Articles: amyloidosis.
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Amyloidosis is a rare disease characterized by the misfolding of autologous proteins and extracellular deposition of fibrils, which can involve 1 or more vital organs in the body. Nodular pulmonary amyloidosis with extensive pulmonary cysts is even less common. This study discusses the diagnosis and treatment of a case of pulmonary nodular amyloidosis with extensive pulmonary cysts and calcification in the middle of the cysts on chest computed tomography, and reviews the related literature. We hope that this rare case will raise awareness of this disease among clinicians. ⋯ For patients with multiple pulmonary nodules combined with extensive pulmonary cysts, we also need to be alert to the possibility of pulmonary nodule amyloidosis. Secondly, when the main lesion is located outside the bronchial cavity near the hilum of the lung, the method of pathological tissue biopsy should also consider endobronchial ultrasound-guided tunnel biopsy.
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The current treatment paradigm of AL amyloidosis lacks effective fibril-directed therapies. Doxycycline has been shown to have anti-fibril properties in preclinical models. In 2012, we reported that posttransplant prophylaxis with doxycycline was associated with improved survival compared to penicillin in patients with haematologic response. We provide here updated results after long-term follow up. ⋯ After long-term follow-up, there is no clear evidence to support benefit of doxycycline in the post-transplant setting.
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Very small case series of patients with apolipoprotein A1 (ApoA1) amyloidosis are available. ⋯ Subjects with the APOA1 Leu75Pro variant displayed minor echocardiographic signs of cardiac involvement, but 14% met echocardiographic criteria for CA. Subjects with suspected CA had a worse outcome.