Rare Overlap of Granulomatosis With Polyangiitis in a Patient With Rheumatoid Arthritis.

Granulomatosis with polyangiitis (GPA) is a systemic small/medium-sized vessel vasculitis, which is a member of the family of antineutrophil cytoplasmic auto-antibody-associated vasculitides. This disorder affects multiple organs as it is a systemic disease, but overlapping with rheumatoid arthritis is extremely rare, with few cases reported in the medical literature. We report a case of a 55-year-old female with a history of rheumatoid arthritis who presented with recurrent upper/lower respiratory tract symptoms that responded poorly to antibiotics. The patient had elevated antiproteinase antibodies, ANCA IgG titer with a cytoplasmic staining pattern, proteinuria, hematuria, chest imaging showing cavitating and non-cavitating masses, and biopsies of lung and nasal tissue confirming the diagnosis of GPA. Our patient was given immunosuppressant therapy and improvement in lab work and clinical symptoms were seen throughout the course of treatment. This case report is unique as GPA usually rarely presents with rheumatoid arthritis (RA), but in this case, the patient had a history of rheumatoid arthritis with a new biopsy-proven GPA. This case report will help future physicians to better diagnose similar cases and help to facilitate clinical recognition and treatment for the same.

Endobronchial Hamartoma Presenting as Recurrent Pneumonia and Chronic Cough.

Pneumonia is an infection of the lungs that can result from various etiologies, including bronchial obstruction. It is estimated that 5.4% of community-acquired pneumonia occurs as a result of an endobronchial obstruction, classifying them as post-obstructive pneumonia. Pulmonary hamartomas are benign and exceedingly rare tumors. These hamartomas are usually asymptomatic and found incidentally on imaging, however, they can cause patients to develop post-obstructive pneumonia. We present a 40-year-old female with cough, fatigue, and recurrent right lower lobe pneumonia. Upon workup with bronchoscopy and biopsy, she was subsequently found to have an endobronchial hamartoma resulting in recurrent pneumonia in the same location. We are happy to report that the patient had a resection of the mass, as well as of the affected lung lobe, and has been pneumonia-free for five months. We hope to encourage a greater index of suspicion for endobronchial masses, including rare tumors, when a patient presents with recurrent pneumonia in the same location.

Focal Neurological Seizure due to Hyperglycemic Hyperosmolar Non-Ketotic Syndrome in Undiagnosed Diabetes Mellitus.

Hyperglycemic hyperosmolar non-ketotic syndrome (HHNS) is a life-threatening complication of type 2 diabetes mellitus with a wide range of presenting symptoms. Neurological symptoms, such as coma, can also be part of the manifestation of HHNS; however, focal seizures remain a rare but notable association.  A 85-year-old male patient with no history of diabetes presented to our emergency department complaining of a two-day history of twitching movements of his left wrist. Laboratory findings suggested HHNS and his hemoglobin A1c were found to be 10.2%. He was aggressively treated in the intensive care unit with fluids and insulin which also resolved his seizure episodes. He was ultimately discharged in stable condition without any seizure-like activity while having good glycemic control.  According to the American Diabetes Association, about 25% of all individuals 65 years and older have diabetes mellitus. With an increasing prevalence, the complications of uncontrolled diabetes are also becoming more notable. While the neurological deficits associated with HHNS are focal, the mechanism by which this occurs is still poorly understood and underreported warranting further studies.

Atypical hemolytic uremic syndrome: Laboratory characteristics, complement-amplifying conditions, renal biopsy, and genetic mutations.

Atypical hemolytic uremic syndrome (aHUS) is characterized by microangiopathic hemolytic anemia, consumptive thrombocytopenia, and widespread damage to multiple organs including the kidney. The syndrome has a high mortality necessitating the need for an early diagnosis to limit target organ damage. Because thrombotic microangiopathies present with similar clinical picture, accurate diagnosis of aHUS continues to pose a diagnostic challenge. This article focuses on the role of four distinct aspects of aHUS that assist clinicians in making an accurate diagnosis of aHUS. First, because of the lack of a single specific laboratory test for aHUS, other forms of thrombotic microangiopathies such as thrombotic thrombocytopenic purpura and Shiga toxin-associated HUS must be excluded to successfully establish the diagnosis of aHUS. Second, application of the knowledge of complement-amplifying conditions is critically important in making an accurate diagnosis. Third, when available, a renal biopsy can reveal changes consistent with thrombotic microangiopathy. Fourth, genetic mutations are increasingly clarifying the underlying complement dysfunction and gaining importance in the diagnosis and management of patients with aHUS. This review concentrates on the four aspects of aHUS and calls for heightened awareness in making an accurate diagnosis of aHUS.

Prevalence of chronic kidney disease among patients undergoing transradial percutaneous coronary interventions.

BACKGROUND: While transradial approach to conduct percutaneous coronary interventions offers multiple advantages, the procedure can cause radial artery damage and occlusion. Because radial artery is the preferred site for the creation of an arteriovenous fistula to provide dialysis, patients with chronic kidney disease are particularly dependent on radial artery for their long-term survival. METHODS: In this retrospective study, we investigated the prevalence of chronic kidney disease in patients undergoing coronary interventions via radial artery. Stage of chronic kidney disease was based on estimated glomerular filtration rate and National Kidney Foundation - Kidney Disease Outcomes Quality Initiative guidelines. RESULTS: A total of 497 patients undergoing transradial percutaneous coronary interventions were included. Over 70.4% (350/497) of the patients had chronic kidney disease. Stage II chronic kidney disease was observed in 243 (69%) patients (estimated glomerular filtration rate = 76.0 ± 8.4 mL/min). Stage III was observed in 93 (27%) patients (estimated glomerular filtration rate = 49 ± 7.5 mL/min). Stage IV chronic kidney disease was observed in 5 (1%) patients (estimated glomerular filtration rate = 25.6 ± 4.3 mL/min) and Stage V chronic kidney disease was observed in 9 (3%) patients (estimated glomerular filtration rate = 9.3 ± 3.5 mL/min). Overall, 107 of 350 patients (30%) had advanced chronic kidney disease, that is, stage III-V chronic kidney disease. Importantly, 14 of the 107 (13%) patients had either stage IV or V chronic kidney disease. CONCLUSION: This study finds that nearly one-third of the patients undergoing transradial percutaneous coronary interventions have advanced chronic kidney disease. Because many of these patients may require dialysis, the use of radial artery to conduct percutaneous coronary interventions must be carefully considered in chronic kidney disease population.

Contrast-induced nephropathy: Pathophysiology, risk factors, and prevention.

Contrast-induced acute kidney injury is a common iatrogenic complication associated with increased health resource utilization and adverse outcomes, including short- and long-term mortality and accelerated progression of preexisting renal insufficiency. The incidence of contrast-induced nephropathy (CIN) has been reported to range from 0% to 24%. This wide range reported by the studies is due to differences in definition, background risk factors, type and dose of contrast medium used, and the frequency of other coexisting potential causes of acute renal failure. CIN is usually transient, with serum creatinine levels peaking at 2-3 days after administration of contrast medium and returning to baseline within 7-10 days after administration. Multiple studies have been conducted using variety of therapeutic interventions in an attempt to prevent CIN. Of these, careful selection of patients, using newer radiocontrast agents, maintenance of hydration status, and avoiding nephrotoxic agents pre- and post-procedure are the most effective interventions to protect against CIN. This review focuses on the basic concepts of CIN and summarizes our recent understanding of its pathophysiology. In addition, this article provides practical recommendations with respect to CIN prevention and management.

Analysis of Asthma-Chronic Obstructive Pulmonary Disease Overlap Syndrome Defined on the Basis of Bronchodilator Response and Degree of Emphysema.

RATIONALE: Despite the increasing recognition of asthma-chronic obstructive pulmonary disease overlap syndrome (ACOS) as a clinical entity, it remains poorly characterized due to a lack of agreement on its definition and diagnostic criteria. OBJECTIVES: The aim of this study was to use spirometry and computed tomography (CT) to help better define ACOS as well as to classify subjects with ACOS based on Global Initiative for Chronic Obstructive Lung Disease (GOLD) letter grade. METHODS: We analyzed 10,192 subjects enrolled in the COPDGene Study. Subjects were non-Hispanic white or African American current or former smokers aged 45-80 years with at least a 10-pack-year smoking history. Subjects were categorized as having either ACOS with a bronchodilator response or chronic obstructive pulmonary disease with emphysema on the basis of spirometry, high-resolution CT, and a history of asthma or hay fever. MEASUREMENTS AND MAIN RESULTS: Subjects with ACOS were younger (60.6 vs. 65.9 years old; P < 0.0001), more likely to be African American (26.8% vs. 14.4%; P < 0.0001), had a higher body mass index (29.6 vs. 25.1 kg/m(2); P < 0.0001), and were more likely to be current smokers (50.9% vs. 20.7%; P < 0.0001). The majority of subjects with ACOS were categorized as GOLD grade B. Despite less severe spirometry and CT findings in subjects with ACOS, there was no significant difference in severe or frequent exacerbations. CONCLUSIONS: Bronchodilator responsiveness and degree of emphysema can help define ACOS. When defined on the basis of bronchodilator responsiveness and degree of emphysema, patients with ACOS represent a unique and high-risk group with distinct clinical features.