| 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. ( info) |
2/20. A pediatric case of sclerodermatous chronic graft-versus-host disease. We report a rare case of sclerodermatous chronic graft-versus-host disease (GVHD) in a 6-year-old boy that occurred after bone marrow transplantation for his aplastic anemia. The clinical manifestation and histopathologic findings were typical of scleroderma. Although various kinds of treatment have been tried for scleroderma, no established therapy exists. Furthermore, treating this disease is even more difficult in children. In the future, clarification of the pathogenesis of chronic GVHD and establishment of therapy will be necessary. ( info) |
3/20. Treatment of chronic graft-versus-host disease with anti-CD20 chimeric monoclonal antibody. We reviewed the clinical outcome of 8 patients with steroid-refractory chronic graft-versus-host disease (GVHD) who received an anti-CD20 chimeric monoclonal antibody (rituximab). Rituximab was given by intravenous infusion at a weekly dose of 375 mg/m(2) for 4 weeks. All patients had received extensive treatment with various immunosuppressive agents; 6 patients had also received extracorporeal photopheresis. All patients had extensive chronic GVHD with diffuse or localized sclerodermoid GVHD and xerophthalmia. Other extracutaneous involvements included cold agglutinin disease with the Raynaud phenomenon, membranous glomerulonephritis, and restrictive or obstructive lung disease. Four patients responded to treatment with ongoing resolution or improvement ranging from 265 to 846 days after therapy, despite recovery of B cells in 3 patients. Rituximab seems to have significant activity in the treatment of refractory chronic GVHD and should be considered for further study in patients with early disease. This study suggests a participating role of B cells in the pathogenesis of chronic GVHD. ( info) |
4/20. 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. ( info) |
5/20. Bilateral breast cancer associated with diffuse scleroderma. There is a strong evidence to suggest the association between breast cancer and diffuse scleroderma, though it is an infrequent occurrence. We describe the clinical and radiological findings in a patient who, over a period of 2 years, was diagnosed initially of diffuse scleroderma, next with right breast cancer and finally with left breast cancer. A review of the literature evidencing the relation between these two pathologies is provided. We suggest that special vigilance for tumoral pathology of the breast should be performed in patients with systemic scleroderma. ( info) |
6/20. 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. ( info) |
7/20. 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. ( info) |
8/20. A complete and durable clinical response to high-dose dexamethasone in a patient with scleromyxedema. We report a case of a patient with scleromyxedema limited to the skin with an associated IgG lambda monoclonal protein treated successfully with high-dose dexamethasone. We encourage the continued investigation of this complex relationship between the clinical presentation of scleromyxedema and its frequently associated paraproteinemia. ( info) |
9/20. 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. ( info) |
10/20. Chemotherapy-induced scleroderma: a pleiomorphic syndrome. A scleroderma-like disease has recently been described in association with taxanes. We present the first case of diffuse scleroderma occurring in a woman treated with doxorubicin and cyclophosphamide for breast cancer. The clinical pattern of skin involvement and histological alterations were identical to those found in the classical form of scleroderma. skin involvement progressed to affect 80% of total body area, and subsequently remained unchanged despite progression of the underlying cancer, making a paraneoplastic aetiology of the scleroderma unlikely. Specific chemotherapeutic agents might be directly responsible for the clinical manifestations and the parameters of progression. Analysis of all similar case reports defines the particular features and clinical course of this phenomenon. ( info) |