Background Anti-interferon-gamma autoantibody-associated immunodeficiency syndrome is a rare and underrecognized adult onset immunodeficiency syndrome associated with severe opportunistic infections such as disseminated nontuberculous mycobacterium. Few cases have documented a relationship with IgG4-related disease. Concomitant diagnoses of these diseases present a diagnostic and management challenge. Case presentation A 61 year old man of Southeast Asian descent with pulmonary mycobacterium avium complex infection presented to our hospital system with a new skin rash and worsening lymphadenopathy. He was eventually diagnosed with IgG4-related disease through excisional nodal biopsy. He was managed with immunosuppressive treatment with prednisone, rituximab and cyclophosphamide. He later re-presented with disseminated mycobacterium avium complex infiltration of his joints, bones and prostate. Original titers of anti-interferon-gamma autoantibodies were falsely negative due to being on immunosuppressive therapy for his IgG4-related disease. However, anti-interferon-gamma autoantibody titers were re-sent after immunosuppression was held and returned strongly positive. Conclusions This case reviews diagnostic criteria and discusses management strategies with existing challenges in treating a patient with concomitant adult onset immunodeficiency syndrome, IgG4-related disease and a disseminated mycobacterial avium complex infection.
BackgroundPosaconazole is an extended-spectrum triazole used in the treatment and prophylaxis of many fungal diseases. There have been case reports of posaconazole-induced pseudohyperaldosteronism; however, its occurrence and association with serum posaconazole drug levels need further investigation.MethodsIn this single-center retrospective observational study, we examined the occurrence of posaconazole-induced pseudohyperaldosteronism (PIPH), diagnosed either clinically or via laboratory abnormalities, and evaluated differences in serum posaconazole concentration and clinical characteristics between those with and without this syndrome.ResultsSixty-nine patients receiving posaconazole were included; of whom, 16 (23.2%) met the definition of PIPH. Patients with PIPH were significantly older (61.1 vs. 44.7 years, P = 0.007), more frequently had hypertension prior to starting posaconazole (68.8% vs. 32.1%, P = 0.009), and were more frequently prescribed posaconazole for active treatment instead of prophylaxis compared with patients without PIPH (62.5% vs. 24.5%, P = 0.005). Patients with PIPH had a significantly higher median serum posaconazole level than those without PIPH (3.0 vs. 1.2 µg/mL, P = 0.0001). There was a positive correlation between serum posaconazole level and change in systolic blood pressure (r = 0.37, P = 0.01), a negative correlation between serum posaconazole level and change in serum potassium (r = −0.39, P = 0.006), and a positive correlation between serum posaconazole level and serum 11-deoxycortisol (r = 0.69, P < 0.0001).ConclusionPosaconazole is associated with secondary hypertension and hypokalemia, consistent with pseudohyperaldosteronism, and development is associated with higher serum posaconazole concentrations, older age, and baseline hypertension. Management may include dose reduction, the addition of an aldosterone antagonist, or an alternative triazole agent. Disclosures All authors: No reported disclosures.
Background Anti-interferon-gamma autoantibody-associated immunodeficiency syndrome is a rare and underrecognized adult onset immunodeficiency syndrome associated with severe opportunistic infections such as disseminated nontuberculous mycobacterium. Few cases have documented a relationship with IgG4-related disease. Concomitant diagnoses of these diseases present a diagnostic and management challenge. Case Presentation: A 61 year old man of Southeast Asian descent with pulmonary mycobacterium avium complex infection presented to our hospital system with a new skin rash and worsening lymphadenopathy. He was eventually diagnosed with IgG4-related disease through excisional nodal biopsy. He was managed with immunosuppressive treatment with prednisone, rituximab and cyclophosphamide. He later re-presented with disseminated mycobacterium avium complex infiltration of his joints, bones and prostate. Original titers of anti-interferon-gamma autoantibodies were falsely negative due to being on immunosuppressive therapy for his IgG4-related disease. However, anti-interferon-gamma autoantibody titers were re-sent after immunosuppression was held and returned strongly positive. Conclusions This case reviews diagnostic criteria and discusses management strategies with existing challenges in treating a patient with concomitant adult onset immunodeficiency syndrome, IgG4-related disease and a disseminated mycobacterial avium complex infection.
Background Posaconazole-induced pseudohyperaldosteronism (PIPH) has been associated with elevated posaconazole serum concentrations. Clinicians are faced with the difficult task of managing patients with PIPH while not compromising the efficacy of antifungal prophylaxis or treatment. Commonly, modifications to posaconazole therapy are utilized in managing PIPH including dosage reduction of posaconazole or therapeutic switch to an alternative antifungal. Methods We retrospectively reviewed 20 consecutive adult patients diagnosed with PIPH in this case series. Patient data including blood pressure, electrolytes, endocrine laboratory values, and posaconazole serum concentrations collected before and after therapeutic intervention. Results Out of 20 patients included, 17 patients (85%) underwent therapeutic modification with posaconazole dose reduction (N=11) as the most common change. Other modifications included posaconazole discontinuation (N=3), switch to an alternative antifungal (N=2), and addition of spironolactone (N=1). Clinical improvement (a decrease in systolic blood pressure and increase in serum potassium) was observed in 9 of 17 patients (52.9%). Table 1. Management of Posaconazole-Induced Pseudohyperaldosteronism - p1 Table 1. Management of Posaconazole-Induced Pseudohyperaldosteronism - p2 Table 1. Management of Posaconazole-Induced Pseudohyperaldosteronism - p3 Conclusion We report our experience with PIPH management, for which there is currently no universally effective strategy. We suggest a stepwise approach for PIPH management, starting with posaconazole dose reduction and repeat assessment of clinical and laboratory parameters. If resolution of PIPH is not achieved, an alternative triazole antifungal or the addition of an aldosterone antagonist are additional potential interventions. Even with this approach, it is possible for PIPH to persist after therapeutic modification. Thus, early diagnosis and continuous, close monitoring of patients is warranted. Disclosures All Authors: No reported disclosures
Introduction Combined chemotherapy and radiotherapy increases long term survival in patients with nasopharyngeal carcinoma. However, radiotherapy of the carotid sinus or brain stem can evolve labile hypertension and orthostatic intolerance from chronic baroreflex failure. Diabetes would also cause this neuropathy. Management of patients with Supine hypertension-Orthostatic hypotension can be very challenging. Methods A case report was done on a 71-year-old man with metastatic nasopharyngeal carcinoma status post radiation therapy who was admitted with severe supine hypertension-orthostatic hypotension. Patient was managed with both non-pharmacological and pharmacological methods, and monitored for postural symptoms, complications of severe supine hypertension—which has been linked to left ventricular hypertrophy and kidney dysfunction, and placed on 24 hour ambulatory blood pressure monitoring to aid in management so as to prevent hypertension induced organ damage. Results This review outlines the pathophysiology of Supine hypertension-Orthostatic hypotension, treatment complications and potential management strategies recommendations for this group of patients. It revealed the benefit of having a 24 hour ambulatory blood pressure monitoring, which provides insight on the timing and magnitude of an individual’s blood pressure fluctuations throughout the day so as to further guide management. Conclusion Chronic baroreflex failure is a late sequela of neck irradiation for naso-pharyngeal carcinoma due to accelerated atherosclerosis in the region of the carotid sinus baroreceptor. Treatment goal is achieved with adequate control of pre-syncopal symptoms and prevention of long term complications. Non-pharmacological interventions remain the first line of therapy, followed by pharmacological interventions as necessary. Nonetheless, management of blood pressure in these elderly patients with baroreflex dysfunction remains challenging and should be individualized. Moving forward, a prospective study on the incidence of late onset, iatrogenic baroreflex failure as a late complication of neck irradiation and its particular relationship to carotid arterial rigidity should be conducted to increase awareness, timely diagnosis and management of the condition among physicians.
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