Effect of delayed diagnosis on disease course and management of Churg–Strauss syndrome: a retrospective study

Delayed diagnosis in patients with Churg–Strauss syndrome (CSS) is largely attributed to the variable and nonspecific presentation of the disease’s initial symptoms. The aim of the study was to evaluate the effect of delayed diagnosis on the course of CSS. We conducted a retrospective study of 30 CSS patients followed up in our department. In each patient, we assessed the delay in CSS diagnosis (the time when patients already fulfilled four out of six of the American College of Rheumatology criteria and the diagnosis was not yet established), the disease activity at the time of diagnosis, and organ involvement during CSS course. A median value of 2 weeks was chosen as the cutoff point after which the diagnosis was considered as delayed. Sixteen patients were diagnosed before (group 1) and 14 patients after this cutoff point (group 2). In group 2, we found a higher Birmingham Vasculitis Activity Score at the moment of diagnosis (20.4 vs 25.1, p?<?0.05) and a more severe disease course, resulting in more frequent hospitalization rates (0.64 vs 2.26/year, p?<?0.00001), higher corticosteroids dose requirements (5.87 vs 11.57 mg/day converted to methylprednisolone, p?<?0.0001), and additional immunosuppressive therapy administration (56.2 vs 92.8 %, p?<?0.05) to maintain disease remission. All six perinuclear pattern of antineutrophil cytoplasmic antibobodies (pANCA)-positive patients (20 %) were found in group 1. Concluding, the delay in diagnosis of CSS of more than 2 weeks was found to be associated with a disease course that was more severe. The presence of the pANCA antibodies may occasionally facilitate establishment of the diagnosis.     

Pulmonary involvement in primary Sj?gren's syndrome: spectrum of pulmonary abnormalities and computed tomography findings in 60 patients.

The purpose of this study was to describe the high-resolution computed tomography (HRCT) findings of pulmonary involvement in primary Sj?gren's syndrome. The study included 60 patients who met the diagnostic criteria for primary Sj?gren's syndrome. The authors retrospectively reviewed the presence, extent, and distribution of various HRCT findings. Results showed that the most common HRCT findings were areas with ground-glass attenuation (92%), followed by subpleural small nodules (78%), non-septal linear opacity (75%), interlobular septal thickening (55%), bronchiectasis (38%), and cysts (30%).     

Two subtypes of Churg–Strauss syndrome with neuropathy: the roles of eosinophils and ANCA

The aim of this study was to clarify the differences in the pathogenesis of neuropathy between myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA)-positive and -negative patients with Churg–Strauss syndrome (CSS). Eight MPO-ANCA-positive and 14 MPO-ANCA-negative patients were included. In addition to the standard histology, nerve biopsies were examined, employing immunohistochemistry for eosinophil major basic protein and electron microscopy. The groups did not differ significantly in clinical profiles, including the peak disability score and number of blood eosinophils. In nerve biopsies, necrotizing vasculitis was found in 63% (5/8) of the ANCA-positive and 21% (3/14) of the ANCA-negative patients. Fibrinoid necrosis of vessel walls was noted in 4 ANCA-positive patients (50%), and in one ANCA-negative patient (p = 0039). In contrast, a large number of eosinophilic infiltrations in the epineurium was shown in 36% (5/14) of the ANCA-negative patients, with no eosinophilic infiltrations shown in ANCA-positive patients. In 3 ANCA-negative patients, endoneurial eosinophils were seen where focal axonal loss and capillary dilatation were occasionally noted. There may be 2 pathogenetic mechanisms of neuropathy with CSS: ANCA-related vascular fibrinoid necrosis, and a toxic eosinophilic effect on nerve fibers which is independent of ANCA. Therapy targeting activated eosinophils may be a possible treatment for intractable neuropathy of CSS.     

Clinical utility of cardiac magnetic resonance imaging in Churg–Strauss syndrome: case report and review of the literature

We describe a case of an individual with Churg–Strauss syndrome who presented with a cerebrovascular accident (CVA) secondary to left ventricular intracavitary thrombi. Noninvasive cardiovascular imaging using transthoracic echocardiography (TTE) and cardiac magnetic resonance (CMR) was used to identify the cardioembolic source of CVA. The clinical utility of CMR in the management of patients with Churg–Strauss syndrome is reviewed.     

Nephropathy,polyneuropathy, and gastroenteritis in a child with Churg–Strauss syndrome

Churg–Strauss syndrome (CSS) is a serious but rare pauci-immune vasculitis of small- and medium-sized blood vessels. It is commonly seen in association with bronchial asthma and/or allergic disorders. The syndrome is characterized by the presence of asthma, hypereosinophilia, and vasculitis in any part of the body. Vasculitis is often associated with significant distortion of normal functions. A rather severe case of CSS in an 8-year-old Nigerian girl with asthma and allergic rhinoconjunctivitis is reported. She presented with multiple morbidities, namely, vasculitic polyneuropathy and also nephritic–nephrotic syndrome that eventuated in acute renal failure after an onset of vasculitic gastroenteritis. Routine screening of all asthmatic patients for CSS is advocated.     

Churg–Strauss syndrome and congenital factor Leiden thrombophilia as nontypical causes of intracardiac thrombosis

We present and discuss the case of a 26-year-old man suffering from Churg–Strauss syndrome (CSS). In our patient the disease severely affected both the heart and the lungs. Left ventricular dysfunction and intracardiac thrombosis, probably secondary to the cardiotoxic effect of eosinophils, were revealed by echocardiography. The risk of thrombosis was additionally increased by the coexistence of the factor V Leiden gene mutation. An opportunely established diagnosis allowed institution of an appropriate pharmacotherapy and subsequently to obtain a significant abatement of the clinical symptoms as well as the laboratory manifestations of the disease.     

Postpartum Churg–Strauss syndrome with severe cardiac involvement: Description of a case and review of the literature

Churg–Strauss syndrome (CSS) is a rare small- or intermediate-vessel necrotizing vasculitis typically characterized by asthma, lung infiltrates, necrotizing granulomas, and hypereosinophilia. In this report, we describe the case of a 35-year-old woman who, during her third trimester of pregnancy, developed dyspnea and, after delivery, severe cardiac failure which required heart transplantation. Diagnosis of CSS was made after performing a myocardial biopsy. We have also undertaken a review of the English-language literature regarding previously reported cases of pregnancies in women suffering from Churg–Strauss syndrome with particular attention to those patients with cardiovascular involvement.     

Churg–Strauss syndrome complicated by central retinal artery occlusion: case report and a review of the literature

Abstract

A 68-year-old man was admitted with rapid visual loss. Churg–Strauss syndrome (CSS) was diagnosed, based upon the symptoms of asthma, eosinophilia, interstitial pneumonitis, and positive myeloperoxidase-anti neutrophil cytoplasmic antibody (MPO-ANCA). Light reflexes were absent and vision was completely lost in both eyes. Bilateral central retinal artery occlusion (CRAO) was observed by fluorescence angiography. Steroid pulse along with an anticoagulant improved the visual acuity to light perception and hand motion. CSS-associated CRAO should be considered when acute visual loss occurs.     

Churg–Strauss syndrome complicated by central retinal artery occlusion: case report and a review of the literature

A 68-year-old man was admitted with rapid visual loss. Churg–Strauss syndrome (CSS) was diagnosed, based upon the symptoms of asthma, eosinophilia, interstitial pneumonitis, and positive myeloperoxidase-anti neutrophil cytoplasmic antibody (MPO-ANCA). Light reflexes were absent and vision was completely lost in both eyes. Bilateral central retinal artery occlusion (CRAO) was observed by fluorescence angiography. Steroid pulse along with an anticoagulant improved the visual acuity to light perception and hand motion. CSS-associated CRAO should be considered when acute visual loss occurs.     

Churg–Strauss syndrome: a rare presentation with otological and pericardial manifestations: case report and review of the literature

Churg–Strauss syndrome (CSS) is a rare multisystem autoimmune disease characterized by diffuse eosinophilic infiltration and necrotizing vasculitis. There are typical manifestations of longstanding rhinosinusitis and polyposis but otological manifestations are rare and characterized by dense aural discharge, granulomatous eosinophilic infiltrate in the mastoid and middle ear with conductive hearing loss, and progressive sensory neural hearing loss—all of which are not responsive to conventional treatment. We describe the case of a 59-year-old man with a rare presentation of CSS that included chronic bilateral otitis media with hearing loss and life-threatening pericardial tamponade. Treatment with pericardiocentesis, prednisone and cyclophosphamide was beneficial and resulted in an improvement of the pericardial and otological manifestations. Early recognition and treatment of otological involvement in CSS is extremely important because of the dramatic response to corticosteroids which may prevent progression of hearing loss.     

Complete remission of coronary vasculitis in Churg–Strauss Syndrome by prednisone and cyclophosphamide

The heart is involved in up to 50% of all patients with Churg–Strauss syndrome, but vasculitis of the coronary arteries has only been rarely documented. We present a young patient with severe coronary aneurysms and stenotic lesions due to a Churg–Strauss vasculitis. Prompt therapy with prednisone and cyclophosphamide resulted in the complete resolution of all lesions.     

What is the value of high resolution electrocardiography in the identification of patients at risk?

Sudden cardiac death occurs in the Federal Republic of Germany with an incidence of 60,000 to 90,000 per year. Ambulatory ECG monitoring has demonstrated that in about 80% of such events, the heart rhythm before and during sudden cardiac death shows ventricular tachycardia leading to ventricular fibrillation and circulatory standstill. From experimental studies it is known that the substrate for precipitation of the ventricular arrhythmia is localized injury in myocardial tissue with conduction delays and conduction blocks resulting in inhomogeneous spread of the impulse between normal and injured myocardium enabling the existence of an electrical re-entry circuit. Anisotrophy, that is differing velocities of conduction parallel and perpendicular to fiber direction as well as dispersion of repolarization appears responsible for propagation of the circuit. Because of the delayed impulse spread, the late depolarization after the end of the QRS complex can be detected in the ECG as ventricular late potentials by means of high resolution systems. Ventricular late potentials may be regarded as indicative of increased electrical vulnerability of the ventricles. Prevalence of ventricular late potentials: In our own studies using the signal-averaged ECG technique, ventricular late potentials were found most frequently in patients with coronary artery disease and only in 6/100 healthy subjects, in 5/30 patients with dilated cardiomyopathy, in none of 30 patients with aortic stenosis or ten with "small vessel disease". With continuously-registered high resolution electrocardiography but not with the signal-averaged ECG, patients with dilated cardiomyopathy or QT-syndrome can be found to have labile, intermittent ventricular late potentials. In patients with coronary artery disease, the number of those in whom ventricular late potentials can only be detected with continuously-registered high resolution ECG in addition to signal-averaged technique lies between 6 and nearly 30%. With respect to frequency analysis of the ST-segment, which is based on the hypothesis that the fractionated signals in arrhythmogenic areas are of higher frequency than the normal low frequency signals of the ST-segment in normal myocardial areas, there is only limited experience and no data from larger collectives.(ABSTRACT TRUNCATED AT 400 WORDS)     

Advances in the diagnosis and treatment of Sjogren’s syndrome

Sjogren’s syndrome (SS) is a systemic autoimmune disease that primarily affects the exocrine glands, resulting in dryness of the eyes and mouth due to lymphocytic infiltration of the salivary and lacrimal glands along with arthritis, kidney, liver, and lung involvement, chronic fatigue, musculoskeletal pain, vasculitis, and so on. Considerable advance has been made for the classification and treatment of primary SS in the past few years. This article reviews the recent classification criteria for primary SS and briefly discusses the conventional and novel therapies of the disease.     

18F-fluorodeoxyglucose positron emission tomography–computed tomography for the evaluation of bone metastasis in patients with gastric cancer

BackgroundThe roles of positron emission tomography and bone scanning in identifying bone metastasis in gastric cancer are unclear.AimWe compared the usefulness of positron emission tomography–computed tomography and scanning in detecting bone metastasis in gastric cancer.MethodsData from 1485 patients diagnosed with gastric cancer who had undergone positron emission tomography–computed tomography and scanning were reviewed. Of 170 enrolled patients who were suspected of bone metastasis in either positron emission tomography or scanning, 81.2% were confirmed to have bone metastasis.ResultsThe sensitivity, specificity, and accuracy were 93.5%, 25.0%, and 80.6%, respectively, for positron emission tomography and 93.5%, 37.5%, and 82.9%, respectively, for scanning. 87.7% of patients with bone metastasis showed positive findings on two modalities. 15.0% of solitary bone metastases were positive on positron emission tomography only. Positron emission tomography was superior to scanning for the detection of synchronous bone metastasis, but the two modalities were similar for the detection of metachronous bone metastasis. The concordance rate of response assessment after treatment between two modalities was moderate.ConclusionsPositron emission tomography–computed tomography may be more effective for the diagnosis of bone metastasis in the initial staging workup. Conversely, bone scanning and positron emission tomography–computed tomography may be similarly effective for the detection of metachronous bone metastasis.     

Analysis of the functional muscle–bone unit of the forearm in patients with phenylketonuria by peripheral quantitative computed tomography

Bone disease in patients with phenylketonuria (PKU) is incompletely characterized. We therefore analyzed, in a cross-sectional study radius macroscopic bone architecture and forearm muscle size by peripheral quantitative computed tomography (pQCT) and muscle strength by hand dynamometry in a large cohort (n?=?56) of adolescent and adult patients with PKU aged 26.0?±?8.9 (range, 11.8–41.5) years. Data were compared with a reference population (n?=?700) from the DONALD study using identical methodology. We observed a significant reduction of cortical thickness (z-score ?1.01?±?0.79), Strength–Strain Index (SSI) (z-score ?0.81?±?1.03), and total bone mineral density (BMD) of the distal radius (z-score ?1.05?±?1.00). Mean muscle cross-sectional area (z-score ?0.98?±?1.19) and muscle grip force (z-score ?0.64?±?1.26) were also significantly reduced, indicating an impaired muscular system as part of the clinical phenotype of PKU. SSI positively correlated (r?=?0.53, P?<?0.001) with the corresponding muscle cross-sectional area in the reference population; however, the regression line slope in PKU patients was less steep (P?<?0.001), indicating that bone strength is not adequately adapted to muscle force. In conclusion, the radial bone in PKU patients is characterized by reduced bone strength in relation to muscular force, decreased cortical thickness, and impaired total BMD at the metaphyseal site. These alterations indicate a mixed bone defect in PKU, both of which are due to primary alterations of bone metabolism and to secondary alterations in response to neuromuscular abnormalities.     

Pulmonary vasculitis in Hughes-Stovin syndrome (HSS): a reference atlas and computed tomography pulmonary angiography guide—a report by the HSS International Study Group

Clinical Rheumatology - Hughes-Stovin syndrome (HSS) is a systemic vasculitis characterized by widespread venous/arterial thrombosis and pulmonary artery aneurysms (PAAs), which is associated with...