Daniel M. Lichtstein

The evolution of Lemierre syndrome: Report of 2 cases and review of the literature

Lemierre syndrome (postanginal septicemia) is caused by an acute oropharyngeal infection with secondary septic thrombophlebitis of the internal jugular vein and frequent metastatic infections. A high degree of clinical suspicion is necessary for diagnosis. Fusobacterium necrophorum is the usual etiologic agent. The disease progresses in several steps. The first stage is the primary infection, which is usually a pharyngitis (87.1% of cases). This is followed by local invasion of the lateral pharyngeal space and IJV septic thrombophlebitis (documented in 71.5% of cases), and finally, the occurrence of metastatic complications (present in 90% of cases at the time of diagnosis). A sore throat is the most common symptom during the primary infection (82.5% of cases). During invasion of the lateral pharyngeal space and IJV septic thrombophlebitis, a swollen and/or tender neck is the most common finding (52.2% of patients) and should be considered a red flag in patients with current or recent pharyngitis. The most common site of metastatic infection is the lungs (79.8% of cases). In contrast to the preantibiotic era, cavitating pneumonia and septic arthritis are now uncommon. Most patients (82.5%) had fever at some stage during the course of the disease. Gastrointestinal complaints such as abdominal pain, nausea, and vomiting were common (49.5% of cases). An elevated white blood cell count occurred in 75.2% of cases. Hyperbilirubinemia with slight elevation of liver enzyme levels occurred in one-third of patients, but frank jaundice was uncommon, in contrast to its high frequency reported in the preantibiotic era. We conclude that, most likely as a consequence of widespread antibiotic use for pharyngeal infections, the typical course of the disease has changed since Lemierre’s original description. The typical triad in our series was: pharyngitis, a tender/swollen neck, and noncavitating pulmonary infiltrates. The previous classical description of severe sepsis with cavitating pneumonia and septic arthritis was not commonly seen in our review. Mortality was low in our series (6.4%), but significant morbidity occurred, which was likely preventable by early diagnosis and treatment. The pathophysiology, natural history, diagnostic methods for internal jugular vein thrombosis, and management are discussed.

Endocarditis associated with antineutrophil cytoplasmic antibodies: a case report and review of the literature

We report a case of subacute bacterial endocarditis associated with small vessel vasculitis and a strongly positive cytoplasmic antineutrophil cytoplasmic antibody (c-ANCA) test. It is important to recognize this cause of positive c-ANCA because infectious endocarditis may closely mimic the clinical manifestations of ANCA-associated vasculitides such as Wegener granulomatosis or microscopic polyangiitis. Furthermore, ANCA-associated vasculitis may result in noninfectious endocarditis, which may be confused with bacterial endocarditis. In this paper, we review reported cases of ANCA-positive bacterial endocarditis and compare them to the reported cases of ANCA-associated idiopathic vasculitis with endocardial compromise.

Large vessel involvement in ANCA-associated vasculitides: report of a case and review of the literature.

Vasculitides are currently classified according to the size of the vessels involved and characteristic clinical and histopathologic findings. Antineutrophil cytoplasmic antibodies (ANCA) and other serologic tests have been used to further characterize small vessel vasculitides. Large vessel involvement in ANCA-associated small vessel vasculitides has been overlooked in the medical literature. Here, we report a case of fatal aortitis and aortic dissection in a patient with microscopic polyangiitis and review reported cases of large vessel involvement in ANCA-associated vasculitides since 1990. We have attempted to characterize this subgroup of patients. Large vessel disease in ANCA-associated vasculitis may present as stenosing large vessel arteritis, aneurysmal disease, aortic dissection, aortic rupture, aortic regurgitation, and death. Prominent perivascular inflammation may present as mediastinal, cervical or abdominal soft tissue masses. ANCA-associated large vessel disease should be considered in the differential diagnosis of these disorders. The epidemiologic, clinical and pathologic characteristics of these patients differ from those of the well-defined large vessel vasculitides such as giant cell (temporal) arteritis or Takayasu’s arteritis. We suggest that large vessel involvement is part of the spectrum of ANCA-associated vasculitis rather than an overlap with other large vessel vasculitides. It occurs in both myeloperoxidase- and proteinase 3-positive patients with either Wegener’s granulomatosis or microscopic polyangiitis, but has not been reported in Churg–Strauss syndrome. Large vessel vasculitis can precede small vessel vasculitis or occur in the absence of small vessel involvement. We hope this report will contribute to the ongoing development of classification systems for the vasculitic syndromes.

Septic thrombophlebitis: diagnosis and management.

Septic thrombophlebitis, as a result of invasion from adjacent nonvascular infections, includes conditions such as Lemierre syndrome (internal jugular vein septic thrombophlebitis), pylephlebitis (portal vein septic thrombophlebitis), and septic thrombophlebitis of the dural sinuses and the pelvic veins. All of these conditions are associated with a very high mortality if untreated. Appropriate antibacterial therapy dramatically improves the outcome of these infections and results in a low mortality rate, with the notable exception of septic thrombophlebitis of the durai sinuses. The endovascular nature of these infections results in secondary metastatic disease, including pneumonia, endocarditis, and arthritis due to septic embolization and/or hematogenous bacterial spread. The appropriate diagnosis and management of these infections depends on a high degree of clinical suspicion, the use of imaging studies, and early initiation of empiric antibacterial therapy. In this article, we review the diagnosis and management of septic thrombophlebitis, focusing on Lemierre syndrome, pylephlebitis, and septic thrombophlebitis of the pelvic veins.

Rhabdomyolysis associated with hyperthyroidism

Background:Nontraumatic rhabdomyolysis has been associated with alcohol and drug abuse, seizures, strenuous exercise, muscle hypoperfusion, hyperthermia, electrolyte disturbances, diabetic coma, and hypothyroidism. Hyperthyroidism can be associated with several neuromuscular manifestations, such as thyrotoxic myopathy and thyrotoxic periodic paralysis, both associated with weakness and normal creatine phosphokinase levels. There have been only three reported cases of rhabdomyolysis as a result of thyrotoxicosis. We are reporting the fourth case of such association. Case Report:The patient is a 26-year-old black woman with history of hypertension. She presented to the clinic with blurred vision, headaches, palpitations, weight loss, weakness, and persistent high blood pressure. She was found to have exophthalmus, lid lag, and a symmetric, smooth, and diffuse goiter. Ptosis and diplopia were absent; neurologic examination findings was normal. The patient had positive TPO antibodies, elevated free T4 level, and low thyroid-stimulating hormone (TSH) level. Graves disease was diagnosed and propylthiouracil was prescribed. The patient then returned to the clinic 2 weeks later with weakness and myalgias. Her physical examination findings were unchanged except for mild muscle weakness. Laboratory evaluation showed normal electrolytes, normal renal function, and negative urine drug screening. Creatine phosphokinase was 1276 U/L. Her free T4 and T3 levels were elevated and TSH level was low. The patient was treated with aggressive oral fluid resuscitation. Propylthiouracil was continued and free T4 and T3 normalized along with creatine phosphokinase with resolution of symptoms. Conclusions:Hyperthyroidism may, theoretically, cause rhabdomyolysis by means of increasing energy consumption associated with depletion of muscle energy stores and substrates. Our patient constitutes the fourth reported case of rhabdomyolysis associated with hyperthyroidism.

Stauffer's Syndrome Variant with Cholestatic Jaundice A Case Report

Cholestasis is a common feature of several malignant diseases, including pancreatic, hepatic, gallbladder, and ampullary carcinomas. It is usually secondary to main bile duct obstruction or widespread hepatic metastasis, but it can also be a paraneoplastic syndrome of other underlying malignancies. Stauffer’s syndrome is a rare paraneoplastic manifestation of renal cell carcinoma (RCC) that is characterized by elevated alkaline phosphatase, erythrocyte sedimentation rate, α-2-globulin, and γ-glutamyl transferase, thrombocytosis, prolongation of prothrombin time, and hepatosplenomegaly, in the absence of hepatic metastasis and jaundice. A rare variant of this syndrome with jaundice has recently been described in 3 cases in the literature. We report a patient who presented with abdominal pain and cholestatic jaundice in whom RCC was incidentally found during initial workup. Jaundice and liver dysfunction resolved completely after surgical resection of the tumor. This case illustrates the protean manifestations of RCC, and the importance of considering Stauffer’s syndrome and its variant in the differential diagnosis of anicteric and icteric cholestasis, which may allow early recognition and treatment of an underlying malignancy.

Interprofessional education in community-based Alzheimer’s disease diagnosis and treatment

Abstract As the population ages and understanding of Alzheimer’s disease (AD) improves, the number of older adults diagnosed and treated for AD and related dementias is projected to increase. Dementia diagnosis, treatment and patient and family education are complex processes best done through collaboration among healthcare professions. The educational program described in this article aimed to create an interprofessional team approach to the diagnosis and treatment of dementia involving medical and family nurse practitioner students. A two-group treatment/control pretest posttest design was used to measure changes in knowledge, attitudes and appreciation for an interprofessional team approach to patient care. Findings from this interprofessional program demonstrated that nurse practitioner students gained higher levels of knowledge regarding AD, and medical students gained more positive attitudes toward these patients and their caregivers. Comments from students indicated that both medical and nursing students found the experience valuable. Understanding the roles that various providers play will help healthcare professional meet the challenge of caring for the increasing number of patients with memory loss and for their families.

ANCA-associated large vessel compromise

The recent report by Carels et al. [1] adds to the increasing number of cases of clinically significant large vessel involvement in antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis. At least three additional cases have been reported after their review. Wiatr et al. [2] reported the case of a 30-year-old woman with ANCA-positive Wegener’s granulomatosis with aortic (peri)aortitis without narrowing or obliteration of the lumen. We recently reported a case of (peri)aortitis in a 50-year-old woman with microscopic polyangiitis which initially presented as a mediastinal mass and was later complicated by fatal aortic dissection and rupture [3]. Similarly, at least three cases of medium-sized vessel involvement and rupture in ANCA-associated vasculitis have recently been reported [4–6]. Based on the above-mentioned and other reported cases of ANCA-associated large vessel compromise (reviewed by Carels et al. and in Ref. [3]), we would like to emphasize a few characteristics that distinguish ANCAassociated large vessel disease from Takayasu’s arteritis. First, ANCA-associated large vessel disease has been reported mostly in non-Asian countries and often in people older than 50 years, without a female predominance. In addition, prominent perivasculitis and dissection are both important features or complications of ANCA-associated large vessel disease, which can present as perivascular soft tissue masses, aneurysms, dissection and/or rupture [3, 7]; this contrasts with the predominantly stenotic complications of Takayasu’s arteritis (without prominent perivasculitis) [8]. As pointed out by Carels et al., it is important to recognize ANCA-associated vasculitis as a cause of large vessel disease. Not only can large vessel involvement precede manifestations resulting from small vessel compromise such as pulmonary capillaritis and glomerulonephritis [1, 3, 9], but ANCA-associated large vessel disease may occur in the absence of (clinically obvious) small vessel involvement, as occurred in a case reported by Sakemi et al. [10] and in a recently reported case of a patient with isolated carotid artery involvement with prominent (peri)vasculitis and extremely high titers of PR3-positive cANCA [11]. Finally, although Carels et al. correctly considered the possible role of vasa vasoritis in ANCA-associated large vessel compromise in at least some patients, the report by Schildhaus et al. [12] describing a case of a 63-year-old man with high titers of cANCA (anti-PR3) in which patchy inflammatory infiltration by granulocytes and mononuclear cells in the aortic intima without compromise of the media or adventitia (or vasa vasoritis) deserves mention. The increasing number of reported cases of ANCAassociated large vessel involvement should lead to the recognition of large vessel (peri)vasculitis as part of the spectrum of ANCA-associated disease rather than as an ‘‘overlap’’ syndrome.

Diabetic Ketoacidosis in Patients with Type 2 Diabetes on Sodium-Glucose Cotransporter-2 Inhibitors - A Case Series

BACKGROUND Diabetic ketoacidosis (DKA) is a serious complication of diabetes seen commonly in autoimmune Type 1 diabetes mellitus (DM), however patients with Type 2 diabetes are also at risk. Diabetic ketoacidosis may be precipitated by the catabolic stress of acute illness such as trauma, surgery, or infections. Recent studies have suggested that sodium-glucose cotransporter-2 (SGLT-2) inhibitors precipitate DKA in Type 2 diabetes. We present a case series of four patients on SGLT-2 inhibitors who presented with DKA. METHODS Medical records were reviewed and patients who were admitted with diabetic ketoacidosis in the last one year at our institute were identified. The charts of such patients were reviewed and we were able to identify 4 patients who were admitted with DKA and were on SGLT-2 inhibitors at the time of admission for the management of their diabetes. RESULTS The age group of the four patients was between 45-65 years. Interestingly, all four patients were female. The admission blood glucose levels of these patients ranged from 203 to 400(mg/dl). The pH at the time of admission was in the acidotic range with anion gap ranging from 19 to 24. Two of these four patients had symptoms of a localized infection at the time of admission, which was confirmed by laboratory and radiological evaluation. Three of these patients required management in the intensive care unit. CONCLUSION Ketoacidosis is a rare but serious side effect of SGLT2 inhibitors. It is being increasingly reported as these drugs are now commonly being prescribed in the primary care setting. Awareness that DKA can occur in the setting of relative euglycemia is critical to recognize this life-threatening complication of diabetes. More research is needed to better understand the underlying pathophysiology and precipitating factors leading to ketoacidosis in SGLT-2 inhibitor treated patients.