History Of Connective Tissue Disease Research Articles

Predictors for pathologically confirmed aortitis after resection of the ascending aorta: A 12-year Danish nationwide population-based cross-sectional study

IntroductionAssessing the prevalence of, and predictors for, pathologically-confirmed inflammation of the aorta in Denmark, using a nationwide population-based study design.MethodsWe identified all adults with first-time surgery on the ascending aorta between January 1, 1997 and March 1, 2009 in Denmark. Presence of aortic inflammation was ascertained through linkage to a nationwide pathology registry. We used logistic regression to compute prevalence odds ratios (ORs) for sex, age at surgery, cardiovascular risk factors, cancer, connective tissue disease, and infectious diseases associated with the presence of aortitis.ResultsA total of 1,210 adults underwent resection of the ascending aorta, of who 610 (50.4%) had tissue submitted for pathological examination. Aortitis was found in 37 (6.1%) patients whose tissue was examined. Ten of the 37 patients were diagnosed with conditions known to be associated with aortitis or aortic aneurysm: five patients with temporal arteritis, one with Crohn's disease, one with rheumatoid arthritis, one with systemic lupus erythematosus, one with infectious aortitis, and one with Marfan's disease. Twenty-seven patients had idiopathic aortitis. Predictors of aortitis included history of connective tissue disease (adjusted OR 4.7, 95% confidence interval (CI) 1.6, 13.6), diabetes (OR 5.2, 95% CI 0.9, 29.7), advanced age (> 67 years OR 2.5, 95% CI 0.8, 7.6), and aortic valve pathology (OR 2.3, 95% CI 1.1, 4.9).ConclusionsAortitis was present in 6.1% of adults in Denmark who had pathological examination after resection of the ascending aorta. Predictors of inflammation included connective tissue disease, diabetes, advanced age, and aortic valve pathology.

Myxoid Change of the Myometrium and Cervical Stroma: Description of a Hitherto Unreported Non-neoplastic Phenomenon With Discussion of Myxoid Uterine Lesions

We report 3 cases of a hitherto undescribed phenomenon in women aged 46 to 49 in which there was replacement of the myometrium or cervical stroma by an accumulation of hypocellular myxoid stromal material containing bland spindle-shaped cells and small blood vessels. In all cases, this change was conspicuous, and in 2 it was multifocal and widespread, resulting in consideration of an infiltrative myxoid mesenchymal neoplasm. Immunohistochemistry revealed a characteristic immunophenotype with diffuse strong positivity with CD34 and CD10 but no immunoreactivity with the smooth muscle markers desmin, alpha-smooth muscle actin, and h-caldesmon. In comparison, 3 cases of uterine myxoid leiomyoma were positive with smooth muscle markers and negative with CD34 and CD10. We believe the lesion we describe to be an unusual pseudoneoplastic, possibly degenerative, phenomenon. The patients past medical histories were unremarkable, with no history of connective tissue disease (myometrial myxoidosis has rarely been described in association with lupus erytematosus) or Carney's syndrome. Two patients had been prescribed local or systemic progestogens, raising the possibility of an association with these compounds. Pathologists should be aware of this unusual pseudoneoplastic phenomenon to avoid a misdiagnosis of a neoplastic lesion.

Case 11-2010

A 69-year-old woman presented to this hospital because of a 2-month period of increasing apathy, confusion, and depression. She had a history of connective-tissue disease and idiopathic thrombocytopenic purpura. Ten days before admission, a magnetic resonance image of the brain was obtained, which was abnormal.

Expression of rheumatoid factor associated cross-reactive idiotopes by glandular B cells in Sjögren's syndrome

B cell expression of the germline gene-encoded, kappa IIIb-associated, rheumatoid factor (RF) cross-reactive idiotope (CRI) 17-109 and three VHI associated RF CRIs (G6, G8, H1) was investigated immunocytochemically in labial salivary glands from nine patients with primary and six with secondary Sjögren's syndrome, and in inflamed submandibular salivary glands from 10 patients with no history of connective tissue disease. Expression of CRIs by B cell infiltrates in labial glands from patients with primary and secondary Sjögren's syndrome were similar. Lymphoid infiltrates of labial glands from Sjögren's syndrome patients contained a higher proportion of kappa III+ cells reactive for the kappa IIIb-associated 17-109 idiotope (P less than 0.01) and larger G6 (P less than 0.02) and H1 (P less than 0.01) positive B cell populations than those within inflamed submandibular salivary glands. Furthermore, in labial glands there was a significant correlation between numbers of 17-109 and G6 idiotope reactive cells (r = 0.61; P less than 0.02), reflecting the known association between these H and L chain CRIs in RF IgM paraproteins. These results indicate that B cells bearing both VKIII and VHI-associated CRI are increased in the glandular infiltrates in Sjögren's syndrome and support the idea that this condition is associated with proliferation of immature B cell clones retaining germ-line V genes.

Antiendothelial cell antibody levels in patients with masked hypertension

Recent evidence demonstrate that masked hypertension (MH) is a significant predictor of cardiovascular disease, while, elevated levels of circulating antibodies against endothelial cell surface antigens (antiendothelial cell antibodies — AECA) seem to play an important role at the early stages of atherosclerosis process and of borderline hypertension as well. Aim of this study was to investigate the presence of AECA in patients (pts) with MH and to compare the AECA title among pts with MH and healthy normotensives (HN), matched for age, sex and body mass index. Methods One hundred-thirty (60 M, 70 F) healthy subjects mean age 45 ± 12 yrs who had clinic blood pressure < 140/90 mm Hg were studied. The whole study population underwent 24 hour ambulatory blood pressure monitoring (ABPM). According to the ABPM recordings, 24 individuals (8 M, 16 F) had MH (daytime systolic blood pressure ≥ 135 mm Hg or daytime diastolic blood pressure ≥ 85 mm Hg — group A) and the remainder 106 subjects (52 M, 54 F) had normal ABPM recordings, group B. IgG and IgM AECA levels were determined by ELISA method. AECA levels were expressed as mean value ± SD. None of the study population had a history of connective tissue disease or any metabolic disorder. Results Significantly increased titles of AECA class IgG were found in 8/24 pts of group A (30%) vs. 5/106 (4.6%) of group B ( p < 0,001). Significantly increased titles of AECA class IgM were also found in 6/24 pts of group A (25%) vs. 3/80 (3.8%) of group B ( p < 0,001). Conclusions Our results suggest that patients with MH have significantly higher AECA levels of both classes (IgG, IgM) compared to healthy normotensives. These findings may indicate a possible explanation of the increased cardiovascular risk in MH. The possibility that high AECA levels may be a driving mechanism for the development of MH needs further investigation.

Pterygium inversum unguis secondary to acrylate allergy

To the Editor: A 47-year-old African American woman presented with periungual dermatitis involving all 10 digits of her hands. The patient had worn acrylic nails for the past 10 years, but pruritus and an abnormal appearance of the periungual tissue had only begun to develop 6 months earlier. There was no personal or family history of connective tissue disease, and complete review of systems and physical examination revealed no evidence of connective tissue disease. Examination of the digits showed periungual hyperpigmentation with ragged cuticles on all 10 fingernails (Fig 1).

Acute High-Output Failure from an Aortoventricular Fistula Due to a Ruptured Sinus of Valsalva Aneurysm After Blunt Chest Trauma

Acute High-Output Failure from an Aortoventricular Fistula Due to a Ruptured Sinus of Valsalva Aneurysm After Blunt Chest Trauma

A Retrospective Case Review of Adverse Drug Reactions Using Intravenous Iron in Non-Hemodialysis Patients.

Background:Intravenous iron therapy is commonly used in hemodialysis patients but has not been well studied in other patient populations. Intravenous iron has many documented adverse drug reactions and the types and incidence of reactions differ based on the type of intravenous iron therapy used. In Canada, two forms of iron therapy are currently being used: Iron dextran marketed as Infufer or DexIron and iron sucrose marketed as Venofer are available. The general consensus in the literature is that the incidence of serious adverse reactions is relatively low approximately 0.6–0.7% with intravenous iron dextran. The objective of this retrospective chart review was to observe recorded adverse events of iron dextran (Infufer) and iron sucrose (Venofer) in our own non-hemodialysis patients.Methods:240 non-hemodialysis adult outpatient charts were reviewed. Iron dextran (Infufer) or iron sucrose (Venofer) infusions were recorded from July 20, 2000 to July 13, 2004. For each chart, the patient age, sex, date of birth, past medical history, medications and allergies were recorded. The type of intravenous iron, if premedication was used, and a description of any reaction if it occurred was also recorded. Each adverse reaction was graded on causality, severity, and system classification based on WHO standards done by two investigators virtually blinded to the type of iron therapy used.Results:Of the 240 patient charts reviewed, there were a total of 403 intravenous iron infusions given within the study period. The age of the patients ranged from 19 to 91 with mean age 60.3 +/− 16.3. The majority of our patients were end-stage renal disease peritoneal dialysis patients 187 (78%). 11 (5%) had a history of connective tissue disease or vasculitis, 15 (6%) had a history of asthma, and 84 (35%) used an angiotension converting enzyme inhibitor (ACEI). Only 17 patients (38 total infusions) received premedication.The total number of adverse events of all descriptions was 103 (26%) of the 403 total intravenous iron infusions. This was equally distributed to males 40 out of 156 (26%) and females 63 out of 247 (26%). Of the 365 intravenous therapies not given premedication there were 89 (24%) adverse events. The total number of "certain severe allergic" reactions (CSAs) was 25/403 (6%). In iron dextran (Infufer) a total of 77/295 (26%) ADRs were noted and CSAs of 23/295 (8%). In iron sucrose (Venofer) there was a total of 26/105 (25%) ADRs and 2/105 (2%) CSAs.Of the 295 intravenous iron dextran infusions, 209 had a test dose given. Of these 209, there were 60 ADRs - 25 during the test dose (12%) and 35 (17%) after the test dose.Conclusions:Adverse events and CSAs in our adult outpatient non-hemodialysis patients receiving intravenous iron therapy with either iron dextran (Infufer) and iron sucrose (Venofer) are much higher than previously reported in the literature. There are more ADRs and CSAs in the iron dextran group than the iron sucrose group. Premedication did not appear to reduce ADRs. Having a normal test dose did not preclude to getting ADRs afterwards.

Amyopathic dermatomyositis presenting during pregnancy

Amyopathic dermatomyositis presenting during pregnancy

Submucosal calcified nodule of the lower lip: report of a case

Background The subepidermal calcified nodule is a form of idiopathic calcinosis cutis that affects children and is occasionally present at birth. The most common locations are the extremities and the face. The oral and perioral regions are very rarely affected by calcinosis cutis of any type. Case report A 1-year-old white girl was referred to the Department of Oral Medicine and Maxillofacial Pathology of the School of Dentistry, Aristotle University, Thessaloniki, Greece, for evaluation of a white hard nodular lesion that had been present on her lower lip since birth. The patient had no history of connective tissue disease or calcium and phosphorus abnormalities. The nodule was surgically excised and was histopathologically and immunohistochemically examined. The sections stained with H&E revealed a cystic lesion filled with calcified material. A thin layer of inflammatory and epithelioid cells was arranged around the calcium deposits. Immunohistochemically these cells were CD68-positive and negative to keratins. These findings were compatible with a diagnosis of submucosal calcified nodule. Conclusion The subepidermal calcified nodule is characterized by the presence of calcified material which is usually located in the uppermost lamina propria. The calcium is present as irregular granules and globules. The exact mechanism that leads to excessive calcification is not clear.

Influence of metacarpophalangeal joint position on basal joint-loading in the thumb.

Conventional wisdom holds that hyperextension of the metacarpophalangeal joint of the thumb is secondary to degenerative subluxation of the trapeziometacarpal joint as occurs in osteoarthritis. We propose that a hypermobile metacarpophalangeal joint may have a causative role in the development of primary osteoarthritis at the base of the thumb by concentrating forces on the palmar aspect of the trapeziometacarpal joint. Twenty fresh-frozen cadaveric forearm specimens were obtained post mortem from donors with no history of connective-tissue disease. Each specimen was categorized by its passive range of metacarpophalangeal joint motion. Testing was conducted with Fuji ultra-low-pressure-sensitive film while the hand was in the lateral-pinch mode with the metacarpophalangeal joint in each of the following positions: unrestrained, pinned in neutral, pinned in 30 degrees of flexion, and pinned in maximal hyperextension. Quantitative analysis of the trapezial contact surface at each of the metacarpophalangeal joint positions was performed, and the center of pressure was determined. Each specimen was then classified according to the extent of arthritic disease (nonarthritic, moderately arthritic, or affected by end-stage arthritis). In specimens affected by end-stage osteoarthritis, the center of pressure on the trapeziometacarpal joint moved dorsally by 56.8% of the length of the trapezial surface with metacarpophalangeal joint flexions of 30 degrees (p < 0.01), whereas the corresponding values were 28.2% and 40.9% in the hyperextended and neutral metacarpophalangeal joint positions, respectively. In specimens with moderate osteoarthritis, 30 degrees of metacarpophalangeal joint flexion also produced the most dorsal trapeziometacarpal center of pressure (44.8%); however, this center of pressure was not significantly different from the centers of pressure at the other metacarpophalangeal joint positions. In nonarthritic specimens, the center of pressure was again significantly more dorsal with metacarpophalangeal joint flexion of 30 degrees than it was at the other positions (p < 0.01). Metacarpophalangeal joint flexion effectively unloaded the most palmar surfaces of the trapeziometacarpal joint regardless of the presence or severity of arthritic disease in this joint.

De novo ANCA-associated vasculitis occurring 14 years after kidney transplantation

A cadaveric kidney transplant recipient, with no history of a connective tissue disease, was admitted with malaise, arthralgias, diplopia, mild headache, and a painful left eye. The patient was on maintenance immunosuppression for 14 years with cyclosporine and methylprednisolone. Initial laboratory data indicated an elevated serum creatinine from baseline, 2+ proteinuria, and 50 to 100 red blood cells (RBCs)/high-power field (HPF) in the urine. Renal biopsy was consistent with necrotizing vasculitis involving glomerular capillaries, with crescent formation and an absence of immune complexes. Perinuclear antineutrophil cytoplasmic autoantibodies (P-ANCA) and anti-myeloperoxidase (MPO) were found to be elevated. To the best of our knowledge, this is the first reported case of an ANCA-associated small vessel vasculitis (SVV) developing in a renal transplant recipient without history of connective tissue disease.

Antinuclear antibodies using HEp‐2 cells in normal children and in children with common infections

Antinuclear antibody (ANA) immunofluorescence tests, using HEp-2 cells, were performed on 100 children without a history of connective tissue disease. Eighteen (18%) were positive at titres greater than or equal to 1:40, nine (9%) being greater than 1:160. Interlaboratory variability was demonstrated with some specimens. No association with possible intercurrent infection was found to account for positive results. Of 44 children with proven infections five (11%) were positive. Antinuclear antibody may be found in some normal children when using the sensitive HEp-2 cell substrate, and in the absence of clinical features should not necessarily suggest the presence of a connective tissue disease.