131/1592. Extensive sterile abscess in an invasive fibrous thyroiditis (Riedel's thyroiditis) caused by an occlusive vasculitis. Riedel's thyroiditis is a rare disease determined by an invasive fibrosclerotic transformation of the thyroid gland. It may be one manifestation of multifocal fibrosis with still unknown etiology. Because it mimics carcinoma, a biopsy must be performed to get the correct diagnosis. The condition is self-limiting when confined to the neck. prognosis depends on the extent of extracervical fibrosclerosis. We present a patient with a huge cervical and mediastinal, unilateral thyroid mass expanding to the aortic curve, which led to tracheal deviation and compression with symptoms of stridor and dyspnea. These symptoms continued under a course of high-dose steroids; thus an operation was necessary to relieve the airway obstruction and limit inflammation. Intraoperative and pathological findings showed an inflammatory infiltration of the adjacent neck muscles and a sterile abscess caused by an occlusive vasculitis. Therefore, hemithyroidectomy had to be performed instead of a local limited resection. ( info) |
| We report a 55-year old woman with microscopic polyangiitis who presented with idiopathic pulmonary fibrosis and 1 year later developed hematuria and proteinuria. She had a high serum level of perinuclear anti-neutrophilic cytoplasmic antibodies. Renal angiogram was normal. The diagnosis of microscopic polyangiitis was confirmed by renal biopsy, which showed pauci-immune crescentic glomerulonephritis. The patient received immunosuppressive therapy and improved markedly. Consideration of small vessel vasculitis is important in the differential diagnosis of idiopathic pulmonary fibrosis. ( info) |
| A patient with type I cryoglobulinemia and monoclonal gammopathy of uncertain significance was found to have acute gallbladder vasculitis. The most prominent manifestation was upper abdominal pain in the setting of normal liver tests. An abdominal ultrasound demonstrated a thickened gallbladder wall, along with gallstones. HIDA scanning showed a nonfunctioning gallbladder with an edematous and thickened wall. There was characteristic leukocytoclastic vasculitis affecting the gallbladder. The patient recovered uneventfully subsequent to cholecystectomy. gallbladder vasculitis should be considered in patients with unexplained upper abdominal pain and systemic vasculitis. ( info) |
134/1592. Granulomatous angiopanniculitis of the breast. A case report. BACKGROUND: Granulomatous angiopanniculitis (GAP) is a rare benign condition of the breast of unknown etiology. Clinically and by fine needle examination, GAP may simulate breast carcinoma. The cytologic characteristics have not been described before. CASE: A 63-year-old female exhibited a palpable mass in her left breast. The fine needle aspirate contained both epithelioid and stromal elements. The epithelioid component consisted of dissociated individual cells and small groups and clusters of atypical cells. The stromal component showed a uniform, not-atypical pattern. The lumpectomy specimen showed nonnecrotizing granulomatous panniculitis and lymphoid angiitis without involvement of ducts or lobules. CONCLUSION: Granulomatous lesions should be borne in mind in the differential diagnosis of breast cancer in fine needle aspiration cytology. GAP must be histopathologically distinguished from granulomatous inflammation in the breast of autoimmune or infectious origin as specific medical therapy may be available for these latter diseases. ( info) |
| The etiology of acute intraabdominal bleeding is often unclear at the time emergent arteriography is performed. During localization and embolization, the arteriogram may suggest the diagnosis of vasculitis. However, controlling the bleeding remains the priority. connective tissue diseases such as ehlers-danlos syndrome (EDS) may also cause bleeding and mimic vasculitis and must be included in the differential diagnosis. We present such a case in which the initial findings were misleading. ( info) |
136/1592. Localized neurological necrotizing vasculitides. Three cases with isolated mononeuritis multiplex. Localized vasculitic neuropathies are increasingly reported. We describe 3 cases of peripheral neuropathy with necrotizing vasculitis confined to nerves and muscles without systemic involvement. These neuropathies were severe and relapsing, in contrast to a usually benign prognosis. Our cases appear to be isolated vasculitic neuropathies, with vasculitis strictly limited to the peripheral neuromuscular system without nonspecific clinical and/or biological systemic involvement. ( info) |
137/1592. Reversible posterior leucoencephalopathy syndrome in systemic lupus and vasculitis. OBJECTIVES: Reversible posterior leucoencephalopathy syndrome (RPLS) may develop in patients with renal insufficiency, hypertension, and immunosuppression, and is managed by prompt antihypertensive and anticonvulsant treatment. Four patients with renal insufficiency and fluid overload associated with Wegener's granulomatosis (one patient) and systemic lupus erythematosus (SLE) (three patients) are described, whose clinical picture and neuroimaging indicated RPLS. case reports: All patients had headache, seizures, visual abnormalities, and transient motor deficit, and were hypertensive at the onset of the symptoms. Head computed tomography (CT) scan and magnetic resonance imaging showed predominantly posterior signal abnormalities, which were more conspicuous on T(2) weighted spin echo images than on CT scan. All patients had some form of cytotoxic treatment shortly before the syndrome developed, and dramatically responded to blood pressure control and anticonvulsant treatment. In two patients with SLE, dialysis was required for renal insufficiency. DISCUSSION: Follow up neuroimaging studies showed almost complete resolution of signal abnormalities, and suggested that RPLS was associated with cerebral oedema without concomitant infarction. The treatment of hypertension and neurotoxic condition such as uraemia appears of primary importance, while immunosuppressive treatment may cause further damage of the blood-brain barrier. ( info) |
138/1592. Isolated digital vasculitis in a patient with rheumatoid arthritis: good response to tumour necrosis factor alpha blocking treatment. Tumour necrosis factor alpha (TNFalpha) blocking agents are among the most promising new treatments for rheumatoid arthritis (RA). However, no data exist about the effect of these agents on extra-articular manifestations of RA. A patient is described with small vessel vasculitis that repeatedly responded well to treatment with the soluble p55 TNFalpha receptor fusion protein Ro 45-2081 (lenercept). ( info) |
139/1592. Lepromatous vasculitis successfully treated by plasma exchange (PE). Vascular involvement is presently considered a "common pathway" in a number of diseases that is mediated by circulating immune complexes (CIC). CIC are found in the circulation when the disease is active and in single patients their level may parallel disease activity. Lepromatous leprosis is characterized by the presence of CIC and deposits of immunoglobulins and complement in vascular lesions of the different organs and an Arthus-like mechanism is considered as the basis for the clinical picture. The same mechanism is considered to play an essential pathophysiologic role in Lucio's phenomenon, which is characterized by lymphohistiocytic vascular infiltrates with or without thrombosis and secondary cutaneous infarction. Lepromatous vascular involvement is mediated by CIC whose antigen composition is known, the same as it is with HCV mediated cryoglobulinemia, HBV positive panarteritis nodosa, rheumatoid vasculitis, or Wagner's granulomatosis, which are usually treated by PE [1-3]. PE has been employed for lepromatous vasculitis since 1979 [4] and other cases have been successfully treated afterwards [5,6]. We report on another patient successfully treated by plasma exchange. ( info) |
140/1592. microscopic polyangiitis complicated with massive intestinal bleeding. microscopic polyangiitis (MPA) is associated with renal dysfunction, in most cases, and occasionally with pulmonary hemorrhage. However, massive intestinal bleeding is a rare manifestation. We report a case of MPA in a man who developed arterial bleeding in the small intestine. A 74-year-old man was admitted after enduring a fever for 4 weeks. Laboratory examination revealed leucocytosis with neutrophil predominance, and renal dysfunction was noted. He did not respond to treatment with antibiotics, and the worsened renal function soon required hemodialysis. On the seventh hospital day, he suddenly developed massive melena. A colonoscopic examination suggested bleeding in the small intestine, and abdominal angiography demonstrated that the bleeding originated in a branch of the ileal artery. hemostasis was achieved by transarterial embolization (TAE), but the patient suffered a massive pulmonary hemorrhage 2 days after the TAE. Although he responded well to a combination treatment with corticosteroid and cyclophosphamide, recurrence of pulmonary hemorrhage led to death, on the 87th hospital day. MPA in this patient was associated with three serious complications; deteriorating renal function, massive melena, and pulmonary hemorrhage. Of the various manifestations associated with MPA, arterial bleeding in the gastrointestinal tract, although rare, should be considered as one of the serious complications in MPA. ( info) |