Multiple Sclerosis with Idiopathic Dilated Cardiomyopathy: A Case Report

Multiple sclerosis and idiopathic dilated cardiomyopathy are two conditions in which an autoimmune process is implicated in the pathogenesis. There is evidence to support clustering of autoimmune diseases in patients with multiple sclerosis and their families. To our knowledge, this is the first report of idiopathic dilated cardiomyopathy occurring in a patient with multiple sclerosis.

Diagnostic performance of Antineutrophil Cytoplasmic Antibody tests for Idiopathic Vasculitides: Metaanalysis with a focus on antimyeloperoxidase antibodies

Objective. The diagnostic value of tests for antimyeloperoxidase antibodies (anti-MPO) for systemic vasculitis is less established than that for cytoplasmic antineutrophil cytoplasmic antibody (cANCA)/antiproteinase 3 antibodies (anti-PR3). Controversy exists regarding the optimal utilization of indirect immunofluorescence (IIF) ANCA testing versus antigen-specific ANCA testing. To summarize the pertinent data, we conducted a metaanalysis examining the diagnostic value of ANCA testing systems that include assays for anti-MPO. Methods. We performed a structured Medline search and reference list review. Target articles in the search strategy were those reporting the diagnostic value of immunoassays for anti-MPO for the spectrum of systemic necrotizing vasculitides that includes Wegener's granulomatosis, microscopic polyangiitis, the Churg-Strauss syndrome, and isolated pauci-immune necrotizing or crescentic glomerulonephritis, regardless of other types of ANCA tests. Inclusion criteria required specification of a consecutive or random patient selection method and the use of acceptable criteria for the diagnosis of vasculitis exclusive of ANCA test results. Weighted pooled summary estimates of sensitivity and specificity were calculated for anti-MPO alone, anti-MPO + perinuclear ANCA (pANCA), and anti-MPO/pANCA + anti-PR3/cANCA. Results. Of 457 articles reviewed, only 7 met the selection criteria. Summary estimates of sensitivity and specificity (against disease controls only) of assays for anti-MPO for the diagnosis of systemic necrotizing vasculitides were 37.1% (confidence interval 26.6% to 47.6%) and 96.3% (CI 94.1% to 98.5%), respectively. When the pANCA pattern by IIF was combined with anti-MPO testing, the specificity improved to 99.4%, with a lower sensitivity, 31.5%. The combined ANCA testing system (anti-PR3/cANCA + anti-MPO/pANCA) increased the sensitivity to 85.5% with a specificity of 98.6%. Conclusion. These results suggest that while anti-MPO is relatively specific for the diagnosis of systemic vasculitis, the combination system of immunoassays for anti-MPO and IIF for pANCA is highly specific and both tests should be used together given the high diagnostic precision required for these conditions. Because patients with ANCA associated vasculitis have either anti-MPO with pANCA or anti-PR3 with cANCA, and rarely both, a combined ANCA testing system including anti-PR3/cANCA and anti-MPO/pANCA is recommended to optimize the diagnostic performance of ANCA testing. (J Rheumatol 2001;28:1584-90)

Usefulness of myocardial tissue Doppler echocardiography to evaluate left ventricular dyssynchrony before and after biventricular pacing in patients with idiopathic dilated cardiomyopathy

Tissue Doppler imaging allows assessment of left ventricular dyssynchrony and resynchronization after biventricular pacing.

Idiopathic necrotizing dermatitis: Current management

Objectives: To identify and demonstrate necrotizing dermatitis in infancy; an uncommon, puzzling syndrome, in which anecdotal reporting and personal experience indicates that one third of cases may require skin grafting. Much informed discussion about the pathogenesis of this distressing syndrome centres on the role of spider envenomation; and in particular on the speculative role of the Australian White-tailed spider, Lampona cylindrata. Methods: We present here six cases of necrotizing dermatitis treated surgically at the Royal Children's Hospital and Mater Children's Hospital in Brisbane over the period from 1991 to 1999. Clinical history, surgical details and pathological investigations were reviewed in each case. Microbiological investigation of necrotic ulcers included standard aerobic and anaerobic culture. Result: Nocardia and Staphylococcus were cultured in two cases, but no positive bites were witnessed and no spiders were identified by either the children or their parents. All cases were treated with silver sulphadiazine creme. Two of the infants required general anaesthesia, excision debridement and split skin grafting. The White-tailed spider, Lampona cylindrata, does not occur in Queensland, but Lampona murina does; neither species has necrotizing components in its venom. Circumstantial evidence is consistent with this syndrome being due to invertebrate envenomation, possibly following arachnid bites. Conclusion: In our experience there is insufficient evidence to impute a specific genus as the cause, at this stage of scientific knowledge. If the offending creature is a spider, we calculate that the syndrome of necrotizing dermatitis occurs in less than 1 in 5000 spider bites.

Widespread sensory hypersensitivity is a feature of chronic whiplash-associated disorder but not chronic idiopathic neck pain

Objectives: To investigate sensory changes present in patients with chronic whiplash-associated disorders and chronic idiopathic neck pain using a variety of quantitative sensory tests to better understand the pain processing mechanisms underlying persistent symptoms. Methods: A case control study was used with 29 subjects with chronic whiplash-associated disorders, 20 subjects with chronic idiopathic neck pain, and 20 pain-free volunteers. Pressure pain thresholds were measured over the articular pillars of C2-C3, C5-C6, the median, radial, and ulnar nerve trunks in the arm and over a remote site, the muscle belly of tibialis anterior. Heat pain thresholds, cold pain thresholds, and von Frey hair sensibility were measured over the cervical spine, tibialis anterior, and deltoid insertion. Anxiety was measured with the Short-Form of the Spielberger State Anxiety Inventory. Results: Pressure pain thresholds were decreased over cervical spine sites in both subject groups when compared with controls (P < 0.05). In the chronic whiplash-associated disorders group, pressure pain thresholds were also decreased over the tibialis anterior, median, and radial nerve trunks (P < 0.001). Heat pain thresholds were decreased and cold pain thresholds increased at all sites (P < 0.03). No differences in heat pain thresholds or cold pain thresholds were evident in the idiopathic neck pain group at any site compared with the control group (P > 0.27). No abnormalities in von Frey hair sensibility were evident in either neck pain group (P > 0.28). Discussion: Both chronic whiplash-associated disorders and idiopathic neck pain groups were characterized by mechanical hyperalgesia over the cervical spine. Whiplash subjects showed additional widespread hypersensitivity to mechanical pressure and thermal stimuli, which was independent of state anxiety and may represent changes in central pain processing mechanisms. This may have implications for future treatment approaches.

Cytokine gene polymorphisms in idiopathic pulmonary fibrosis

Pro- and anti-fibrotic cytokine gene polymorphisms may affect expression of idiopathic pulmonary fibrosis (IPF). The aims of the present case-control study were to examine polymorphisms in the IL-6, transforming growth factor (TGF)-beta1, tumour necrosis factor (TNF)-alpha and interleukin-1 (IL-1)Ra genes in patients with IPF (n=22)-compared to healthy controls (n=140). Genotyping was performed on DNA extracted from peripheral blood lymphocytes, using polymerase chain reaction-restriction fragment length polymorphism with gene polymorphisms determined according to-published techniques. The following sites were examined: (i) IL-1Ra*1-5 (86 bp variable tandem repeat intron 2), (ii) IL-6 (-174G>C), (iii) TNF-alpha (-308G>A) and (iv) TGF-beta1 (Arg25Pro). The TNF-alpha (-308 A) allele was over-represented in the IPF (p(corr)=0.004) group compared to controls. Risk of IPF was significant for heterozygotes for: (i) the TNF-alpha (-308 A) allele (A/G) (odds ratio (OR) 2.9; 95% confidence interval (CI) 1.2-7.2; P=0.02), (ii) homozygotes (A/A) (OR 13.9; 95%CI 1.2-160; P=0.04) and (iii) carriage of the allele (A/A+A/G) (OR 4; 95%CI 1.6-10.2; P=0.003). The distribution of alleles and genotypes for IL-6, TGF-beta1 and IL-1Ra between the two groups was not significantly different. This is the third study to independently confirm that there is a significant association of the TNF-alpha (-308 A) allele with IPF. Further research is needed to assess the utility of cytokine gene polymorphisms as markers of disease-susceptibility.

Retinopathy associated with photodynamic therapy for treatment of idiopathic choroidal neovascularization

A case of retinopathy with retinal pigment epithelial alterations due to photodynamic therapy for the treatment of idiopathic choroidal neovascularization is reported. The possible mechanisms are discussed.

Ectopic calcification in the Azores - Clinical and radiological manifestations of Diffuse Idiopathic Skeletal Hypertosis and Chondrocalcinosis families

Objective. Twelve families that were multiply affected with diffuse idiopathic skeletal hyperostosis (DISH) and/or chondrocalcinosis, were identified on the island of Terceira, The Azores, potentially supporting the hypothesis that the 2 disorders share common etiopathogenic factors. The present study was undertaken to investigate this hypothesis. Methods. One hundred three individuals from 12 unrelated families were assessed. Probands were identified from patients attending the Rheumatic Diseases Clinic, Hospital de Santo Espirito, in The Azores. Family members were assessed by rheumatologists and radiologists. Radiographs of all family members were obtained, including radiographs of the dorsolumbar spine, pelvis, knees, elbows, and wrists, and all cases were screened for known features of chondrocalcinosis. Results. Ectopic calcifications were identified in 70 patients. The most frequent symptoms or findings were as follows: axial pain, elbow, knee and metacarpophalangeal (MCP) joint pain, swelling, and/or deformity, and radiographic enthesopathic changes. Elbow and MCP joint periarticular calcifications were observed in 35 and 5 patients, respectively, and chondrocalcinosis was identified in 12 patients. Fifteen patients had sacroiliac disease (ankylosis or sclerosis) on computed tomography scans. Fifty-two patients could be classified as having definite (17%), probable (26%), or possible (31%) DISH. Concomitant DISH and chondrocalcinosis was diagnosed in 12 patients. Pyrophosphate crystals were identified from knee effusions in 13 patients. The pattern of disease transmission was compatible with an autosomal-dominant monogenic disease. The mean age at which symptoms developed was 38 years. Conclusion. These families may represent a familial type of pyrophosphate arthropathy with a phenotype that includes peripheral and axial enthesopathic calcifications. The concurrence of DISH and chondrocalcinosis suggests a shared pathogenic mechanism in the 2 conditions.

Automatic measurement of vertebral rotation in idiopathic scoliosis

Study Design. Development of an automatic measurement algorithm and comparison with manual measurement methods. Objectives. To develop a new computer-based method for automatic measurement of vertebral rotation in idiopathic scoliosis from computed tomography images and to compare the automatic method with two manual measurement techniques. Summary of Background Data. Techniques have been developed for vertebral rotation measurement in idiopathic scoliosis using plain radiographs, computed tomography, or magnetic resonance images. All of these techniques require manual selection of landmark points and are therefore subject to interobserver and intraobserver error. Methods. We developed a new method for automatic measurement of vertebral rotation in idiopathic scoliosis using a symmetry ratio algorithm. The automatic method provided values comparable with Aaro and Ho's manual measurement methods for a set of 19 transverse computed tomography slices through apical vertebrae, and with Aaro's method for a set of 204 reformatted computed tomography images through vertebral endplates. Results. Confidence intervals (95%) for intraobserver and interobserver variability using manual methods were in the range 5.5 to 7.2. The mean (+/- SD) difference between automatic and manual rotation measurements for the 19 apical images was -0.5 degrees +/- 3.3 degrees for Aaro's method and 0.7 degrees +/- 3.4 degrees for Ho's method. The mean (+/- SD) difference between automatic and manual rotation measurements for the 204 endplate images was 0.25 degrees +/- 3.8 degrees. Conclusions. The symmetry ratio algorithm allows automatic measurement of vertebral rotation in idiopathic scoliosis without intraobserver or interobserver error due to landmark point selection.

Intracardiac echo guided valvuloplasty of a stenotic tricuspid prosthetic valve in a patient with idiopathic hypereosinophilic syndrome

A 52-year-old male with idiopathic hypereosinophilic syndrome (HES) was transferred to our institution following the development of acute respiratory failure and shock. He had previously undergone tricuspid valve replacement with bioprosthetic valves on two occasions: the initial surgery for severe native tricuspid valve stenosis and the redo surgery for severe prosthetic valve stenosis and regurgitation. Conventional imaging assessment using transoesophageal echocardiography was suboptimal and comprehensive assessment of prosthetic valve function was aided by the use of intracardiac echocardiography (ICE). ICE provided high quality 2D imaging of the prosthesis demonstrating thrombus-like material coating the inner surfaces of the prosthetic valve stents effectively forming a tunnel-like obstruction. Unusual hemodynamics secondary to severe tricuspid stenosis were demonstrated by CW Doppler with intermittent signal fusion resulting from blunted respiratory variation in the markedly elevated right atrial pressure relative to right ventricular pressure. Successful balloon valvuloplasty was performed with ICE proving highly valuable in guiding balloon position as well as monitoring the efficacy of the subsequent inflations.