Conditions/diagnostic imaging , Respiratory Tract Neoplasms/pathology , Respiratory Tract Neoplasms/diagnostic imaging , Microscopy, Confocal/methods , Endoscopy/methods , Pulmonary Alveoli/pathology , Pulmonary Alveoli/diagnostic imaging , Prospective Studies , Reproducibility of Results , ElastinABSTRACT
Alveolar hemorrhage [AH] is a rare, but serious manifestation of SLE. It may occur early or late in disease evolution. Extrapulmonary disease may be minimal and may be masked in patients who are already receiving immunosuppressants for other symptoms of SLE. The capacity of AH to occur and recur despite ongoing immunosuppressive therapy is emphasized. Reporting our experience with alveolar hemorrhage in patients with systemic lupus erythematosus. Records of SLE patients admitted between years 2000 and 2008 were reviewed. Seven patients with SLE admitted with nine episodes of AH were found. For all study subjects, the pertinent demographic, clinical, laboratory, histologic, therapeutic and outcome data were abstracted and chest X-ray reviewed. The disease activity was assessed using SLEDAI. The seven patients were females. Their age ranged from 17 to 35 years and disease duration ranged from 4 to 48 months. AH occurred within 4 months of SLE onset in two patients. All patients presented with hemoptysis, new pulmonary infiltrates and hemoglobin drop. Glomerulonephritis was the most common extrapulmonary SLE manifestation [85%]. Initial treatment included IV methylprednisolone in all cases [100%], with cyclophosphamide in four episodes. Plasmapheresis [one session] was done in only one episode. Survival rate was 14%.Alveolar hemorrhage is a rare but lethal complication of SLE and represents a remarkable challenge. It should be diagnosed promptly with falling red cell indices and new infiltrates on chest radiograph. It occurs in vicinity of active disease. Lupus nephritis is most associated manifestation. Alveolar hemorrhages frequently recur despite ongoing immunosuppressant therapy. Early treatment with intra venous [IV] pulse methylprednisolone and IV cyclophosphamide should be instituted for a better outcome
Subject(s)
Humans , Female , Pulmonary Alveoli/diagnostic imaging , Signs and Symptoms, Respiratory , HemoptysisABSTRACT
Pulmonary alveolar proteinosis is a rare cause of respiratory distress in neonates. We present a 4-month-old infant who presented with progressive respiratory distress since birth and failure to thrive. He was initially treated as a case of diffuse alveolar disease but on open lung biopsy was diagnosed as pulmonary alveolar proteinosis. The child expired at 7 months of age.
Subject(s)
Fatal Outcome , Humans , Infant, Newborn , Male , Pulmonary Alveolar Proteinosis/complications , Pulmonary Alveolar Proteinosis/diagnosis , Pulmonary Alveolar Proteinosis/therapy , Pulmonary Alveoli/pathology , Pulmonary Alveoli/diagnostic imaging , Respiratory Distress Syndrome, Newborn/diagnosis , Respiratory Distress Syndrome, Newborn/etiology , Respiratory Distress Syndrome, Newborn/therapyABSTRACT
We report two cases of pulmonary alveolar microlithiasis (PAM) in siblings. The male child was diagnosed initially and family screening led to the diagnosis of the asymptomatic sister. The diagnosis was confirmed by high resolution computed tomography (HRCT) and bone scintigraphy which demonstrated the calcific nature of the lesions. We emphasize the importance of family screening of PAM index cases to detect the disease in the early, asymptomatic stage.