Complication Of Connective Tissue Diseases Research Articles

Hypertension artérielle pulmonaire associée aux connectivites

Purpose. – Pulmonary hypertension is a rare but well-known life-threatening complication of connective tissue diseases. The aim of this article is to analyse the available literature and to report the experience of a pulmonary vascular diseases centre about this complication. Current knowledge and key points. – Scleroderma and its limited variant, the CREST syndrome (calcification, Raynaud phenomenon, esophageal dysmotility, sclerodactily, telangiectasia), is the most common connective tissue disease affected by pulmonary hypertension. Dyspnea is the main symptom and is frequently severe. Echocardiography is an excellent exam to detect pulmonary hypertension. However, right heart catheterization is necessary to confirm the diagnosis of pulmonary hypertension and to test vasoreactivity with a potent vasodilator such as nitric oxide. Pulmonary hypertension is less severe in patients with connective tissue diseases perhaps because of an earlier diagnosis. A significantly lower proportion of patients presents an acute vasodilator response, suggesting an early constitution of irreversible pulmonary vascular lesions. Continuous intravenous epoprostenol therapy seems to be less effective as compared with patients with primitive pulmonary hypertension and does not improve survival. So, we observed dramatic improvement in rare cases after immunosuppressive therapy. Future prospects and projects. – New treatments with oral, subcutaneous or inhaled stable prostacyclin analogs or with an endothelin receptor antagonist are currently being evaluated. The role of immunosuppressive therapy has to be defined.

Neuromuscular complications of connective tissue diseases.

The connective tissue diseases, such as rheumatoid arthritis, Sjögren's syndrome, systemic lupus erythematosus, systemic sclerosis, and vasculitis, may cause various disorders of the peripheral nervous system. In this review, the clinical effects of the connective tissues diseases on nerve and muscle are examined with particular attention to mononeuritis multiplex, distal symmetric neuropathy, fulminant motor neuropathy, compression neuropathy, sensory neuronopathy, and trigeminal sensory neuropathy.

NGF, a useful tool in the treatment of chronic vasculitic ulcers in rheumatoid arthritis

Vasculitic necrosis and ulceration of the skin are frequent complications of connective tissue diseases and are very difficult to heal. We treated chronic vasculitic leg ulcers in rheumatoid arthritis and systemic sclerosis by topical application of nerve growth factor (NGF). In all patients with rheumatoid arthritis, NGF led to rapid healing, whereas less striking results were obtained in patients with systemic sclerosis. The efficacy of NGF could be due to its promoting activity on keratinocytes proliferation and vascular neoangiogenesis. We suggest that topical application of NGF could represent a powerful pharmacological tool for the treatment of vasculitic ulcers.

Autoantigen Ku in the brain. Developmentally regulated expression and subcellular localization.

A double-stranded DNA end-binding factor with high levels of expression in brain and testis of adult mice was identified as the Ku protein, earlier described as an autoantigen in connective tissue diseases and found to be essential for recombination of the immunoglobulin genes and DNA repair. High Ku levels were found in the cerebellum and pituitary gland, lower levels in the hippocampus, hypothalamus and white matter structures. Ku levels were much higher in embryonic rat brain than in the adult brain, suggesting a role of the Ku protein in brain development. In embryonic rat brain, Ku was associated with cell nuclei, but was predominantly located in the cytosol in the adult rat cerebellum and hippocampus. The abundant expression of Ku in the brain suggests the involvement of Ku autoantibodies in the pathogenesis of neuropsychiatric complications in connective tissue diseases.

When the lungs are involved by connective tissue disease.

Pulmonary involvement by a connective tissue disease can result in clinically important complications. Pathogenic mechanisms vary from granulomatous reaction and interstitial inflammation to primary vasculitis and immune complex-mediated disease. Understanding the pulmonary complications of connective tissue diseases is challenging in that several distinct patterns of involvement are associated with the same disease but the same lung abnormalities are found with several different diseases. Early recognition and treatment of pulmonary involvement may offer the patient a better chance of recovery from serious conditions that often carry a grim prognosis if undetected.

Natural cell mediated cytotoxicity (NCMC) in Sjögren's syndrome and rheumatoid arthritis

The natural cell mediated cytotoxicities (NCMC) were measured in 4 patients with Sjogren's syndrome (SjS) without complication of connective tissue disease (CTD), 4 patients with SjS associated with CTD other than rheumatoid arthritis (RA), 11 patients with SjS associated with RA, and 29 patients with RA without SjS. All 19 patients with SjS showed significantly suppressed NCMC. In the patients with RA without SjS, NCMC of 21 female cases were rather enhanced when compared with controls, while 8 male patients did not differ from controls. The reduced NCMC in SjS did not seem to correlate with the presence of antilymphocyte antibody (ALA). It is of interest that more accelerated NCMC were demonstrated in the patients with RA receiving gold therapy.

Natural cell-mediated cytotoxicity in Sjögren's syndrome and rheumatoid arthritis.

Natural cell-mediated cytotoxicity (NCMC) was measured in 7 patients with Sjögren's syndrome without complication of connective tissue disease, 5 patients with Sjögren's syndrome associated with connective tissue disease other than rheumatoid arthritis (RA), 13 patients with Sjögren's associated with RA, and 29 patients with RA without Sjögren's. All 25 patients with Sjögren's syndrome showed a significantly suppressed NCMC. In the patients with RA but without Sjögren's, the NCMC for 21 female patients was rather enhanced when compared with controls, while 8 male patients did not differ from controls. The reduced NCMC in the Sjögren's patients did not seem to correlate with the presence of antilymphocyte antibody. A more accelerated rate of NCMC was demonstrated in the patients with RA who were receiving gold therapy.