1/46. Solitary plasmacytoma of the larynx and upper trachea associated with systemic lupus erythematosus.Solitary plasmacytoma rarely involves the larynx and trachea. This tumor caused severe laryngeal and tracheal obstruction in an adolescent female with systemic lupus erythematosus of nine years' duration. Her systemic disease had been in remission at the time she developed symptoms of laryngeal and tracheal obstruction by plasmacytoma invasion. A complete survey failed to reveal any other focus of tumor. Because steroid therapy failed to clear the plasma cell infiltrate, she was treated with irradiation after the tumor was surgically debulked endoscopically. Postirradiation biopsy showed no evidence of tumor, but the patient continued to experience moderate airway obstruction from fibrosis in the irradiated trachea. The patient has been followed, and is free of disease nine years after establishing the diagnosis.- - - - - - - - - - ranking = 1keywords = obstruction (Clic here for more details about this article) |
2/46. octreotide treatment of chronic intestinal pseudoobstruction secondary to connective tissue diseases.Chronic intestinal pseudoobstruction (CIPO) is a rare syndrome that may occur in association with connective tissue diseases (CTD). Effective management is a major challenge. We report 3 cases in which subcutaneous octreotide was efficacious in the treatment of digestive symptoms in CIPO. In 2 of the 3 cases, previous treatment with domperidone, cisapride, or erythromycin had been unsuccessful. All 3 patients underwent a regimen of oral antibiotics along with octreotide to stimulate small bowel motility. The effects of octreotide were evident within 48 hours after the first injection in all patients. In 2, the efficacy seemed to decrease after 1 week and 6 months respectively, but increasing the dosage led to another remission. CIPO in CTD is a severe condition that can evolve regardless of the underlying disease activity. octreotide appears to be efficacious in improving both clinical symptoms and manometric patterns. When its therapeutic effect diminishes, increasing the dosage can be useful.- - - - - - - - - - ranking = 1.6666666666667keywords = obstruction (Clic here for more details about this article) |
3/46. intestinal pseudo-obstruction in systemic lupus erythematosus: an uncommon but important clinical manifestation.OBJECTIVES: To document intestinal pseudo-obstruction (IpsO) as a recognised clinical manifestation of systemic lupus erythematosus (SLE) and a possible new clinical entity with its apparent association with ureterohydronephrosis. METHODOLOGY: We report six lupus patients who presented with IpsO and review 12 other cases from an English literature search. IpsO is defined as the presence of clinical features suggestive of intestinal obstruction but without organic obstruction, namely absence of bowel sounds, presence of multiple fluid levels on plain abdominal x-rays and exclusion of organic obstruction by imaging or surgical procedure. Other clinical characteristics related to the underlying lupus, serological and histological findings, treatment modalities and outcomes of these patients were reviewed. RESULTS: All 18 patients fulfilled the ACR revised classification criteria for SLE. None showed any clinical features of scleroderma or overlap syndrome. The mean age of onset of IpsO was 29.0 (15-47) y. The female to male ratio was 16:2. Nine patients had IpsO as the initial presentation of their underlying lupus. Coexisting lupus involvement of other organ systems included glomerulonephritis (n=7), thrombocytopenia (n=5) and cerebral lupus (n=3). The serology data and autoantibody profile of some of the previously reported patients were incomplete. In our series, anti-Ro antibody was positive in 5/6 while anti-RNP was found in 1/6 patients only. All our patients had active lupus serology at presentation. 17/18 patients required the use of high dose systemic corticosteroid therapy while one patient responded to topical adrenocorticotrophin hormone treatment. Response was good and was observed early after commencement. azathioprine was used as maintenance therapy in 6/18 patients with good effects. An apparent association with the presence of bilateral ureterohydronephrosis was found in 12/18 patients. These patients presented with dysuria without positive bacterial culture though features of chronic interstitial cystitis were not invariably found in these patients. CONCLUSION: IpsO is an uncommon but important manifestation of SLE. The underlying pathology is not fully understood but it may be related to immune complex deposition. The finding of coexisting ureterohydronephrosis suggests that there may also be a central smooth muscle motility problem of neuropathic or myogenic pathophysiology which may or may not be secondary to vasculitis. Early recognition and treatment of IpsO in SLE is important.- - - - - - - - - - ranking = 2.6666666666667keywords = obstruction (Clic here for more details about this article) |
4/46. Chronic intestinal pseudo-obstruction in systemic lupus erythematosus due to intestinal smooth muscle myopathy.We report the case of a woman with systemic lupus erythematosus initially manifesting with fever, rash and arthritis, and two years later with Class IV lupus nephritis. Following treatment with cyclophosphamide she developed symptoms and signs of chronic intestinal pseudo-obstruction (CIPO) that was initially thought to be due to a neutropenic enterocolitis. However, persistence of symptoms resulted in segmental resection of the ileum which showed widespread myocyte necrosis and active inflammation within the muscularis propria. A subsequent, more extensive ileocolic resection showed severe diffuse atrophy and fibrosis of the muscularis propria throughout the resected bowel. The absence of mesenteric vasculitis and the clinical response of the CIPO to the immunosupressive regimen of prednisolone and cyclosporin A suggest that the bowel muscle coat changes reflect an intestinal myopathy secondary to systemic lupus erythematosus, and may have an auto-immune etiology.- - - - - - - - - - ranking = 1.6666666666667keywords = obstruction (Clic here for more details about this article) |
5/46. Parafoveal arteriolar obstruction after ocular trauma in a patient with systemic lupus erithematosus.BACKGROUND: Retinal arterial obstruction after ocular trauma is a rarely described entity which presents a still controversial pathogenesis. However, intravascular leucoembolization in response to activation of complement factor 5 (C5a) has now been hypothesized as a possible cause of arterial embolization after severe trauma. Coincidentally, the pathogenesis of vascular lesions seen in active systemic lupus erithematosus is also related to the activation of the same complement factor. methods: We present the case of a 32-year-old man with active systemic lupus erithematosus who received a blunt trauma in his right eye and immediate developed severe visual loss. RESULTS: ophthalmoscopy of the affected eye revealed a cherry-red spot macula, small hemorrhages and edema of the posterior pole. fluorescein-angiography showed obstructions and staining of small branches of arterioles in the parafoveal region. After 2 months of follow-up, the ophthalmoscopic picture evolved to optic atrophy and the visual acuity remained severely reduced. CONCLUSION: We hypothesize that patients with complement-mediated diseases are predisposed to retinal arteriolar obstruction after ocular trauma.- - - - - - - - - - ranking = 2.3333333333333keywords = obstruction (Clic here for more details about this article) |
6/46. Cricoarytenoid arthritis with rheumatoid arthritis and systemic lupus erythematosus.A 56-year-old woman with rheumatoid arthritis (RA) suddenly developed severe respiratory distress and laryngeal stridor, which required endotracheal intubation. She had had RA for 12 years, which had been controlled well with prednisolone (3 mg/day) at the orthopedic clinic. laryngoscopy revealed cricoarytenoid arthritis. She was finally diagnosed as having overlap syndrome with RA and systemic lupus erythematosus. She was given high dose corticosteroids that improved her clinical symptoms and laryngoscopic findings. She represents the first patient with overlap syndrome who developed an acute airway obstruction due to cricoarytenoid arthritis.- - - - - - - - - - ranking = 0.33333333333333keywords = obstruction (Clic here for more details about this article) |
7/46. life-threatening angioedema in systemic lupus erythematosus.life-threatening angioedema involving the upper respiratory tract is an uncommon manifestation in systemic lupus erythematosus (SLE). We report three patients in their late adolescence who had laryngeal oedema causing airway obstruction and requiring mechanical ventilation during active disease following symptoms of a respiratory tract infection. All of them had major organ involvement from lupus and none had a family history of hereditary angioedema (HAE). The mechanisms of angioedema in SLE are heterogeneous. There has been little evidence so far of anti-Cl inhibitor (Cl INH) autoantibody in SLE patients with angioedema. These are the first three cases reported in the literature of life-threatening angioedema following respiratory tract infection in SLE.- - - - - - - - - - ranking = 0.33333333333333keywords = obstruction (Clic here for more details about this article) |
8/46. Severe upper airway obstruction from cricoarytenoiditis as the sole presenting manifestation of a systemic lupus erythematosus flare.Upper airway obstruction due to laryngeal involvement is a known complication of systemic lupus erythematosus (SLE). Laryngeal involvement typically accompanies inflammatory activity involving other sites and varies from mild mucosal inflammation to bilateral vocal cord immobility. Cricoarytenoid arthropathy is a rare cause of severe airway obstruction in patients with SLE and almost always occurs in the presence of other associated symptoms. Furthermore, in contrast to patients with rheumatoid arthritis, in whom chronic involvement of cricoarytenoid joints occurs more commonly and often requires surgical intervention, patients with SLE typically present with acute arthritis of cricoarytenoid joints and respond to corticosteroid therapy alone. We describe a patient with known SLE who presented with severe acute upper airway obstruction as the sole manifestation of active SLE after several years of quiescence. The laryngeal involvement progressed from mucosal inflammation to acute cricoarytenoiditis, despite the administration of high-dose corticosteroid therapy, necessitating emergent intubation and tracheostomy. This case illustrates the importance of considering SLE in the differential diagnosis of patients presenting with acute upper airway obstruction.- - - - - - - - - - ranking = 2.6666666666667keywords = obstruction (Clic here for more details about this article) |
9/46. intestinal pseudo-obstruction in systemic lupus erythematosus.In order to document intestinal pseudo-obstruction (IPO) as a recently recognized manifestation of systemic lupus erythematosus (SLE), we report the case or a woman with SLE who presented with IPO and we review 21 other previously reported cases from an English literature search. In 41% of the cases, IPO was the initial manifestation of their underlying lupus. The clinical and laboratory features were not significantly different from those reported in large series of patients with SLE, except for an apparent association with an urinary tract involvement (ureterohydronephrosis and interstitial cystitis). The pathogenic mechanism of this complication is not fully understood, but seems to be heterogeneous. IPO responded readily to high dose steroid therapy in all patients, but in some cases this complication evolved regardless of the underlying disease activity. A high level of awareness of this complication is needed to avoid unnecessary surgical intervention.- - - - - - - - - - ranking = 1.6666666666667keywords = obstruction (Clic here for more details about this article) |
10/46. Laryngeal involvement in systemic lupus erythematosus.Laryngeal involvement in systemic lupus erythematosus (SLE) can range from mild ulcerations, vocal cord paralysis, and edema to necrotizing vasculitis with airway obstruction. In this report, four cases showing the range of severity of this disease manifestation are presented, accompanied by a comprehensive review of the literature. The clinical course of 97 patients with laryngeal involvement with SLE are reviewed, of whom 28% had laryngeal edema and 11% had vocal cord paralysis. In the majority of cases, symptoms such as hoarseness, dyspnea, and vocal cord paralysis resolved with corticosteroid therapy. Other, less common causes of this entity included subglottic stenosis, rheumatoid nodules, inflammatory mass lesions, necrotizing vasculitis, and epiglottitis. The clinical presentation of laryngeal involvement in patients with SLE follows a highly variable course, ranging from an asymptomatic state to severe, life-threatening upper airway compromise. With its unpredictable course and multiple causations, this complication remains a diagnostic and therapeutic challenge to physicians involved in the care of patients with SLE.- - - - - - - - - - ranking = 0.33333333333333keywords = obstruction (Clic here for more details about this article) |
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