Regional diastolic function by tissue Doppler echocardiography in systemic sclerosis: correlation with clinical variables

The incidence of left ventricular (LV) diastolic dysfunction is increased in systemic sclerosis (SSc), while systolic dysfunction is present in a small percentage of patients. The aim of this study was to asses the LV "regional" diastolic abnormalities in SSc patients by the mean of Doppler tissue imaging (DTI). Echocardiographic echo-Doppler (DE) and DTI parameters were analyzed for 67 SSc patients: abnormal E/A ratio at DE was detected in 24, while abnormal e/a at DTI was observed in 41. A significant prevalence of DTI diastolic abnormalities in the segments reflecting longitudinal versus those reflecting radial LV motion was found. The segments of the basal regions of LV myocardium were significantly more involved than those of the middle portion. Linear correlation was observed between the extent of the diastolic abnormalities and the duration of disease. Longitudinal myocardial systolic velocities were significantly reduced in patients with abnormal e/a DTI

CURRENT STRATEGIES FOR THE TREATMENT OF AUTOIMMUNE DISEASES

Autoimmune disease therapy may be considered a puzzle under construction. Current treatments for autoimmune diseases include physical therapy, non-steroidal anti-inflammatory drugs (NSAIDs), corticosteroids, disease-modifying anti-inflammatory drugs (DMARDs), anticytokine therapies, inhibition of intracellular-signaling pathways, costimulation inhibition, biological inhibitors of T cell function, B-cell anergy and depletion, regulatory T cells, stem cell transplantion. New biologic drugs that target specific cells or cytokines involved in the early inflammatory response started because of their improved efficacy and limited toxicity. The hematopoietic stem cell transplantation represents a possible therapeutic strategy for autoimmune diseases resistant to available treatments

Digital ischemic necrosis in a patient with systemic sclerosis: The role of laser Doppler perfusion imaging

The impairment of microcirculation is a cardinal feature of systemic sclerosis (SSc). Raynaud's phenomenon and SSc can cause painful digital ulcers on the fi ngers or toes, with resulting complications such as serious infections, gangrene or possible amputation. This case report shows the possible role laser Doppler perfusion imaging (LDPI) could play in diagnosis and follow-up of digital arterial obstructions in SSc patients. LDPI is a non-invasive microvascular imaging technique able to provide maps of the cutaneous blood flow (CBF). We describe here the case of a woman affected by SSc who suddenly presented with pallor and pain; afterwards an area of necrosis became clearly visible at the distal phalanx of the third right fi nger. The LDPI of the dorsal surface of that fi nger showed a reduction of CBF. Selective arteriography was performed in order to assess the degree and location of the obstruction before proceeding with adventitiectomy. Following this procedure LDPI showed a normal CBF in the distal phalanx and complete healing of the necrotic area. Consequently, we can state that LDPI alone provides an indirect functional estimation of possible digital artery obstruction. Further, we can assert that the overall effect of the adventitiectomy resulted in an increase of the CBF. © 2009 by Verlag Hans Huber, Hogrefe AG, Bern

Skin Perfusion of Fingers Shows a Negative Correlation with Capillaroscopic Damage in Patients with Systemic Sclerosis

[No abstract available

SUBCUTANEOUS IMMUNOGLOBULIN THERAPY IN A PATIENT WITH MYOPATHIC DROPPED HEAD SYNDROME AND COMMON VARIABLE IMMUNODEFICIENCY

Prominent neck extension weakness is an uncommon clinical entity, also termed dropped-head syndrome, that may be part of a generalized neuromuscular disorder. We report here the case of a woman with dropped-head syndrome and pulmonary arterial hypertension secondary to systemic sclerosis. Subsequently, she developed common variable immunodeficiency and subcutaneous immunoglobulin therapy was started. After two months from the start of therapy we did not observe any improvement in the degree of flexion of the head, although the clinical examination shows an improvement in neck extensor muscle strength. Subcutaneous immunoglobulin therapy could be a possible therapeutic option for the treatment of myopathic neck extensor weakness

An unusual manifestation in a patient with Churg-Strauss syndrome: Isolated cardiac septum involvement

[No abstract available

Laser Doppler Perfusion Imaging Is Useful in the Study of Raynaud's Phenomenon and Improves the Capillaroscopic Diagnosis

OBJECTIVE: To investigate capillary morphology and skin blood flow of dorsal hands by nailfold videocapillaroscopy (NVC) and laser Doppler perfusion imaging (LDPI), respectively, in patients with primary Raynaud's phenomenon (PRP) and systemic sclerosis (SSc) and to compare the results with those obtained in healthy controls. METHODS: The study group consisted of 142 patients with SSc, 88 patients with PRP, and 147 healthy controls. NVC was performed in all the groups examined. In patients with SSc the capillaroscopic pattern was classified as early, active, or late group pattern. A baseline skin blood flow determination of the dorsum of the subject's hands was acquired through a low-energy 670 nm Lisca Laser Doppler Perfusion Imager. RESULTS: In the healthy controls the perfusion distribution pattern was homogeneous, with a proximal-distal perfusion gradient. In patients with PRP, the perfusion distribution pattern was homogeneous, but the proximal-distal perfusion gradient was absent. Finally, in patients with SSc the perfusion distribution pattern was dyshomogeneous and a proximal-distal gradient was absent. The minimum perfusion, mean perfusion, maximum perfusion, and standard deviation, calculated as variation by means of each measurement site, were significantly different in all the groups examinated. CONCLUSION: NVC represents the best method to analyze microvascular damage in rheumatic diseases. LDPI improves the evaluation of vascular damage in patients with SSc. The LDPI and the capillaroscopic images fully matched the definition of the various stages of vascular digital damage in SSc

Digital Ulcers as an Initial Manifestation of Systemic Lupus Erythematosus

Digital ulcers (DUs) and gangrene are common skin manifestations of connective tissue diseases, especially systemic sclerosis. Raynaud's phenomenon (RP) is an almost universal manifestation of systemic sclerosis, with 95% of all patients being affected, and resulting in DUs in approximately 30% of the patients each year. Although RP may be present in approximately 25-50% of the patients, DUs are relatively rare in systemic lupus erythematosus (SLE) and never present as an initial manifestation of disease. In this case report DUs appear as the initial manifestation of SLE in a young woman with a capillaroscopic scleroderma pattern and elevated systolic pulmonary arterial pressure