Salik Nazir

Statin-Associated Autoimmune Myopathy: A Systematic Review of 100 Cases

Background Statins are a group of drugs that reduce the levels of triglycerides and cholesterol in blood by inhibiting HMG-CoA reductase, an enzyme involved in rate limiting step in cholesterol synthesis. About 2–20% patients on statins develop toxic myopathies, which usually resolve on discontinuation of statin. More recently, an immune-mediated necrotizing myopathy has been found to be associated with statin use which in most cases requires treatment with immunosuppressants. Objective To perform a systematic review on published case reports and case series of statin-associated autoimmune myopathy. Methods A comprehensive search of PUBMED, EMBASE, Cochrane library and ClinicalTrials.gov databases was performed for relevant articles from inception until March 19, 2016 to identify cases of statin-associated necrotizing myopathy and characterize their symptoms, evaluation and response to treatment. Results A total of 16 articles describing 100 patients with statin-associated autoimmune myopathy were identified. The mean age of presentation was 64.72 years, and 54.44% were males. The main presenting clinical feature was proximal muscle weakness, which was symmetric in 83.33% of patients. The mean creatine kinase (CK) was 6853 IU/l. Anti-HMG-CoA reductase antibody was positive in all cases tested (n = 57/57, 100%). In patients with no anti-HMG-CoA antibody results, diagnosis was established by findings of necrotizing myopathy on biopsy. Among the 83 cases where muscle biopsy information was available, 81.48% had necrosis, while 18.51% had combination of necrosis and inflammation. Most (83.82%) patients received two or more immunosuppressants to induce remission. Ninety-one percent had resolution of symptoms after treatment. Conclusion Statin-associated necrotizing myopathy is a symmetric proximal muscle weakness associated with extreme elevations of CK. It is common in males and can occur after months of statin use. It is associated with necrosis on muscle biopsy and the presence of anti-HMG-CoA reductase antibodies. It usually requires discontinuation and immune suppression for resolution. Rechallenge with statin is unsuccessful in most cases.

Takotsubo cardiomyopathy associated with epinephrine use: A systematic review and meta-analysis

BACKGROUND Takotsubo cardiomyopathy is a syndrome of transient cardiac dysfunction that is frequently associated with sudden emotional or physical stress. Epinephrine use has been implicated in precipitating Takotsubo cardiomyopathy in multiple case reports and case series. We sought to systematically review the current English literature on this association. METHODS We searched relevant articles on Takotsubo cardiomyopathy associated with epinephrine administration and extracted data on demographic characteristics, clinical features, investigations and clinical outcomes. RESULTS We identified total of 41 cases from 36 articles. The mean age of presentation was (47.07±15.73years) with strong female preponderance (83%, P=0.0001). The most common symptom at presentation was chest pain (82%). Mean peak troponin I level was (7.12±11.22ng/ml). The most common EKG abnormality was ST elevation, seen in 40% of patients. The most common finding on echocardiography was apical hypokinesis, seen in 48.78% cases. Patients younger than 45 were less likely to have apical cardiomyopathy (n=5/20, 25%) compared to patients with age >45 (n=14/21, 66%, p value 0.001, OR 0.17). The most common route of administration of epinephrine was intravenous (65.85%). All patients except one survived with complete recovery of systolic function reported in most cases within an average of 14.7days. CONCLUSION Exposure to epinephrine in clinical practice can trigger Takotsubo cardiomyopathy, which is rapidly reversible with good prognosis in most cases. This review further supports the notion that both exogenous and endogenous catecholamines are associated with the pathogenesis of Takotsubo cardiomyopathy.

Acute myocardial infarction and antiphospholipid antibody syndrome: a systematic review.

Background Antiphospholipid antibody syndrome (APS) is a disorder associated with both arterial and venous thromboembolic disease, including acute myocardial infarction (AMI). Given that management with anticoagulants is critical and differs from usual AMI care, identification of key discriminators of patients with AMI with APS is important. Methods We performed an English-language systematic review of the literature of cases and case series of patients with AMI and APS from inception until 20 March 2016, collecting demographics, investigations, and outcomes. Results Forty cases of AMI because of APS were identified from 27 articles. Patients were younger than typical AMI patients (41.10±13.61 years) and 45% were women. STelevation myocardial infarction was the presentation in 45% (18/40) of cases. The average platelet count was 130 000±138 912 c/mm3 in the 10 cases reporting it and partial thromboplastin time was elevated in all four reporting it. Coronary arteries were described as normal or with acute thrombosis in 75%. Three died during hospitalization and six had recurrence of myocardial infarction within 3 months after admission. Conclusion APS should be considered in young patients with AMI, especially if previous unprovoked thromboses, lower platelet counts, high partial thromboplastin times, and normal coronary arteries or coronary thromboses are identified.

Omalizumab-associated eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)

Omalizumab is a humanized anti-IgE antibody that has been recommended for the management of persistent asthma associated with high serum IgE levels.1 Several cases have been reported in the literature that suggest its association with eosinophilic granulomatosis with polyangiitis (EGPA), which was previously called Churg-Strauss syndrome (CSS). EGPA is a rare necrotizing systemic vasculitis of medium and small blood vessels characterized by asthma, eosinophilia, and eosinophilic or granulomatous tissue inflammation.2 The pathogenesis of this association is poorly understood. Two mechanisms have been hypothesized. First is that omalizumab plays a direct casual effect in the development of EGPA. Second, reduction of steroid use, achieved by omalizumab treatment, could reveal EGPA that beforehand manifested itself only as asthma (forme fruste of CSS).3 The aim of the present study was to expand the understanding of this association by systematic review of all the published cases in the current literature. A systematic electronic search of MEDLINE (via PubMed), EMBASE, SCOPUS, and the Cochrane Library was performed for case reports, case series, and systematic reviews on EGPA associated with omalizumab use from inception till September 28, 2016. Inclusion criteria were age older than 18 years, use of omalizumab before the diagnosis of CSS, and diagnosis of EGPA based on the American College of Rheumatology diagnostic criteria.2 We identified 8 patients with EGPA associated with omalizumab use from 8 articles (Fig 1). The demographic variables, investigations, treatment, and outcomes were abstracted (eTable 1). The mean (SD) age at presentation was 53.5 (17.5) years with a strong male preponderance (6 men [75%]). The mean (SD) number of doses of omalizumab used before diagnosis of CSS was 21.5 (28) (range, 3e81). The mean (SD) time from omalizumab initiation to disease diagnosis was 73 (125) weeks (range, 4e352 weeks). Steroids were tapered in 3 (43%) of 7 patients before disease diagnosis (regarding denominator variability, see eTable 1). Antineutrophilic cytoplasmic antibody (ANCA) status was mentioned in only 5 patients, and all had a negative ANCA status. Omalizumab treatment was continued in of 1 (16.5%) of 6 patients after diagnosis, discontinued in 3 (50%) of 6 patients, and discontinued but restarted later in 2 (33%) of 6 patients because of worsening asthma symptoms. CSS was treated with systemic steroids in in 8 (100%) of 8 patients, cyclophosphamide plus steroids in 3 (37.5%) of 8 patients, and azathioprine plus steroids in 1 (12.5%) of 8 patients. All 8 patients achieved remission with treatment. All patients survived except one, who died because of advanced brain tumor.

Rare case of stress cardiomyopathy due to intramuscular epinephrine administration

We report a case of a 37-year-old woman who presented to our hospital with retrosternal chest pain following intramuscular administration of epinephrine due to presumed anaphylaxis. On arrival, she was found to have ST segment depression in the anterolateral leads on ECG and elevated cardiac troponins. She was diagnosed with stress cardiomyopathy based on left ventricle dysfunction and angiographically normal coronary arteries on cardiac catheterisation. To the best of our knowledge, this is the third reported case of takotsubo cardiomyopathy following appropriately dosed intramuscular administration of epinephrine for anaphylaxis. This case highlights the importance of considering stress cardiomyopathy in patients presenting with chest pain syndrome following systemic administration of epinephrine.

Aerococcus urinae, a rare cause of infective endocarditis.

We present the case of an elderly male who was initially seen in our hospital for a urinary tract infection that was treated with oral ciprofloxacin. He was admitted 2 weeks later with altered mental status and fever, and was found to have bacteraemia with Aerococcus urinae. Owing to altered mental status a brain MRI was performed which showed evidence of embolic stroke. Following this, a transesophageal echocardiogram showed severe mitral regurgitation and a vegetation >1 cm involving the mitral valve with associated destruction of posterior valve leaflets. The patient was started on antibiotics intravenous penicillin G and intravenous gentamicin for a total duration of 6 weeks. He underwent mitral valve replacement on day 4 of hospitalisation. The postoperative course was complicated by ventilator-dependent respiratory failure, requiring tracheostomy and eventual transfer to a skilled nursing facility. Unfortunately, he died after 2 weeks of stay at the facility.

Hypercalcemia associated with cosmetic injections: a systematic review

INTRODUCTION Cosmetic injections with silicone and polymethylmethacrylate are not FDA approved for augmentation of body parts such as breast, buttock or legs, but they have been widely used for decades. Cosmetic injections can cause foreign body granulomas and occasionally severe and life-threatening hypercalcemia. We aimed to systematically analyze the published literature on cosmetic injection-associated hypercalcemia. METHODS We searched relevant articles on hypercalcemia associated with various cosmetic injections and extracted relevant data on demographics, cosmetic injections used, severity of hypercalcemia, management and outcomes. RESULTS We identified 23 eligible patients from 20 articles. Mean age was 49.83 ± 14.70 years with a female preponderance (78.26% including transgender females). Silicone was most commonly used, followed by polymethylmethacrylate and paraffin oil (43.48, 30.43, and 8.70% respectively). The buttock was the most common site followed by the breast (69.57% and 39.13% respectively). Hypercalcemia developed at mean duration of 7.96 ± 7.19 years from the initial procedure. Mean ionized calcium at presentation was 2.19 ± 0.61 mmol/L and mean corrected calcium at presentation was 3.43 ± 0.31 mmol/L. 1,25-Dihydroxyvitamin D (1,25(OH)2D or calcitriol) was elevated while 25-hydroxyvitamin D (25(OH)D) and PTH were low in majority of cases. Hypercalcemia was managed conservatively with hydration, corticosteroids and bisphosphonates in majority of cases. Surgery was attempted in 2 cases but was unsuccessful. Renal failure was the most common complication (82.35% cases) and 2 patients died. CONCLUSION Hypercalcemia from cosmetic injections can be severe and life threatening and can present years after the initial procedure. Cosmetic injection-associated granuloma should be considered a cause of hypercalcemia, especially in middle-aged females presenting with non-PTH-mediated, non-malignant hypercalcemia, which is often associated with elevated calcitriol; however, it should be noted that calcitriol level may be normal as well.

There may be more than meets the eye with Clostridium perfringens bacteremia

ABSTRACT We present the case of an 89-year-old man with a 1 month history of fevers and fatigue. Blood cultures were positive for Clostridium perfringens. The patient had worsening abdominal distension in which an abdominal computed tomography scan uncovered a colonic mass, and further work-up revealed poorly differentiated adenocarcinoma. The patient was treated with antibiotics, but unfortunately, given his age and the new malignancy, he was discharged to hospice care. The association between clostridial bacteremia and colon cancer has been well described in the literature and is further discussed in this article. This case highlights the importance of recognizing possible occult malignancy in the right clinical setting in patients found to have clostridial bacteremia.

Popliteal arterial thrombosis in nephrotic syndrome: a case report

ABSTRACT Thrombosis is a frequent cause of morbidity and mortality in patients with nephrotic syndrome (NS). Though venous thromboses are common in NS, arterial thromboses are relatively rare. Commonly involved arteries include coronary, iliac, femoral, renal, cerebral, pulmonary, mesenteric, and axillary arteries, and the aorta. Arterial thromboses are associated with poor prognosis; treatment options are limited and patients may not always be amenable to treatment. We present the case of a 39-year-old female with NS who presented with thigh pain and was found to have sub-acute popliteal artery thrombosis.

Hypertriglyceridemic pancreatitis associated with confounding laboratory abnormalities

We present the case of a 36-year-old woman who presented to our hospital with epigastric abdominal pain and tenderness. Laboratory evaluation identified high lipase, normal amylase, pseudohyponatremia, and relatively falsely low triglyceride levels (initial value of 2,329 mg/dl which on repeat was found to have corrected value of >10,000 mg/dl). The overall clinical picture was consistent with acute pancreatitis due to hypertriglyceridemia. The patient was commenced on IV insulin and eventually required plasmapheresis with good clinical outcome. This case highlights the importance of being cognizant of falsely low amylase and TG levels that can be present in patients with hypertriglycereidemic pancreatitis