Conclusion-Genetic factors play a major part in the pathogenesis of subarachnoid haemorrhage, and family history of subarachnoid haemorrhage is the strongest independent risk factor for the disease. On the other hand, family history of intracerebral haematoma was not an independent risk factor for haematoma, but it might be a good predictor, which indirectly influences the pathogenesis of intracerebral haematoma via certain hereditary components such as hypertension, and even lifestyle factors such as alcohol consumption. In cerebral infarction, genetic factors play a minor part in its pathogenesis. (J Neurol Neurosurg Psychiatry 1997;62:66-70)
Idiopathic pulmonary haemosiderosis (IPH) is a rare cause of diffuse alveolar haemorrhage during childhood, and its precise pathophysiology and long‐term clinical course remain unclear. A 31‐year‐old man was diagnosed with IPH at four years of age and had recurrent episodes of haemoptysis. The patient's symptoms responded well to steroids. However, pulmonary fibrosis and the cystic region in the lung progressively worsened. At age 27, the patient developed polyarthritis with positive anti‐cyclic citrullinated peptide antibodies. The patient also developed hand synovitis, which was diagnosed with ultrasonography. These results indicate complications from rheumatoid arthritis. The patient's dyspnoea gradually worsened, and at the age of 31, he developed pneumothorax and an acute exacerbation of IPH. The clinical course from ages 4 to 31 included progressive chronic respiratory failure because of pulmonary fibrosis, acute exacerbations, complications with rheumatoid arthritis, and deliberation regarding lung transplantation. The development of rheumatoid arthritis after the onset of IPH supports the theory of an autoimmune mechanism of IPH.
Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome is a rare plasma cell disease. Patients with POEMS syndrome are considered to be at a high risk of developing pulmonary hypertension (PH). We report a 51‐year‐old woman diagnosed with PH associated with POEMS syndrome. She was started on dexamethasone and thalidomide. Although, the plasma vascular endothelial growth factor (VEGF) level decreased, systolic pulmonary artery pressure (sPAP) remained high. Auto‐peripheral blood stem cell transplantation improved the plasma VEGF and sPAP levels. Four years later, she presented with dyspnoea on exertion, and elevated plasma VEGF and sPAP levels. Subsequently, on administering sildenafil and macitentan, the plasma VEGF and PH levels improved. Pulmonary vasodilators can be considered when PH remains after treatment of POEMS syndrome.
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