The spectrum of pulmonary amyloidosis.

Amyloidosis is a disease caused by misfolded proteins that deposit in the extracellular matrix as fibrils, resulting in the dysfunction of the involved organ. The lung is a common target of Amyloidosis, but pulmonary amyloidosis is uncommonly diagnosed since it is rarely symptomatic. Diagnosis of pulmonary amyloidosis is usually made in the setting of systemic amyloidosis, however in cases of localized pulmonary disease, surgical or transbronchial tissue biopsy might be indicated. Pulmonary amyloidosis can be present in a variety of discrete entities. Diffuse Alveolar septal amyloidosis is the most common type and is usually associated with systemic AL amyloidosis. Depending on the degree of the interstitial involvement, it may affect alveolar gas exchange and cause respiratory symptoms. Localized pulmonary Amyloidosis can present as Nodular, Cystic or Tracheobronchial Amyloidosis which may cause symptoms of airway obstruction and large airway stenosis. Pleural effusions, mediastinal lymphadenopathy and pulmonary hypertension has also been reported. Treatment of all types of pulmonary amyloidosis depends on the type of precursor protein, organ involvement and distribution of the disease. Most of the cases are asymptomatic and require only close monitoring. Diffuse alveolar septal amyloidosis treatment follows the treatment of underlying systemic amyloidosis. Tracheobronchial amyloidosis is usually treated with bronchoscopic interventions including debulking and stenting or with external beam radiation. Long-term prognosis of pulmonary amyloidosis usually depends on the type of lung involvement and other organ function.

Expiratory Snoring Predicts Obstructive Pulmonary Disease in Patients with Sleep-disordered Breathing.

RATIONALE: Sleep-disordered breathing and chronic obstructive pulmonary disease are two common conditions that may present concomitantly. The effects of chronic obstructive pulmonary disease on the polysomnographic manifestation of sleep-disordered breathing have not been studied. OBJECTIVES: We hypothesized that the presence of airflow obstruction could be predicted by the presence of expiratory upper airway narrowing during sleep in patients with sleep-disordered breathing. METHODS: Ninety-three patients with sleep-disordered breathing (19 men; age, 51.6 yr; body mass index, 40.1 kg/m(2); apnea-hypopnea index, 37.4 events/h) were observed. Every patient had an in-lab polysomnography study and complete pulmonary function tests. Sleep and respiratory events were scored using American Academy of Sleep Medicine recommended scoring criteria. Expiratory snoring events were identified on polysomnography using microphone sensor and/or pressure flow sensor in each patient. The FEV1/FVC ratio less than 70 was used to define the presence of airflow obstruction. MEASUREMENTS AND MAIN RESULTS: The proportion analysis demonstrated that patients with expiratory snoring have 11 times higher odds of having evidence of lower airway obstruction, defined as FEV1/FVC less than 70 (odds ratio [OR], 11.03; P < 0.001), whereas smokers have increased odds by 13 times (OR, 13.18; P < 0.001). Spearman correlation analysis showed that FEV1 was positively related to mean SaO2 (P < 0.05) and negatively related to expiratory snoring, smoking, 3% oxygen desaturation index, 2% oxygen desaturation index, and age (P < 0.05). Epworth sleepiness scale, sex, and body mass index did not have any association with FEV1. The multiple logistic regression analysis demonstrated that chronic obstructive pulmonary disease (FEV1/FVC < 70) correlated significantly with expiratory snoring and smoking (OR, 11.76; confidence interval, 3.23-42.83; and OR, 9.95; confidence interval, 2.67-37.09), respectively. The multiple linear regression analysis revealed that the linear combination of mean SaO2 and expiratory snoring (P < 0.05) predicted FEV1. However, age and 2% oxygen desaturation index did not predict FEV1. CONCLUSIONS: The presence of expiratory snoring predicts obstructive airway disorders. Patients with expiratory snoring and low mean oxygen saturation during sleep should be carefully assessed for pulmonary disorders such as asthma and chronic obstructive pulmonary disease.

Tetraplegia is associated with enhanced peripheral chemoreflex sensitivity and ventilatory long-term facilitation.

Cardiorespiratory plasticity induced by acute intermittent hypoxia (AIH) may contribute to recovery following spinal cord injury (SCI). We hypothesized that patients with cervical SCI would demonstrate higher minute ventilation (V̇e) following AIH compared with subjects with thoracic SCI and able-bodied subjects who served as controls. Twenty-four volunteers (8 with cervical SCI, 8 with thoracic SCI, and 8 able-bodied) underwent an AIH protocol during wakefulness. Each subject experienced 15 episodes of isocapnic hypoxia using mixed gases of 100% nitrogen (N2), 8% O2, and 40% CO2 to achieve oxygen saturation ≤90% followed by room air (RA). Measurements were obtained before, during, and 40 min after AIH to obtain ventilation and heart rate variability data [R-R interval (RRI) and low-frequency/high-frequency power (LF/HF)]. AIH results were compared with those of sham studies conducted in RA during the same time period. Individuals with cervical SCI had higher V̇e after AIH compared with able-bodied controls (117.9 ± 23.2% vs. 97.9 ± 11.2%, P < 0.05). RRI decreased during hypoxia in all individuals (those with cervical SCI, from 1,009.3 ± 65.0 ms to 750.2 ± 65.0 ms; those with thoracic SCI, from 945.2 ± 65.0 ms to 674.9 ± 65.0 ms; and those who were able-bodied, from 949 ± 75.0 to 682.2 ± 69.5 ms; P < 0.05). LH/HF increased during recovery in individuals with thoracic SCI and those who were able-bodied (0.54 ± 0.22 vs. 1.34 ± 0.22 and 0.67 ± 0.23 vs. 1.82 ± 0.23, respectively; P < 0.05) but remained unchanged in the group with cervical SCI. Our conclusion is that patients with cervical SCI demonstrate ventilatory long-term facilitation following AIH compared with able-bodied controls. Heart rate responses to hypoxia are acutely present in patients with cervical SCI but are absent during posthypoxic recovery.