First report of anti-TIF1γ dermatomyositis in a patient with myelodysplastic syndrome.

Inflammatory myopathies as para-neoplastic phenomena were first described by Sterz in 1916. Recently, myositis specific autoantibodies were described in cancer-associated myositis. Anti-transcription intermediary factor 1 gamma (anti-TIF1γ) antibodies have been found in both young adults affected by juvenile dermatomyositis and in elderly patients with cancer-associated myositis. In this regard, we report herein the first case of anti-TIF1γ dermatomyositis secondary to a myelodysplastic syndrome.

Circulating T cells to infliximab are detectable mainly in treated patients developing anti-drug antibodies and hypersensitivity reactions.

Antibodies recognizing infliximab (IFX) may develop in a proportion of treated patients, leading to loss of response or hypersensitivity reactions (HRs). T cell response to IFX has been poorly investigated. This paper was addressed to detect IFX-specific T cells in treated patients with inflammatory diseases developing, or not, anti-drug antibodies (ADA) and to correlate the presence of specific T cells with the clinical outcomes of the treatment. A co-culture system of IFX-loaded dendritic cells and purified autologous CD4+ T cells was used to detect memory T cells in 32 ADA+ and 39 ADA- IFX-treated patients and control groups. The cytokine profile of IFX-specific T cells was also studied in culture supernatants. IFX-specific cell proliferation was detected mainly in cells from ADA+ patients, irrespective of their different diseases. HR patients displayed higher T cell proliferation than non-responder and tolerant patients. A mixed [interferon (IFN)-γ, interleukin (IL)-13, IL-10] cytokine profile was shown in cells from ADA+ patients, while IL-10 was the most frequently detected cytokine in the supernatants of cultures from ADA- patients. Immunoglobulin (Ig)E+ ADA+ patients with previous HRs exhibited a more pronounced type 2 profile than IgE- ADA+ patients. This work provides evidence that IFX-specific circulating T cells are detectable mainly in ADA+ patients with HRs, regardless of their disease. The IFX-induced cytokine pattern partially correlates with the ADA isotype.

Chest wall kinematics and respiratory muscle action in ankylosing spondylitis patients.

No direct measurements of the pressures produced by the ribcage muscles, the diaphragm and the abdominal muscles during hyperventilation have been reported in patients with ankylosing spondylitis. Based on recent evidence indicating that abdominal muscles are important contributors to stimulation of ventilation, it was hypothesised that, in ankylosing spondylitis patients with limited ribcage expansion, a respiratory centre strategy to help the diaphragm function may involve coordinated action of this muscle with abdominal muscles. In order to validate this hypothesis, the chest wall response to a hypercapnic/hyperoxic rebreathing test was assessed in six ankylosing spondylitis patients and seven controls by combined analysis of: 1) chest wall kinematics, using optoelectronic plethysmography, this system is accurate in partitioning chest wall expansion into the contributions of the ribcage and the abdomen; and 2) respiratory muscle pressures, oesophageal, gastric and transdiaphragmatic (Pdi); the pressure/volume relaxation characteristics of both the ribcage and the abdomen allowed assessment of the peak pressure of both inspiratory and expiratory ribcage muscles, and of the abdominal muscles. During rebreathing, chest wall expansion increased to a similar extent in patients to that in controls; however, the abdominal component increased more and the ribcage component less in patients. Peak inspiratory ribcage, but not abdominal, muscle pressure was significantly lower in patients than in controls. End-inspiratory Pdi increased similarly in both groups, whereas inspiratory swings in Pdi increased significantly only in patients. No pressure or volume signals correlated with disease severity. The diaphragm and abdominal muscles help to expand the chest wall in ankylosing spondylitis patients, regardless of the severity of their disease. This finding supports the starting hypothesis that a coordinated response of respiratory muscle activity optimises the efficiency of the thoracoabdominal compartment in conditions of limited ribcage expansion.

MRI of cerebral rheumatoid pachymeningitis: report of two cases with follow-up.

We report the clinical and neuroradiological features of cerebral rheumatoid pachymeningitis with 1 year follow-up in two patients. MRI of the head enabled noninvasive diagnosis of both the meningeal abnormality and its complications, consisting of hypertensive hydrocephalus and superior sagittal sinus thrombosis, respectively. Dural sinus thrombosis, very uncommon in rheumatoid arthritis, was confirmed by phase-contrast MRA. Worsening of the pachymeningitis at follow-up was observed in both patients despite regression or stability of the clinical picture and long-term therapy.

Aortic connective tissue in atherosclerotic aorta--a biochemical study.

Biochemical analysis of the extracellular matrix of human aortas was performed on samples of ascending and descending aortas affected by atherosclerosis in comparison with a control group of nonatherosclerotic aortas. Ulcerated or heavily calcified atheromas were excised and excluded from the analysis in order to differentiate biochemical alterations leading to the formation of atheromas from those due to complications of already formed atheromas. Our results show that the development of atheromas brings about an extensive destruction of elastic fibers and muscular cells, and their place is occupied by other components of the extracellular matrix, most notably, collagen, non-uronic sugars, water, and lipids, which were found significantly increased.

Aortic connective tissue in ageing--a biochemical study.

The biochemical analysis of samples of aortic connective tissue was carried out in 22 subjects from 9 to 84 years old. Aortic samples were taken at necropsy performed after sudden or, more often, traumatic death. The results suggest that aging of the aorta is accompanied by an increase both in collagen content and in total sugar content when expressed as mg/cm2 while the elastin content, when expressed in the same way, does not undergo any variation.

Biochemical analysis of dermal connective tissue in subjects affected by primary uncomplicated varicose veins.

Biochemical analysis of dermal connective tissue was carried out in 14 subjects affected by primary uncomplicated varicose veins and 14 controls. Skin samples were taken, according to fixed criteria, from operation pieces of total mastectomy for breast cancer. The results suggest that the dermal tissue in these subjects is just thinner than that of controls, confirming previous similar clinical findings. The elective reduction of the collagen content observed, unassociated with changes of other components of the dermal connective tissue, brings evidence for a systemic biochemical defect of the extracellular matrix i.e. a collagen defect affecting the entire body structure and not only the varicose or pre-varicose veins of the lower limbs.

Connective tissue in aging lung.

Collagen, elastin and structural glycoproteins were measured in 21 intact lungs taken at postmortem examination after sudden death from subjects aged from 15 to 83. The data were expressed as milligrams per cubic centimeter of lung peripheral parenchyma inflated and fixed at the standard pressure of 25 cm H2O to exclude the pitfall of referring to dried tissue weight. The volume/weight ratio of inflated dried lung parenchyma increased significantly with aging; likewise the collagen content decreased and so did the collagen/elastin ratio, while the elastin content did not show any significant correlation with age. The present findings indicate that biochemical, morphological and functional data on the senile lung agree well.

[Incidence of the manifestations of the so-called status varicosus of Curtius in subjects with idiopathic varices of the lower extremities]. / Incidenza delle manifestazioni del cosidetto status varicosus di Curtius nei soggetti portatori di varici idiopatiche degli arti inferiori.

Two groups of subjects have been studied: the first one affected by varicose veins in lower legs, the second one as control (both groups include 138 subjects, mostly corresponding about age, sex and general health conditions). Acrocyanosis, blue sclerae, juvenile spontaneous epistaxis, hand's primary osteoarthrosis, articular hypermobility, thin skin and hernia were present more frequently in the group affected by varicose veins, the difference being statistically very significant. We suggest that mechanical revealing factors lead to the development of varicose veins in subjects who have a constitutional and probably hereditary systemic weakness of connective tissue network.

Connective tissue in varicose veins.

The collagen, elastin, total sugar, and nonscleroprotein content was evaluated in 32 samples of saphenous varicose vein and in 34 controls. A significantly lower collagen and elastin content was found in the varicose samples without correlation with the degree of pathologic broadening. Otherwise the total sugars and the soluble nonscleroproteins were found to be increased in varicose samples. The results are more significant when expressed as milligrams per surface unit of endothelium. Our data support the hypothesis that the decrease in collagen and elastin content is a primary rather than secondary change.

Collagen, elastin and sugar content in primary varicose veins.

The collagen, elastin and total sugar content was evaluated in 32 samples of saphenous varicose veins and in 34 controls. A significantly lower collagen and elastin content was found in the varicose samples. In addition, the total sugars and the soluble non-scleroproteins were found to be increased in varicose samples. The results are more significant when expressed as mg per unit surface of endothelium. Our findings support the hypothesis that the decrease in collagen and elastin content is a primary rather than a secondary change.