Cases reported "Sarcoidosis"

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1/18. 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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2/18. Nontraumatic, nonneoplastic subglottic stenosis.

    During a 20-year period, 12 patients with nontraumatic, nonneoplastic subglottic stenosis were seen at the Mayo Clinic. The etiologic factors were relapsing polychondritis, amyloidosis, sarcoidosis, and Wegener's granulomatosis. Because of the diverse initial presentation of the disease, the clinician should consider that the stenosis is a manifestation of a systemic disorder and carry out an otolaryngologic and physical examination with the appropriate roentgenograms and blood and urine tests. Treatment, if a systemic disease is proved, consists of appropriate medication. Surgery may be necessary, depending on the nature of the lesion. Small strictures may not need to be treated.
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3/18. Sudden death due to granulomatous myocarditis: a case of sarcoidosis?

    INTRODUCTION: We report a case of sudden death due to granulomatous myocarditis and propose that cardiac sarcoid could have been the underlying aetiology. This is the first case reported in singapore. The differential diagnoses for granulomatous myocarditis including sarcoidosis and its cardiac manifestations as well as idiopathic giant cell myocarditis are discussed. CLINICAL PICTURE: A 53-year-old Indian woman died suddenly and autopsy revealed bilateral hilar adenopathy and myocardial infiltrates which proved to be granulomatous in nature. CONCLUSION: sarcoidosis may not be a rarity here and it is important to recognise the different clinical manifestations.
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4/18. cryptococcosis and sarcoidosis: strange bed-fellows. A report of five cases.

    BACKGROUND AND AIM: sarcoidosis is known to predispose to cryptococcal infection. In this case series, variations in presentation, diagnostic dilemmas and responses to treatment are highlighted. methods: Of several hundred patients referred to the sarcoidosis clinic at The Prince Charles Hospital, Brisbane and to the private clinic of the co-author between 1990 and 2002, five subsequently developed cryptococcal infections as a complication of sarcoidosis. All five subjects were treated by the co-author (RKAA). A review of the literature was also performed. RESULTS: Cryptococcal infection occurred in 4 patients with sarcoidosis being treated with steroids and one patient who was not on treatment. All responded to antifungal therapy. cryptococcosis was diagnosed by transbronchial (2), bronchial (1) and cutaneous (2) biopsies. fluconazole was used in those with pulmonary infections but not in cutaneous disease where excision sufficed. One patient with pulmonary cryptococcosis from immunosuppressants also developed a nocardial brain abscess. CONCLUSIONS: Our series of patients with sarcoidosis and cryptococcal infections is unique in australia. Although an unusual infection, cryptococcosis should always be considered in patients with sarcoidosis as it may be overlooked particularly in the lungs and can be fatal if untreated. Further immune dysregulation through steroid use may contribute significantly to the disease manifestations. Understanding why cryptococcosis and not other infections is more common in patients with sarcoidosis may reveal more about the mechanisms of granuloma formation and the nature of sarcoidosis itself.
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5/18. Blau syndrome presenting with ichthyosis.

    A 12-year-old girl presented with uveitis, joint disease and ichthyosis resembling ichthyosis vulgaris. A biopsy taken from the affected lower leg demonstrated sarcoidal-type granulomas. Synovial biopsy from the knee also showed granulomas. There was a family history of similar clinical features in the patient's younger sister. There were no other systemic features present to suggest a diagnosis of sarcoidosis or other granulomatous disease such as Crohn's disease or tuberculosis. The familial nature of the condition also made these diagnoses less likely. A clinical diagnosis of Blau syndrome was made. Blau syndrome is an uncommon sarcoidosis-like multisystem autosomal-dominant granulomatous disorder caused by mutations in the CARD15 gene. This gene has also recently been found to be a factor in the development of psoriatic arthritis and Crohn's disease. Although many forms of skin involvement have been described in Blau syndrome, this is the first case described of ichthyosis as the primary skin manifestation.
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6/18. Interpretation of granulomatous lesions in malignancy.

    In patients with malignancy the most frequent granuloma-associated conditions are tumor-related sarcoid reactions, sarcoidosis, tuberculosis and other infections. Quite often, the finding of granulomatous lesions in patients with cancer may lead to difficulties of interpretation resulting in inappropriate treatment of both granulomatous disease and malignancy. This problem is reviewed and exemplified by a number of typical case histories. A systematic diagnostic approach must integrate anamnestic, clinical, histomorphological, immunohistological and laboratory information. In addition, prolonged follow-up may be necessary in order to establish the true nature of a granuloma-associated condition.
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7/18. 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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8/18. sarcoidosis: a unique presentation of dysphagia, myopathy, and photophobia.

    We report the case of a patient who presented with symptoms of dysphagia, muscle weakness, and photophobia. The diagnosis of sarcoidosis was made by the histologic evidence of widespread noncaseating epithelioid cell granulomas in more than one organ (anterior mediastinal node and right quadriceps muscle). The disease was proven to be active by elevated angiotensin-converting enzyme (ACE) level and gallium-67 imaging. Esophageal dysfunction was demonstrated by barium swallow and manometric study. A review of the literature on sarcoidosis involving the esophagus and the muscular system is presented. Dysphagia and acute symptomatic myopathy are rare presentations of sarcoidosis. The combination of symptoms is unique, and clearly demonstrates the protean multisystemic nature of sarcoidosis. patients presenting with dysphagia and myopathy should be investigated for sarcoid granulomas in these organs for appropriate treatment.
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9/18. Lymphocytic hypophysitis and pulmonary sarcoidosis. Report of a case.

    Lymphocytic hypophysitis is one of the causes of hypopituitarism in the postpartum period. Some of these patients have been reported to have other organ-specific autoimmune diseases, and the disease is considered to be autoimmune in nature. The authors describe a unique case of lymphocytic hypophysitis in a young woman. She had hemianopia that developed three days after delivery. Computed tomography scans revealed an intrasellar mass lesion. Secretions of adrenocorticotropic hormone and growth hormone were decreased. Although no organ-specific autoantibodies were detected in her serum, OKT4-positive helper T lymphocytes were increased in peripheral blood. Pituitary biopsy at craniotomy showed typical features of lymphocytic hypophysitis. Pulmonary and eye sarcoidosis developed 11 months after the delivery. This is, to the authors' knowledge, the first case of lymphocytic hypophysitis associated with sarcoidosis in other organs.
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10/18. Subdural mass lesion secondary to sarcoid granuloma MR and CT findings and differential diagnosis.

    A patient with surgical and neuropathologically confirmed subdural sarcoid granuloma was evaluated using angiography, contrast-enhanced CT and MR. MR images were obtained on a superconducting magnet with T1, intermediate and multi-echo T2 weighted sequences. review of the 2 prior cases of subdural sarcoid granuloma from the literature evaluated with MR confirm the variable nature of signal intensities of the lesion. MR was most useful in anatomically evaluating the lesion and planning neurosurgical intervention but both CT and MR alone, in this case, did not definitely obviate the other differential diagnosis including meningioma en-plaque, lymphoma, or metastasis.
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