Cases reported "Sarcoidosis"

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1/35. Annular vasculitis in association with sarcoidosis.

    We report a case of cutaneous vasculitis with annular features in association with sarcoidosis. A 36-year-old woman presented with fever, polyarthralgias, erythema nodosum, bilateral hilar lymphadenopathy, and induration of a long-standing scar on the face. In addition, she developed annular, erythematous, and purpuric patches over her thighs and buttocks that were histologically characterized by a small vessel leukocytoclastic vasculitis. The presence of circulating immune complexes in the early stages of sarcoidosis might be related to the occurrence of the vascular damage.
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2/35. Histopathological findings in proliferative membrane from a patient with sarcoid uveitis.

    BACKGROUND: Sarcoid uveitis is occasionally accompanied by proliferative changes, such as retinal neovascularization and vitreous hemorrhage. Steroid administration, retinal photocoagulation, and vitrectomy may be indicated in such proliferative cases. CASE: A 19-year-old woman presented with proliferative sarcoid uveitis accompanied by recurrent vitreous hemorrhage. OBSERVATIONS: At the initial examination, bilateral vitreous opacity, retinal exudates, mild vitreous hemorrhage, retinal vasculitis, and neovascularization of the retina and optic disc were observed. Although prednisolone was administered and panretinal photocoagulation was performed several times, recurrent vitreous hemorrhage continued. Since the vitreous hemorrhage was not absorbed, pars plana vitrectomy and lensectomy were performed. After surgery, neovascularization and intraocular inflammation decreased, and the corrected visual acuity in the right eye improved to 20/50. Histopathologic analysis of the proliferative membrane removed during surgery revealed substantial neovascularization and numerous neutrophils in the vessels. CONCLUSIONS: Based on these findings, an inflammatory reaction as well as retinal ischemia were thought to be involved in the proliferative changes in this patient.
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3/35. sarcoidosis presenting with large vessel vasculitis and osteosclerosis-related bone and joint pain.

    A 34-year-old African-American female diagnosed earlier with idiopathic thrombocytopenic purpura (ITP), lymphadenopathy, splenomegaly, uveitis, and pulmonary nodules, developed a subclavian artery aneurysm, and generalized annular osteosclerotic lesions with disabling arthralgias. Biopsies from bone and lymph node revealed non-caseating granulomas and no evidence of malignancy or infection, confirming the clinical impression of sarcoidosis.
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4/35. sarcoidosis and systemic vasculitis.

    BACKGROUND: systemic vasculitis is an unusual complication of sarcoidosis. Over a 10-year period, the authors have provided care for six patients who had features of both sarcoidosis and vasculitis. Vasculitis could not be attributed to other causes. OBJECTIVES: To report six patients (five children) who had sarcoidosis and systemic vasculitis and compare our experience with previous literature. To better delineate the clinical spectrum of sarcoid vasculitis and its response to therapy. methods: Retrospective analysis and a medline literature review of sarcoid and concurrent vasculitis from 1966. RESULTS: Our six patients had systemic illnesses that included fever, peripheral adenopathy, hilar adenopathy, rash, pulmonary parenchymal disease, musculoskeletal symptoms, and scleritis or iridocyclitis. Biopsies revealed features compatible with the diagnosis of sarcoidosis or necrotizing sarcoid granulomata in either skin, lymph node, lung, synovium, bone, bone marrow, liver, trachea, or sclera. Arteriography showed features of large vessel vasculitis in three patients, all of whom were African American, whereas patients with small vessel vasculitis were white. Prior reports of sarcoid and vasculitis included 14 adults, of whom half had predominantly small vessel disease, and half had medium- or large-sized vessel disease. Eight previously reported children included seven with primarily large vessel sarcoid vasculitis. Racial background was noted in 15 reported cases and included whites (6), african americans (5), and Asians (4). Among the authors' six patients, four improved when treated with prednisone alone. However, relapses occurred when the drug was tapered or withdrawn. CONCLUSIONS: sarcoidosis may be complicated by systemic vasculitis that can affect small- to large-caliber vessels. Sarcoid vasculitis can mimic hypersensitivity vasculitis, polyarteritis nodosa, microscopic polyangiitis, or Takayasu's arteritis. African American and Asian patients are disproportionately represented among cases with large vessel involvement. Corticosteroid and cytotoxic therapy is palliative for all forms of sarcoid vasculitis. However, relapses and morbidity from disease and treatment is common.
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5/35. Concurrence of sarcoidosis and aortitis: case report and review of the literature.

    takayasu arteritis (TA) is a rare manifestation of systemic large vessel vasculitis which affects predominantly the aorta and its main branches, but often remains unrecognised owing to delayed diagnosis and non-characteristic clinical features. sarcoidosis, too, is a systemic inflammatory disease which can affect virtually any organ system. Reports about the coincidence of both diseases have appeared. The case presented here is characterised by a significant time lag between detection of TA and appearance of clinical signs of sarcoidosis. The woman, now 39 years old, had erythema nodosum, circumscript alopecia, and recurrent uveitis, which dated back to 1980 and was attributed to sarcoidosis. At least 12 years later aortic valve insufficiency with progressive cardiac failure developed. histology performed at the time of aortic valve prosthesis in 1997 disclosed a diagnosis of TA, which was confined to the aortic root. Incidentally, sarcoidosis was diagnosed in adjacent lymph nodes. A thorough check up failed to detect further manifestations of TA; thus, possibly, the patients had aortitis similar to, but not identical with, TA.Several related cases previously reported are discussed, suggesting that both diseases may be inherently related as they are characterised by certain non-specific, immunoinflammatory abnormalities. This case report suggests that the prevalence of TA, or related forms of arteritis, may be higher than expected and should be considered, especially in younger patients with non-characteristic cardiovascular symptoms and suspected systemic inflammatory disease. Moreover, the association with sarcoidosis in this and other previously described cases suggests that the two diseases may be related and that TA or TA-like vasculitis may even be a complication of sarcoidosis.
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6/35. Radionuclide studies in bronchogenic carcinoma of the Hilum. Scintigraphy and tomography: their complementary features.

    Thirty-eight cases with direct or indirect signs of hilar masses were investigated by roentgenologic, radioisotopic and surgical methods. Reasonable correlation between tomography and scintigraphy was confirmed, substantiating their complementary nature. Bronchogenic carcinoma of the central airways was most frequent among the hilar masses. Masses as well as other involvement of the bronchovascular structures of the hilum on conventional tomography were confirmed by the gallium-67 scan, and inhalation and perfusion scintigraphy. Some cases which simulated bronchogenic carcinoma were presented. Hilar masses without destruction of the bronchovascular structures showed normal inhalation and perfusion scintigrams with positive gallium-67 accumulation. These lesions were metastatic cancer, malignant lymphoma, and sarcoidosis. If these diseases involve the airways and the vessels of the hilum, differentiation from bronchogenic carcinoma may naturally be difficult.
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7/35. Neuro-ophthalmic sarcoidosis.

    sarcoidosis is a multisystem disorder in which ocular involvement occurs in about one-quarter and neurosarcoidosis in 7 per cent of patients. When the retina is involved, the reported incidence of central nervous system sarcoidosis is 37 per cent. The patient described had a transient papular eruption of the legs, bilateral hilar lymphadenopathy, polyarthralgia with knee effusions, and bilateral facial and peripheral neuropathy. Ocular involvement was characterized by anterior uveitis (in the initial stages), vitreous flare, bilateral disc oedema, macular oedema, streak haemorrhages, peripheral periphlebitis, nerve fibre bundle defects, and candle-wax spots. fluorescein angiography showed no fluorescence of the candle-wax spots nor of the adjacent vessels. However, there was hyperfluorescence of two retinal lesions. This patient had unilateral internal ophthalmoplegia, only three cases of which have been reported in the literature. Her health was restored by heavy, prolonged corticosteroid therapy. Her family history revealed that an uncle died of sarcoidosis complicated by cryptococcal meningitis. The literature on retinopathy in sarcoidosis is reviewed and the lesions noted in the posterior segment are listed.
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8/35. Peripheral retinal neovascularization in sarcoidosis.

    Three black patients with peripheral retinal sea fan neovascularization were found to have sarcoidosis. The periphlebitic process may be the cause of stasis, hypoxia, and a secondary vasoproliferative stimulus. We noted the peculiar finding of a vessel passing through the neovascular tissue into the peripheral retina. This feature may help in the differential diagnosis.
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9/35. Pulmonary sarcoidosis: A clinico-pathological study.

    The light and electron microscopic changes in biopsy tissue from the lung of a 30-year-old housewife severeley incapacitated by diffuse pulmonary sarcoidosis with pulmonary hypertension are presented. The lung tissue was distorted by numerous granulomas in the interstitial tissues and within alveoli. Many pulmonary blood vessels including arteries were damaged by the granulomas. The ultrastructural features of the epithelioid cells were found to be distinctive and probably specific. The giant cells which accompanied the epithelioid cells contained two types of inclusion body: one appeared to be related to the Schaumann body but the nature and origin of the second type was not clear. Many of the granulomas were surrounded by avascular fibrous tissue which contained, in addition to mature fibroblasts, myofibroblasts and a primitive form of cell that appeared to be a fibroblast precursor. It was conjectured that the myofibroblasts, through their contractile powers, might increase the distortion of the lung architecture and thereby the patient's disability. The alveolar walls were thickened by a diffuse infiltrate of macrophages and epithelioid cells but there was no excess of collagen and elastic fibres. The evidence suggested that the epithelioid cells developed from macrophages. From the cellular nature of the diffuse infiltration of the alveolar walls and the absence of fibrosis it seemed that the disease was still at an early and active stage, a conclusion strengthened by the fact that treatment with corticosteroids led to marked and sustained clinical improvement.
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10/35. Effectiveness of infliximab in treating selected patients with sarcoidosis.

    OBJECTIVE: To assess the effectiveness of infliximab (Remicade) in the treatment of patients with sarcoidosis who either do not respond to corticosteroids and other conventional drugs or develop unacceptable side effects to these drugs. DESIGN: A clinical, non-randomized, off-label study. SETTING: sarcoidosis clinic at a university teaching hospital. patients: Twelve biopsy-proven sarcoidosis patients, nine women and three men ranging from 45 to 70 years of age with chronic multisystem sarcoidosis refractory to corticosteroids or alternative treatment. INTERVENTION: Infliximab was infused at a dedicated ambulatory infusion center. The initial dose was 3mg/kg body weight and subsequent doses were given at weeks 2, 4, 6, 10, and 14. All patients received at least six infusions. RESULTS: All 12 patients improved significantly. One patient had a mild allergic drug reaction that responded to antihistamine. One patient, after 3 months of stopping infliximab treatment, died of a ruptured blood vessel in the abdomen. At autopsy a plasma cell dyscrasia was found. CONCLUSION: Infliximab is safe and effective in treating those patients with multisystem sarcoidosis who are either refractory or develop side effects to a standard regimen of corticosteroids and immunosuppressive agents.
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