1/9. Progressive systemic sclerosis-polymyositis overlap syndrome with eosinophilic pleural effusion.Pleural fluid rarely occurs in patients with progressive systemic sclerosis (PSS) or polymyositis (PM) with no lesions in the pulmonary area. Pleural fluids in patients with autoimmune diseases are mostly dominated by monocytes and lymphocytes but very rarely contain increased eosinophils. We report a 55-year-old male with PSS-PM overlap syndrome and eosinophilic pleural effusion. air invasion into the pleural cavity and the antituberculous therapy could be ruled out as causes for the patient's eosinophilic pleural effusion, because the differential eosinophil count was already as high as 19% from the first thoracentesis before the start of antituberculous therapy. Infections and malignant tumor also were unlikely causes based upon the negative pleural fluid results and the negative pleural biopsy findings, except for nonspecific inflammation. After the administration of corticosteroid, the pleural effusion decreased promptly, with normalization of serum creatine phosphokinase and c-reactive protein concentrations.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
2/9. Continuous regional anesthesia before surgical peripheral sympathectomy in a patient with severe digital necrosis associated with Raynaud's phenomenon and scleroderma.BACKGROUND AND OBJECTIVE: Digital ischemia and necrosis caused by Raynaud's phenomenon in patients with connective tissue diseases may not respond to medical therapy and may have major adverse effects on quality of life. We describe the use of continuous ambulatory regional anesthesia for diagnosis and treatment before peripheral sympathectomy in a patient with secondary Raynaud's phenomenon. CASE REPORT: A 55-year-old man with progressive systemic sclerosis and secondary Raynaud's phenomenon presented with severe pain and digital necrosis that were refractory to maximal medical treatment and thoracic sympathectomy. Continuous ambulatory regional analgesia increased digital temperature from 32.3 degrees C at baseline to 34.4 degrees C after 80 minutes. An increase in digital flow was documented by Doppler ultrasound measurements made ventrally at the point of greatest pulsation of the radial artery. Subsequent peripheral sympathectomy resulted in restoration of nutrient flow with healing of ulcers and alleviation of pain. CONCLUSIONS: Continuous ambulatory regional anesthesia appears effective as a treatment bridge for vasospasm and ischemia associated with secondary Raynaud's phenomenon. The enhancement of peripheral blood flow achieved with the regional anesthetic technique suggests that surgical peripheral sympathectomy may provide long-term benefits.- - - - - - - - - - ranking = 0.2keywords = sclerosis (Clic here for more details about this article) |
3/9. Keloidal scleroderma.Progressive systemic sclerosis (PSS) can involve the skin diffusely or in a limited way. Rarely cutaneous involvement can assume a hypertrophic form, described in the literature as keloidal scleroderma. The authors describe a PSS patient who presented cutaneous manifestations compatible with the diagnosis of this clinical situation. The clinical features, pathogenic mechanism and therapeutic approach are discussed and a broad review of the literature is given.- - - - - - - - - - ranking = 0.2keywords = sclerosis (Clic here for more details about this article) |
4/9. Gemcitabine-associated scleroderma-like changes of the lower extremities.Gemcitabine is a nucleosid analog approved for use in the treatment of metastatic urothelial carcinoma of the bladder. We describe an unusual case of scleroderma-like changes of the lower extremities after treatment by gemcitabine for metastatic carcinoma of the bladder. The patient developed initial inflammatory edema (3 kg) restricted to the lower extremities and subsequent scleroderma-like changes after 2 cycles of gemcitabine. Cutaneous biopsy specimen revealed diffuse sclerosis without involvment of the fascia or muscle. Discontinuation of gemcitabine resulted in dramatic removal of the edema, softening of the skin, and partial reversibility of the fibrotic process. This is the first case report of a scleroderma-like reaction associated with gemcitabine. This antineoplastic agent must be added to the very limited number of cytostatic agents capable of giving rise to scleroderma-like features.- - - - - - - - - - ranking = 0.2keywords = sclerosis (Clic here for more details about this article) |
5/9. Ischaemic stroke in progressive systemic sclerosis.Progressive systemic sclerosis (PSS) or scleroderma is a multisystem disease affecting the skin, lungs, myocardium, kidneys and gastrointestinal tract. Primary involvement of cerebral arteries in PSS has been reported but is very rare. A 61-year-old woman suffering from scleroderma for six years was hospitalised for two subsequent episodes of transient acute dysarthria and left hemiparesis. After five hours from the first onset of symptoms, she was submitted to brain magnetic resonance (MR) protocol that showed a right subinsular ischaemic lesion and whole right middle cerebral artery (MCA) territory hypoperfusion. Intracranial and epiaortic MR angiography reported a focal stenosis in the M2 portion of MCA. She was immediately treated with i.v. high dose steroids and oral acetylsalicylic acid. At one-month follow up, MR findings were confirmed. We have documented a cerebral infarct in a PSS patient. In our opinion, the ischaemic stroke was caused by a localised autoimmune angiopathy.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
6/9. multiple myeloma with scleroderma-like changes.We present a case of a patient who presented concomitantly with generalized pruritus, brownish sclerodermatous plaques, sclerodactyly and a monoclonal band for IgG-kappa. The patient was diagnosed as having multiple myeloma by bone marrow examination. The rapidly progressive evolution with acute anuric renal failure, malignant hypertension and the skin sclerosis seem to be related to the neoplastic disorder. The scleroderma-like changes have to be differentiated from systemic scleroderma.- - - - - - - - - - ranking = 0.2keywords = sclerosis (Clic here for more details about this article) |
7/9. Angioimmunoblastic T cell lymphoma with an unusual proliferation of Epstein-Barr virus-associated large B cells arising in a patient with progressive systemic sclerosis.We report an unusual case of angioimmunoblastic T cell lymphoma arising in the setting of 5 years of immunosuppressive treatment for progressive systemic sclerosis. The lymph node lesion was accompanied by large blastic B cells with an association of Epstein-Barr virus. Southern blot study demonstrated the clonal rearrangement of T cell receptor beta-chain gene, but not of immunoglobulin heavy chain gene. Phenotypical examination of the lymph node also revealed the predominance of CD4 T cells in addition to the proliferation of follicular dendritic cells, but no light chain restriction in large B cell components. In the clinical and laboratory aspects, neutrophilia (15.8 x 10(9)/l) and plasmacytosis (40%) in bone marrow were noted, which were considered to be closely related to elevated serum granulocyte colony-stimulating factor, interleukin (IL)-4 and IL-6. Based on the combined data described here, our preferred diagnosis was angioimmunoblastic T cell lymphoma with Epstein-Barr virus-associated B cell lymphoproliferative disorder, the pathogenesis of which was suggested to be closely associated with immunosuppressive treatment for progressive systemic sclerosis.- - - - - - - - - - ranking = 1.2keywords = sclerosis (Clic here for more details about this article) |
8/9. Mandibular resorption in progressive systemic sclerosis: a report of three cases.Progressive systemic sclerosis is a generalized collagen disorder, which is characterized by fibrosis that involves skin, muscles and other organ systems like the gastrointestinal tract, lungs, heart and kidneys. Its oral manifestations include features like restricted mouth opening, widening of periodontal ligament space, pseudoankylosis, malocclusion and mandibular resorption. Mandibular resorption in systemic sclerosis is relatively uncommon and is reported only in 10% of cases. The purpose of reporting these three cases is to highlight the importance of screening all patients with advanced systemic sclerosis with panoramic radiographs. Panoramic radiographs are essential for early detection of resorption in the mandible to prevent possible consequences like pathological fractures, osteomyelitis and neuropathies.- - - - - - - - - - ranking = 1.4keywords = sclerosis (Clic here for more details about this article) |
9/9. Complete heart block in a patient with systemic sclerosis.We describe a patient with diffuse systemic sclerosis and presyncopal episodes where the electrocardiogram revealed complete atrioventricular heart block associated with left posterior and right heart bundle block. The patient underwent implantation of a permanent pacemaker.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |