Therapeutic management of inflammatory heart diseases.

Background Systemic lupus erythematosus (SLE) is a systemic autoimmune disorder that commonly affects the heart, resulting in a 7 to 9 times greater incidence of cardiovascular disease (CVD) in SLE patients compared to healthy controls. Female patients with SLE between 35 and 44 years old have an incidence of myocardial infarction over 50 times greater than that observed in the Framingham cohort. Diffuse myocardial fibrosis can be detected non-invasively by extracellular volume (ECV) mapping based on pre- and postcontrast T1 measurements using cardiovascular magnetic resonance (CMR). We aimed to detect subclinical diffuse myocardial fibrosis in SLE using CMR T1 mapping. Methods 23 SLE patients (22 female, mean age 41 ± 9 years) and 23 matched controls (22 female, mean age 42 ± 9 years) without previously known cardiovascular disease underwent CMR at 1.5T. CMR evaluation included late gadolinium enhancement (LGE) [IV gadoterate meglumine at 0.15 mmol/kg], T1 mapping pre- and postcontrast, cine, tagging, and T2-weighted imaging. Results Regional fibrosis on LGE imaging was found in 5 SLE patients (22%) compared to none of controls. Presence of diffuse myocardial fibrosis in SLE was confirmed by significantly higher precontrast T1 values (981 ± 31 vs. 960 ± 21 ms, p = 0.010), decreased postcontrast T1 values (445 ± 31 vs. 470 ± 24 ms, p = 0.005) and expansion of ECV (31.8 ± 4.1 vs. 28.9 ± 2.0 %, p = 0.004). Diffuse myocardial fibrosis was evident in SLE regardless of the presence of any regional fibrosis. Left ventricular volumes, mass and ejection fraction were similar between SLE patients and controls. However, peak systolic circumferential strain (-17.0 ± 1.6 vs. -19.3 ± 1.1, p < 0.001) and peak diastolic strain rate (79 ± 26 vs. 119 ± 15 s-1, p < 0.001) were impaired in SLE. Presence of diastolic dysfunction is SLE was further supported by larger left atrial diameters (31 ± 5 vs. 26 ± 4 mm, p < 0.001). Abnormal myocardial systolic strain and diastolic strain rate correlated with diffuse myocardial fibrosis indices. There was no evidence of myocardial edema in SLE. Conclusions Cardiac involvement is common in SLE patients with no cardiovascular symptoms, and includes both focal and diffuse myocardial fibrosis, which is associated with impaired systolic and diastolic strain parameters. CMR is a robust non-invasive tool for the assessment of diffuse myocardial fibrosis and subclinical cardiac involvement in inflammatory heart disease.

Annals for Educators - 19 November 2019.

Introduction. Kawasaki syndrome (KS) is infrequent in children under 1 year of age; an incidence of 0.23% of KS in infants under 60 days of age has been informed. Case report. A 60 days old male was admitted with an irrelevant past history; he presented fever of 40° C of 1 week and a polymorphic and erithematous rash, as well as the other classical signs of KS. Coronary aneurysms were demonstrated with an echocardiography. He was managed with acetylsalicylic acid, a single dose of intravenous immunoglobulin (2 g/kg weight), and a second dose of immunoglobulin due to lack of response to the first one. Conclusions. KS should be considered in the differential diagnosis of infants with fever and rash, even if they are less than 60 days of age.

In this review, the cardiac lesions which develop in association with the various collagen-vascular diseases are described. In rheumatoid arthritis, the most frequent lesions are: fibrous obliterative pericarditis, with pericardial deposits of calcium, fibrin, cholesterol, and rheumatoid granulomas; granulomatous or nonspecific myocarditis; valvulitis, vasculitis, and amyloid deposits. In ankylosing spondylitis, the lesions involve mainly the valves (aortic and mitral valves) and the aorta. In systemic lupus erythematosus, the predominant cardiovascular lesions are: pericarditis, Libman-Sacks endocarditis, nonspecific myocarditis, vasculitis with fibrinoid necrosis, and acceleration of atherosclerosis. In scleroderma, the main cardiac lesion is fibrosis with only scanty inflammatory cells; pericarditis and nonbacterial thrombotic endocarditis also occur. In dermatomyositis/polymyositis, fibrous or fibrinous pericarditis can occur, as well as myocarditis with infiltrates of lymphocytes and plasma cells and with degeneration and necrosis of myocytes; valvulitis is uncommon except when the disease is related to mucinous adenocarcinoma. In polyarteritis nodosa, various stages of necrotizing vasculitis involve all layers of the arterial walls; foci of myocardial necrosis of various sizes can occur in association with these lesions; cardiac hypertrophy related to hypertension and pericarditis related to uremia, may also be found. In Wegener's granulomatosis, pericarditis, inflammatory infiltrates, necrotizing granulomas, and vasculitis have been observed in the heart.

Systemic lupus erythematosus (SLE) is a systemic inflammatory autoimmune disease with a broad spectrum of clinical manifestations. Libman-Sacks endocarditis (LSE) is due to sterile vegetations that arise in association with SLE. Nonbacterial thrombotic endocarditis, also known as marantic endocarditis, Libman-Sacks endocarditis, and verrucous endocarditis, is linked to a number of illnesses, the most prevalent of which is advanced cancer. Most often, the surfaces of mitral and aortic valves are involved. However, the involvement of the tricuspid valve is possible and is rarely described in the literature. We present a case of a 25-year-old female who presented with LSE, lupus nephritis, and pulmonary involvement secondary to SLE. On detailed exploration, she was found to have SLE with lupus nephritis and pulmonary hypertension secondary to valvular involvement. Through this case, we would like to elaborate on the course of SLE with triple valvular involvement.

Libman-Sacks endocarditis (LSE) is a form of nonbacterial thrombotic endocarditis (NBTE) observed in patients with malignancies, systemic lupus erythematosus (SLE), and antiphospholipid syndrome (APS), resulting from endothelial injury in a hypercoagulable state. The mitral and aortic valves are most commonly affected. Complications can include valvular injury, heart failure (HF), and widespread embolic events. We present a rare case of embolic stroke caused by NBTE in a 51-year-old man with no significant medical history. The patient presented to the emergency department with the sudden onset of slurred speech, numbness, and weakness in his right hand. Physical examination revealed Janeway lesions, splinter hemorrhages, and widespread livedo reticularis. Laboratory tests showed elevated anticardiolipin antibody (aCL-IgM), increased BNP and high-sensitivity troponin, thrombocytopenia (68,000/μL), and elevated homocysteine. Repeated blood cultures were negative. Computed tomography (CT) of the brain revealed chronic right posterior temporal, occipital, and left occipital infarctions. Magnetic resonance imaging (MRI) of the brain demonstrated numerous small foci of early subacute ischemia in both cerebral hemispheres and the left cerebellar hemisphere. A transthoracic echocardiogram (TTE) identified a 0.93 x 0.51 cm mobile echodensity near the right coronary cusp. Transesophageal echocardiography (TEE) confirmed verrucous and nodular vegetations with heterogeneous echodensity at the commissural border of both aortic valve leaflets, severe aortic regurgitation (AR), and reduced left ventricular ejection fraction (LVEF = 45–50%). The patient was diagnosed with an embolic stroke caused by LSE in the context of underlying APS. He was treated with low molecular weight heparin (LMWH), vancomycin, and piperacillintazobactam (Zosyn). Subsequently, he underwent mechanical aortic valve replacement followed by warfarin therapy (target INR 3-4). Intraoperative findings confirmed thickening and numerous tiny nodular vegetations on the aortic valve. This case highlights the importance of early diagnosis of embolic stroke caused by NBTE in young patients without significant medical history. Prompt and appropriate treatment, including antibiotics, anticoagulants, and surgery, is crucial to prevent serious complications.

Cutaneous vasculitides present with typical clinical signs depending on the size and distribution of the affected vessels. Since there are no large vessels in the skin, giant cell arteritis and Takayasu's arteritis only rarely lead to cutaneous symptoms. The classical systemic polyarteritis nodosa (PAN) is very rare. More frequent is cutaneous PAN presenting with atypical localized livedo racemosa and palpable subcutaneous nodules. The ANCA-associated vasculitides, which belong to the small vessel vasculitides, usually show systemic involvement and manifest on the skin with adiverse picture. Immune complex vasculitides are characterized by deposition of immunoglobulins on the walls of small vessels. The most common form is IgA vasculitis with the cardinal symptoms of palpable, round or oval and partially branched (retiform) purpura and a clear predilection for the legs. Serum disease is apríme example of systemic immune complex diseases due to large circulating immune complexes. Cryoglobulinemic vasculitis primarily involves small as well as medium sized vessels. Simultaneous involvement of vessels of different sizes is characteristic for vasculitis in systemic lupus erythematosus (SLE), Sjögren's syndrome, rheumatoid arthritis and sarcoidosis. Recurrent macular vasculitis in hypergammaglobulinemia is an episodic macular vasculitis of the small blood vessels. Bacteremic vasculitis of the small vessels (without direct microbial infection of the vessel wall) can typically also affect capillaries of dermal papillae and is usually accompanied by adisseminated intravascular coagulation (DIC). In some cases more than one distinct cutaneous vasculitis can occur in the same patient.

Libman-Sacks endocarditis (LSE), or nonbacterial thrombotic endocarditis (NBTE), is a rare condition most commonly associated with systemic lupus erythematosus (SLE), but it may also occur in patients with malignancies. NBTE is an underrecognized cause of cancer-associated embolic strokes of undetermined source. We report the case of a 68-year-old female with metastatic pancreatic adenocarcinoma who presented with acute visual disturbances and headaches. Neuroimaging revealed multiple embolic infarcts in different vascular territories. Transesophageal echocardiography (TEE) identified a mass on the posterior mitral valve, consistent with sterile thrombus formation. Laboratory and imaging studies confirmed advanced pancreatic cancer, creating a hypercoagulable state likely responsible for NBTE. The patient was managed with anticoagulation using low-molecular-weight heparin and palliative chemotherapy targeting the underlying malignancy. Despite these interventions, her clinical course progressed with recurrent embolic events and systemic decline, ultimately necessitating hospice care.This case highlights the diagnostic challenges of NBTE in malignancy, emphasizing the need to consider this condition in patients with multiple embolic strokes of varying ages. Early recognition through neuroimaging and echocardiography is essential for timely anticoagulation and multidisciplinary management. Clinicians should maintain a high index of suspicion for NBTE in patients with prothrombotic cancers, such as pancreatic adenocarcinoma, as prompt diagnosis can guide appropriate therapy and optimize quality of life.

ANCA-associated renal vasculitis

To study the frequency and disease specificity of antinucleosome antibody reactivity in diverse connective tissue diseases (CTD), and to determine factors, such as antibody subclass, that may influence the pathogenicity of these antibodies in relation to disease activity. IgG and IgM antinucleosome activities on nucleosome core particles from 496 patients with 13 different CTD and 100 patients with hepatitis C were measured by enzyme-linked immunosorbent assay (ELISA). Of the patients with CTD, 120 had systemic lupus erythematosus (SLE), 37 had scleroderma (systemic sclerosis; SSc), 20 had mixed connective tissue disease (MCTD), and 319 had other CTD, including Sjögren's syndrome, inflammatory myopathy, rheumatoid arthritis, primary antiphospholipid syndrome, Wegener's granulomatosis, Takayasu arteritis, giant cell arteritis, relapsing polychondritis, Behçet's syndrome, and sarcoidosis. Antinucleosome-positive sera were further analyzed, by isotype-specific ELISA, for antinucleosome and anti-double-stranded DNA (anti-dsDNA) IgG subclasses. SLE, SSc, and MCTD were the only 3 CTD in which antinucleosome IgG were detected (71.7%, 45.9%, and 45.0% of patients, respectively). Antinucleosomes of the IgG3 subclass were present at high levels in patients with active SLE and were virtually absent in those with SSc, MCTD, or inactive SLE, and their levels showed a positive correlation with SLE disease activity. Of note, an increase in levels of antinucleosome of the IgG3 isotype was observed during SLE flares, and this increase was found to be closely associated with active nephritis. Levels of antinucleosome of the IgG1 subclass showed a trend toward an inverse correlation with SLE disease activity. No significant fluctuation in the anti-dsDNA isotype profile was observed in relation to SLE severity or clinical signs. Our data suggest that IgG antinucleosome is a new marker that may help in the differential diagnosis of CTD; antinucleosome of the IgG3 isotype might constitute a selective biologic marker of active SLE, in particular, of lupus nephritis.

SAT0222 COOPERATION OF T FOLLICULAR HELPER CELLS AND B CELLS IN TERTIARY LYMPHOID STRUCTURES IN TAKAYASU ARTERITIS

OA18 A rare presentation of Polyarteritis Nodosa

Article1 April 1955SOME OBSERVATIONS ON TELESCOPED URINARY SEDIMENTSGEORGE E. SCHREINER, M.D.GEORGE E. SCHREINER, M.D.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-42-4-826 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptStudy of the kidney lesions of periarteritis nodosa has been carried on since the first comprehensive description of the disease in 1866.1 Renal involvement has been estimated to be as high as 87%. Many excellent reviews of the renal pathology of periarteritis have been published, and several classifications of the type and degree of renal involvement are available.2-6Similarly, a great volume of published material has considered the high incidence of renal lesions associated with disseminated lupus erythematosus.7-14Despite this vast literature, relatively little attention has been paid to the clinical urinary findings in these diseases. A disregard of this...Bibliography1. KussmaulMaier AR: Über eine bisher nicht beschreibene eigentümliche Arterienkrankung (Periarteritis nodosa, die mit Morbus Brightii und rapid fortschreitender allgemeiner Muskellähmung einhergeht), Deutsches Arch. f. klin. Med. 1: 484, 1866. Google Scholar2. HarrisLynchO'Hare AWGWJP: Periarteritis nodosa, Arch. Int. Med. 63: 1163, 1939. CrossrefGoogle Scholar3. Leishman AW: Clinical diagnosis of polyarteritis nodosa, with report of four recent cases, Journal Lancet 1: 803, 1937. CrossrefGoogle Scholar4. Grant RT: Observations on periarteritis nodosa, Clin. Sc. 4: 245, 1940. Google Scholar5. DavsonBallPiatt JJR: Kidney in periarteritis nodosa, Quart. J. Med. 17: 175, 1948. MedlineGoogle Scholar6. Zeek PM: Periarteritis nodosa and other forms of necrotizing angiitis, New England J. Med. 248: 764, 1953. CrossrefMedlineGoogle Scholar7. Goeckerman WH: Lupus erythematosus as a systemic disease, J. A. M. A. 80: 542, 1923. CrossrefGoogle Scholar8. KeithRowntree NMLG: A study of the renal complications of disseminated lupus erythematosus: report of four cases, Tr. A. Am. Physicians 37: 487, 1922. Google Scholar9. RoseGoldberg ELC: Visceral lesions of acute disseminated lupus erythematosus, M. Clin. North America 19: 333, 1935. Google Scholar10. Snapper I: Kidney trouble in acute lupus erythematosus, in Kidney in health and disease, 1935, Lea and Febiger, Philadelphia, p. 433. Google Scholar11. StickneyKeith JMNM: Renal involvement in disseminated lupus erythematosus, Arch. Int. Med. 66: 643, 1940. CrossrefGoogle Scholar12. Rich AR: Hypersensitivity in disease with especial reference to periarteritis nodosa, rheumatic fever, disseminated lupus erythematosus and rheumatoid arthritis, Harvey Lectures, 1946-1947, Science Press Printing Co., Lancaster, Pa., p. 106. Google Scholar13. GriffithVural GCIL: Acute and sub-acute disseminated lupus erythematosus, Circulation 3: 492, 1951. CrossrefMedlineGoogle Scholar14. Baggenstoss AH: Visceral lesions in disseminated lupus erythematosus, Proc. Staff Meet., Mayo Clin. 27: 412, 1952. MedlineGoogle Scholar15. Spiegel R: Clinical aspects of periarteritis nodosa, Arch. Int. Med. 58: 993, 1936. CrossrefGoogle Scholar16. Krupp MA: Urinary sediment in visceral angiitis (periarteritis nodosa, lupus erythematosus, Libman-Sacks disease), Arch. Int. Med. 71: 54, 1943. CrossrefGoogle Scholar17. Addis T: Glomerular nephritis: diagnosis and treatment, 1948, Macmillan Co., New York, p. 156. Google Scholar18. Miale JB: Characteristic urinary findings in visceral angiitis, Am. J. Clin. Path. 17: 820, 1947. CrossrefMedlineGoogle Scholar19. Cole LR: Periarteritis nodosa. Report of a case with characteristic urinary sediment, J. A. M. A. 149: 1649, 1952. CrossrefMedlineGoogle Scholar20. Schreiner GE: 3-D for diagnosis, GP 9: 70, 1951. Google Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAffiliations: Washington, D. C.*Received for publication July 3, 1954.From the Department of Medicine, Georgetown University Medical Center, Washington, D. C. PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited byDifferential Diagnosis: Lupus Nephritis and IgG4-Related Kidney DiseaseUrinalysis and the Urinary SedimentThe Urinary Sediment in Renal DiseaseThe nephrotic syndromePeriarteriitis nodosaClinical and histologic spectrum of the nephrotic syndromeThe differential diagnosis of acute and chronic glomerulonephritis 1 April 1955Volume 42, Issue 4Page: 826-838KeywordsClinical pathologyLesionsMetastasisRenal diseases ePublished: 1 December 2008 Issue Published: 1 April 1955 PDF downloadLoading ...

Disease activity in large-vessel vasculitis (LVV) is traditionally assessed by clinical and serological variables rather than vascular imaging. This study determined the effect of treatment on 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) vascular activity in relation to clinical- and serologic-based assessments. Patients with giant cell arteritis (GCA) or Takayasu arteritis (TA) were prospectively evaluated at 6-month intervals in an observational cohort. Treatment changes were made at least 3 months before the followup visit and categorized as increased, decreased, or unchanged. Imaging (FDG-PET qualitative analysis), clinical, and serologic (erythrocyte sedimentation rate, C-reactive protein) assessments were determined at each visit and compared over interval visits. Serial assessments were performed in 52 patients with LVV (GCA = 31; TA = 21) over 156 visits. Increased, decreased, or unchanged therapy was recorded for 36-, 23-, and 32-visit intervals, respectively. When treatment was increased, there was significant reduction in disease activity by imaging, clinical, and inflammatory markers (p ≤ 0.01 for each). When treatment was unchanged, all 3 assessments of disease activity remained similarly unchanged over 6-month intervals. When treatment was reduced, PET activity significantly worsened (p = 0.02) but clinical and serologic activity did not significantly change. Treatment of GCA with tocilizumab and of TA with tumor necrosis factor inhibitors resulted in significant improvement in imaging and clinical assessments of disease activity, but only rarely did the assessments both become normal. In addition to clinical and serologic assessments, vascular imaging has potential to monitor disease activity in LVV and should be tested as an outcome measure in randomized clinical trials.

Objective To investigate the clinical characteristics and prognosis of rheumatic disease-associated episcleritis and scleritis. Methods The clinical and laboratory features of 20 in-patients with episcleritis or scleritis from September 2005 to July 2016 in Peking University People's Hospital were analyzed retrospectively. The data of 11 rheumatoid arthritis (RA) patients were compared with 33 RA patients without episcleritis or scleritis of similar gender composition and age presented in the same period. T test, Mann-Whitney U test, chi-square test and rank sum test were used for statistical analysis. Results Among the 20 patients, the average age was (58±15) years old. Episcleritis and scleritis each accounted for half. The spectrum of rheumatic diseases included, in the order of disease frequency, RA, systemic lupus erythematosus (SLE), systemic vasculitis, relapsing polychondritis (RP), ankylosing spondylitis (AS) and undifferentiated connective tissue disease (UCTD). RA and SLE patients accounted for 55% and 20% cases respectively. Episcleritis and scleritis occurred significantly earlier in lupus patients than RA patients [(2.1±1.4) year vs (16.3±12.7) year, t'=3.598, P<0.01]. RA patients with episcleritis or scleritis had higher platelet (PLT) count[(330±138)]×109/L vs (234±91)×109/L, t=2.652, P<0.05), higher X-ray scores (P<0.01), more bone erosion [(90%(10/11) vs 52%(17/33), χ2=3.9, P<0.05] and frequent pulmonary interstitial involvement [46%(15/33) vs 9%(1/11), χ2=5.1, P<0.05]. Among 20 patients, 18 patients received systemic glucocorticosteroid. Patients with sclera involvement received a significantly higher dose of corticosteroid than patients without sclera involvement [(17.1±16.8) mg/d vs (2.2±4.2) mg/d, t'=2.907, P<0.01]. Patients with scleritis received a significantly higher dose of cortico- steroid than patients with episcleritis [(31±14) mg/d vs (5±6) mg/d, t=-3.959, P<0.01]. Disease-modifying anti-rheumatic diseases (DMARDs) and immunosuppressive agents prescribe dincluded methotrexate (MTX), leflunomide (LEF), hydroxychloroquine (HCQ), cyclosporine A (CsA) and cyclophosphamide (CTX). One refractory case of scleritis recovered after the administration of TNF inhibitor agent. Steroids and NSAIDs eye-drops were administered as well. Conclusion Episcleritis and scleritis are relatively rare ocular diseases, which are commonly associated with rheumatic diseases. In patients with RA, the occurrence of episcleritis or scleritis may suggest severe diseases and other co-existing extraarticular manifestations. The scleral involvement in RA patients ismore resistant to treatment compared to SLE. Consideration must be given to both rheumatic diseases and ocular disorders when dealing with the treatment strategy. Key words: Scleritis; Autoimmune diseases; Arthritis, rheumatoid; Lupus erythemat-osus, systemic