1/35. Multisystem neuronal involvement and sicca complex: broadening the spectrum of complications.We report two patients with multisystem neuronal involvement associated with sicca complex. One had a lower motor neuron syndrome combined with a flaccid bladder and rectum. The second patient had unilateral hearing loss, sensory neuronopathy, Adie's pupils, upper motor neuron signs, and autopsy-proven anterior horn cell degeneration. Our cases lead us to propose that the spectrum of neuronal involvement occurring with sicca syndrome may be wider than is currently appreciated.- - - - - - - - - - ranking = 1keywords = upper (Clic here for more details about this article) |
2/35. lung adenocarcinoma in lymphocytic interstitial pneumonitis associated with primary sjogren's syndrome.We experienced a rare case of lung adenocarcinoma associated with lymphocytic interstitial pneumonitis caused by primary sjogren's syndrome. A 78-year-old woman was referred to our hospital because of progressive sicca syndrome and nodular opacities in the right lower lobe on chest radiograph. This patient was diagnosed as primary sjogren's syndrome by a labial gland biopsy and classical clinical features including xerophthalmia, xerostomia and immunoserological findings. Pathological findings including immunohistochemical studies in a surgically resected lung revealed adenocarcinoma in lymphocytic interstitial pneumonitis associated with primary sjogren's syndrome. There was no evidence of malignant lymphoma in the lymph nodes or resected lung tissue. Pulmonary involvement of sjogren's syndrome is now regarded both clinically and histopathologically as a wide spectrum of lymphoproliferative disorders ranging from benign to malignant. However, lung cancer associated with primary Sjogren's syndrome, as in our case, has apparently not been reported previously.- - - - - - - - - - ranking = 3.5418005912389keywords = chest (Clic here for more details about this article) |
3/35. Large annular purpura and paraneoplastic purpura in a patient with sjogren's syndrome and cervical cancer.We report a 79-year-old female with anaphylactoid purpura on her legs and unusual large annular purpura on the trunk. Histopathological characteristics of leukocytoclastic vasculitis were observed in the upper and middle dermis of both types of skin lesions. She was complicated by sjogren's syndrome and advanced cervical cancer. The annular purpura spontaneously resolved in a week and did not recur. However, the anaphylactoid purpura relapsed more frequently and spread more widely following the elevation of her serum SCC antigen levels from the onset of purpura until her death. We consider that the characteristic annular configuration was caused by the complication of sjogren's syndrome and that the recurrent anaphylactoid purpura indicated paraneoplastic vasculitis primarily caused by the tumor specific protein immune complexes. Complication by Sjogren's syndrome many also play a role in the development of allergic vasculitis.- - - - - - - - - - ranking = 1keywords = upper (Clic here for more details about this article) |
4/35. Diffuse angiodysplasia of the upper gastrointestinal tract in a patient with hypertrophic obstructive cardiomyopathy.A 64-year-old woman with a known history of hypertrophic obstructive cardiomyopathy presented with severe anemia of unknown origin. She had also suffered from repeated episodes of upper gastrointestinal bleeding for the previous 3 years. Despite bone marrow examination and panendoscopic and angiographic studies, the origin of anemia remained undefined until a small bleeding site was found during a duodenoscopic examination. The lesion proved to be angiodysplasia. This case report is interesting in that angiodysplasia elicited gastrointestinal bleeding and was the cause of anemia. In the international literature, there are very few reported cases of bleeding from gastrointestinal angiodysplasia in association with subvalvular aortic obstruction.- - - - - - - - - - ranking = 5keywords = upper (Clic here for more details about this article) |
5/35. Localised nodular pulmonary amyloidosis in a patient with sicca syndrome.A 52 year old Chinese woman with a 25 year history of sicca syndrome (primary Sjogrens syndrome) was investigated for 3 episodes of haemoptysis. Clinical examination was unremarkable except for the presence of dry eyes and xerostomia. Computed tomography of the chest revealed a lobulated mass in the posterior basal segment of the left lower lobe. Histopathological examination of this resected nodule confirmed the diagnosis of nodular amyloidosis. The normal radiolabelled serum amyloid P component scintigraphy and the absence of monoclonal plasma cell dyscrasia in the bone marrow strongly support the diagnosis of localised nodular pulmonary AL amyloidosis in this patient. Nodular pulmonary amyloidosis can be associated with sicca syndrome and often simulates bronchogenic carcinoma, bronchiectasis or pulmonary tuberculosis.- - - - - - - - - - ranking = 3.5418005912389keywords = chest (Clic here for more details about this article) |
6/35. Erythematous swelling of the lip associated with sjogren's syndrome and mimicking cheilitis granulomatosa.A 64-year-old Japanese woman developed therapy-resistant erythematous swelling of her upper lip. Our tentative clinical diagnosis of cheilitis granulomatosa was ruled out later by the laboratory findings including increased levels of anti-nuclear-antibody (ANA), anti-SSA/Ro antibody, and positive Schirmer test as well as by a histopathological picture showing a dense perivascular infiltration of plasma cells and mononuclear cells in the dermis instead of granulomatous changes. To the best of our knowledge, this is the first patient in whom annular erythema associated with S ogren's syndrome involved only the upper lip and produced clinical features simulating cheilitis granulomatosa.- - - - - - - - - - ranking = 2keywords = upper (Clic here for more details about this article) |
7/35. diagnosis of a gastric mucosa-associated lymphoid tissue lymphoma by endoscopic ultrasonography-guided biopsies in a patient with a parotid gland localization.We report the case of a 32-year-old man with a low-grade mucosa-associated lymphoid tissue (MALT) lymphoma of the parotid gland associated with Sjogren syndrome. He underwent an upper endoscopy as part of the screening of a gastric localization which showed a diffuse non-specific gastritis. However, endoscopic ultrasonography (EUS) evidenced a focal wall thickening of the vertical portion of the smaller curvature. EUS-guided biopsies of this area disclosed a MALT lymphoma, whereas biopsies under endoscopy concluded to mild chronic gastritis. The search for helicobacter pylori infection remained negative. Four months after treatment with anti-CD20 antibodies, EUS showed a diminution of the abnormal thickening of the second layer. Regression was confirmed histologically on new EUS-guided biopsies. MALT lymphoma is usually considered a localized disease; however, dissemination is probably more frequent than initially believed. Our case reflects the importance of a systematic screening for a gastric localization in patients with MALT lymphoma of the salivary glands. In this situation, association to autoimmune disease such as Sjogren syndrome is more likely to explain the gastric location than infection with H. pylori. Endoscopic ultrasonography has a major impact for the staging of gastric MALT lymphoma, but may also help diagnose focal infiltration by the disease.- - - - - - - - - - ranking = 1keywords = upper (Clic here for more details about this article) |
8/35. Pulmonary MALT lymphoma with amyloid production in a patient with primary sjogren's syndrome.A 53-year-old woman was admitted to our hospital complaining of cough, low grade fever, chest pain and sicca symptoms. A chest radiograph showed an abnormal shadow and chest computed tomography revealed a tumor in left S6. She was diagnosed as sjogren's syndrome by sialography and histological findings of labial biopsy. The surgically resected tumor specimen showed proliferation of lymphoid cells with lymphoepithelial lesions, which were positive for CD20 and kappa light chain. Kappa light chain-positive amyloid was found within the tumor. The tumor showed rearranged kappa light chain genes. The diagnosis was pulmonary mucosa associated lymphoid tissue lymphoma with amyloid production.- - - - - - - - - - ranking = 10.625401773717keywords = chest (Clic here for more details about this article) |
9/35. The inadvisability of thoracoscopic lung biopsy on patients with pulmonary hypertension.The use of video-assisted thoracoscopic surgery (VATS) sometimes leads to additional and unnecessary risks compared with thoracotomy. We report a troubling case of VATS lung biopsy in a 43-year-old woman with mild pulmonary hypertension. A progressive elevation of pulmonary artery pressure (PAP) was noted after the commencement of right unilateral ventilation. When the systolic PAP reached 90 mm Hg (390 min after induction of anesthesia), a massive blood discharge through the chest drain occurred. At repeat thoracotomy, continuous blood spouting was seen from > 10 of the surgical sites. It was supposed that the endoscopic staplers were unable to maintain hemostasis with such a high PAP.- - - - - - - - - - ranking = 3.5418005912389keywords = chest (Clic here for more details about this article) |
10/35. Lymphocytic interstitial pneumonitis associated with Epstein-Barr virus in Systemic Lupus Erythematosus and sjogren's syndrome. Complete remission with corticosteriod and cyclophosphamide.Lymphocytic interstitial pneumonitis (lip) is characterized by diffuse bilateral pulmonary infiltrations in both lower lobes. Pleomorphic lymphohistiocytes including mature lymphocytes, variable admixture of plasma cells and other mononuclear cells infiltrate within the pulmonary interstitium, ranging from widened septa to confluent masses. We report a case of lip associated with Epstein-Barr virus in a patient with SLE and sjogren's syndrome. A 50-year-old woman was admitted with insidious onset of progressive dyspnea for 20 days. She suffered from arthritis 10 years earlier without specific diagnosis. A radiography of chest has showed bilateral consolidative infiltrations with pleural effusion in both lower lung fields. Open lung biopsy documented lymphocytic interstitial pneumonitis which expressed Epstein-Barr virus genome in situ hybridization study. Following corticosteroid and cyclophosphamide therapy, clinical symptoms and radiologic infiltrations gradually remitted.- - - - - - - - - - ranking = 3.5418005912389keywords = chest (Clic here for more details about this article) |
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