Combination targeted pulmonary hypertension therapy in the resolution of Dasatinib-associated pulmonary arterial hypertension.

Dasatinib is a small-molecule tyrosine kinase inhibitor used in the treatment of hematological malignancies. Pulmonary arterial hypertension (PAH) is a rare but known complication. The mainstay of treatment is cessation of Dasatinib, and while clinical improvement is rapid, complete hemodynamic resolution of pulmonary hypertension (PH) still remains exceedingly uncommon. We present a case of Dasatinib-induced PAH in a woman with chronic myeloid leukemia, who demonstrated rapid and complete clinical and hemodynamic resolution following treatment with combination pulmonary vasodilator therapy using an endothelin receptor antagonist and a phosphodiesterase-5 inhibitor. This case suggests there may be an association between the use of targeted PH medication in combination and the complete resolution of dasatinib-associated PAH, but further investigation is required

Re-Expansion Pulmonary Edema

Re-expansion pulmonary edema (RPE) is a rare complication of therapeutic thoracentesis. Unfortunately, there is no definitive treatment modality for RPE. The high mortality rate, reported up to 21%, presses the issue for finding adequate prevention and treatment. Here we report a new management modality that is very useful for a patient with RPE. Case Report: An 80-year-old man with a history of hypertension, atrial fibrillation, congestive heart failure with preserved ejection fraction, and sacral decubitus ulcer, who was admitted for surgical debridement of his ulcer. He developed hospital-acquired pneumonia and was resolved with antibiotics and aggressive hydration. He became hypoxic, and his chest imaging revealed bilateral pleural effusions refractory to diuresis. He underwent right-sided thoracentesis with removal of 2.5 liters of transudative fluid. He immediately improved, and his chest x-ray post-thoracentesis showed a significant reduction in the right pleural effusion. Later that night, the patient developed dyspnea and hypoxia. Lung auscultation revealed new crackles on the right side extending to the apex. Repeated chest x-ray showed diffuse right-sided infiltrates, consistent with re-expansion pulmonary edema. We started him on BiPAP ventilation as well as diuresis. Repeated imaging within five hours demonstrated a significant reduction in the pulmonary edema, and his clinical condition improved markedly. He was transitioned to supplemental oxygen via nasal cannula at 2L/min within 24 hours. Discussion: RPE occurs due to increase permeability of pulmonary capillary vessels secondary to rapid reduction in the pleural cavity pressure and sudden lung expansion. The severity of the intra-pleural negative pressure is thought to contribute to the risk of developing RPE. A recent study has proved a correlation between the amount of volume removed from the pleural cavity and the chance of developing RPE. Treatment for RPE is supportive, with oxygen supplementation and diuresis. However, we found dramatic clinical and radiological changes after applying BiPAP and thereby increasing the intra-pleural pressure. Conclusion: Clinicians should be encouraged to place patients who develop RPE on BiPAP for six to twelve hours to prevent worsening of pulmonary edema. As presented in our case, this management modality had desirable outcomes in as little as five hours. Further studies require to assess the effectiveness of BiPAP to decrease the progression and mortality of RPE

Respiratory rate variability in sleeping adults without obstructive sleep apnea

Characterizing respiratory rate variability (RRV) in humans during sleep is challenging, since it requires the analysis of respiratory signals over a period of several hours. These signals are easily distorted by movement and volitional inputs. We applied the method of spectral analysis to the nasal pressure transducer signal in 38 adults with no obstructive sleep apnea, defined by an apnea‐hypopnea index \u3c5, who underwent all‐night polysomnography (PSG). Our aim was to detect and quantitate RRV during the various sleep stages, including wakefulness. The nasal pressure transducer signal was acquired at 100 Hz and consecutive frequency spectra were generated for the length of the PSG with the Fast Fourier Transform. For each spectrum, we computed the amplitude ratio of the first harmonic peak to the zero frequency peak (H1/DC), and defined as RRV as (100 − H1/DC) %. RRV was greater during wakefulness compared to any sleep stage, including rapid‐eye‐movement. Furthermore, RRV correlated with the depth of sleep, being lowest during N3. Patients spent most their sleep time supine, but we found no correlation between RRV and body position. There was a correlation between respiratory rate and sleep stage, being greater in wakefulness than in any sleep stage. We conclude that RRV varies according to sleep stage. Moreover, spectral analysis of nasal pressure signal appears to provide a valid measure of RRV during sleep. It remains to be seen if the method can differentiate normal from pathological sleep patterns

Contemporary Pharmacotherapeutic Approach in Pulmonary Arterial Hypertension

© 2020 Elsevier Inc. Since the 1973 World Symposium on Pulmonary Hypertension, advancements in the understanding of pathophysiology and pathobiology have led to a myriad of pharmacotherapies for the disease. This article journeys through the development of therapeutic approaches for pulmonary arterial hypertension

Metastatic Breast Cancer and Granulomatosis with Polyangiitis

Introduction: Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis, which includes granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA), are life-threatening vasculitis. Several studies reported that up to 8% of patients with a malignant disease had ANCA-associated vasculitis. Sixty three percent of malignancies associated with vasculitides, were hematologic in origin. In this report, we describe a rare case of a recurrent breast cancer and GPA in the same lung lesion. Case Report: A 66-year-old woman with a past medical history of scleroderma, inflammatory lung disease, and remote bilateral breast cancer status post bilateral mastectomy and chemotherapy was admitted for lung biopsy. The patient developed flu-like symptoms and was started on oral antibiotics after she was seen in an urgent care clinic. Despite this, she developed worsening dyspnea and hemoptysis. Her outpatient CT chest showed multiple lung cavities with bilateral fibrotic changes. Her initial blood test was positive for anti-neutrophil cytoplasmic antibodies (ANCA), anti-cyclic citrullinated peptide antibodies (CCP), and rheumatoid factor (RF). Because of this, she was admitted for a lung biopsy as a workup for vasculitis disease. Initially, the patient was isolated in a room with negative pressure to rule out tuberculosis (TB). Her QuantiFERON TB test and acid fast bacilli smears were negative. Later, a thoracic surgery team was consulted to perform lung biopsy through video-assisted thoracoscopic surgery (VATS). Her lung biopsy revealed metastatic breast cancer that was positive for estrogen receptor, and a necrotizing granulomatous inflammation that was consistent with granulomatosis with polyangiitis (GPA) formerly known as Wegener \u27s disease. The oncology team recommended to start her on anastrozole as a treatment for breast cancer. The rheumatology team recommended a loading dose of intravascular methylprednisone and then a maintenance dose of oral prednisone. A permacath was placed in order to start outpatient rituximab infusion as a treatment for vasculitis. In addition, she was started on trimethoprim-sulfamethoxazole for pneumocystis pneumonia prophylaxis and advised to continued her home dose of mycophenolate mofetile for treatment of scleroderma. Discussion: There are two forms of vasculitis: primary and secondary. Secondary vasculitis has been linked to several processes, such as medications side effect, allergic reaction, rheumatologic and neoplastic disease. A close relationship between the diagnosis of malignancy and onset of vasculitis has been reported in a number of patients. Most vasculitides were cutaneous leukocytoclastic (45%) and polyarteritis nodosa (36%). The exact pathogenesis of malignancy-associated vasculitis is unclear. However, we hypothesize that the inflammatory responses provoked by the underlying neoplasm might contribute to the pathogenesis. To the best of our knowledge, this is the first case report of recurrent breast cancer and development of GPA that may be representative of an association between the two condition. Clinicians must be aware of associations between various medical conditions as it most certainly changes the management. While we do not dispute that, our case may be just a co incidence of two medical conditions at once, we believe a very low incidence of GPA deserve a second look in finding an association with other medical conditions should one be present