Sarcoidosis

A 39-year-old woman who suffered from migraine is described. She developed analgesic nephropathy due to phenacetin, and constrictive pericarditis, probably due to methysergide treatment.     

Sarcoidosis on ritual scarification

Telomeric associations were determined in bone marrow preparations from a patient with B-cell prolymphocytic leukaemia and a clonal isochromosome of the long arm of chromosome 17. Thirteen associations involved 16 chromosome arms, with preferential involvement of the short arm of chromosome 19 and the long arm of 17.     

Sarcoidosis of the heart

Myocardial involvement in sarcoidosis occurs in about 27% of patients. Clinical manifestations include ventricular arrhythmias, conduction abnormalities, and sudden death. Diagnosis requires clinical suspicion and may be supported by electrocardiography, echocardiography, thallium imaging, gallium-67 scanning, and myocardial biopsy. Steroids seem to be beneficial in the treatment of myocardial sarcoidosis. Those who do not respond to medical treatment may benefit from cardiac transplantation.     

Sarcoidosis in identical twins

Identical twin sisters who developed sarcoidosis at an interval of one year are reported. Case 1. (elder sister) A 22-year-old female was admitted to our hospital for examination of cervical lymph node swelling. Her chest roentgenogram showed bilateral hilar lymphadenopathy and the diagnosis was confirmed histologically by scalene node biopsy. Case 2. (younger sister) In 1987, at age 23, a routine chest roentgenogram showed bilateral hilar lymphadenopathy and the diagnosis was confirmed histologically by transbronchial lung biopsy. The occurrence of sarcoidosis in identical twins suggests that a genetic factor might be involved in the pathogenesis.     

Sarcoidosis with cardiac involvement

Patients with significant cardiac sarcoidosis are at increased risk of sudden death from ventricular dysrhythmias or conduction disturbances. We report a patient in whom there was radiographic and histologic evidence of systemic sarcoidosis; though histologic confirmation of involvement of heart by sarcoidosis is lacking, the clinical manifestations, radionuclide image findings, rhythm disturbances, and the response to steroid therapy are strong evidence in favor of myocardial involvement by the granulomatous process.     

Sarcoidosis presenting as obstructive jaundice

Sarcoidosis is an uncommon disorder characterized by a multi-systemic granulomatous disease of undetermined etiology and pathogenesis. The diagnosis is established by the presence of a compatible clinical illness and by histologic demonstration of noncaseating epithelioid cell granulomas in the affected organs. Accurate diagnosis requires a thorough evaluation to exclude infectious and neoplastic diseases that can mimic sarcoidosis. Although all organs and systems can be affected, the lungs and intrathoracic lymph glands are the most common sites of involvement. We describe an unusual case of extrapulmonary sarcoidosis presenting as obstructive jaundice.     

Sarcoidosis: a pediatric perspective

Childhood sarcoidosis is a rare multisystemic granulomatous disease of unknown etiology. The clinical presentation can vary greatly depending upon the organs involved. Two distinct forms of sarcoidosis exist in children. Older children usually present with a multisystem disease similar to the adult manifestation, with frequent hilar lymphadenopathy and pulmonary infiltration. Early-onset childhood sarcoidosis is a unique form of the disease characterized by the triad of rash, uveitis, and arthritis in patients presenting before age 4 years. The diagnosis of sarcoidosis is confirmed by demonstrating a typical noncaseating granuloma on a biopsy specimen. The current therapy of choice for childhood sarcoidosis with multisystem involvement is corticosteroids. Methotrexate given orally in low doses is effective and safe and has steroid-sparing properties.     

Sarcoidosis: 25-year clinical follow-up

Based on the follow-up of 1,600 patients with sarcoidosis from 1971 to 1996, the authors analyzed the value of various methods for identifying the disease, the frequency and nature of misdiagnoses. They showed the efficiency of basic treatments in patients with sarcoidosis: corticosteroidal hormones, nonhormonal antiinflammatory drugs, plasmapheresis, and physiotherapy. Recurrencies and progression were seen in 23.85% of patients. The likely causes of recurrent sarcoidosis are considered. It is concluded that recurrencies are one of the important problems of modern sarcoidosis.     

Sarcoidosis: a primary care review

Sarcoidosis is a multisystemic disorder of unknown etiology that most commonly affects adults between 20 and 40 years of age. Patients with sarcoidosis frequently present with bilateral hilar lymphadenopathy and pulmonary infiltration, and often with ocular and skin lesions. The diagnosis is established when clinical and radiographic findings are supported by histologic evidence of non-caseating epithelioid cell granulomas found on tissue biopsy. Diagnosis of sarcoidosis requires exclusion of other causes of granuloma formation. Sarcoidosis is also characterized by distinctive laboratory abnormalities, including hyperglobulinemia, an elevated serum angiotensin converting enzyme level, evidence of depressed cellular immunity manifested by cutaneous anergy and, occasionally, hypercalcemia and hypercalciuria. Glucocorticoids remain the mainstay of therapy when treatment is required, although other anti-inflammatory agents are being used increasingly often.     

Sarcoidosis manifesting as uveitis and menometrorrhagia

PURPOSE: To report a rare systemic manifestation of sarcoidosis identified in a 47-year-old white woman while she was undergoing evaluation for bilateral recurrent uveitis. METHODS: The patient underwent clinical and laboratory evaluation for bilateral recurrent uveitis including serologic and radiologic testing, a gallium scan, and an endometrial biopsy. RESULTS: Although the serologic tests and chest x-ray were normal, the gallium scan was consistent with sarcoidosis, and the endometrial biopsy provided a tissue diagnosis. CONCLUSION: Sarcoidosis involving the female reproductive tract is rare. A thorough review of systems is crucial in the evaluation of any patient with recurrent uveitis.     

Sarcoidosis with nasal obstruction and septal perforation

Although sarcoidosis is predominantly a pulmonary disorder, it often produces disease in the head and neck. For this reason, otolaryngologists should be comfortable in evaluating and treating the disease. The diagnosis of sarcoidosis often can be made with a biopsy of conjunctival or oral mucosa, even when disease manifestations are not apparent in those parts of the body. In a case of sarcoidosis involving the nose, a 54-year-old black man had a proliferation of small lumps on the tip of his nose. For many years, he had had daily epistaxis, anosmia and severe difficulty breathing through this nose. The bridge of his nose was deformed, and he had a large anterior septal perforation. Physical examination revealed palpable bilateral cervical lymphadenopathy. Laboratory test results were, for the most part, normal; however, fine-needle aspiration of one of the cervical lymph nodes showed multiple epithelioid granulomas with interspersed lymphocyte, and an incisional biopsy of the nasal tissue was highly suggestive of sarcoidosis. Unenhanced computed tomography also revealed widespread nasal inflammation and an abnormal soft tissue mass anterior to the maxilla consistent with chronic osteomyelitis. The patient's condition dramatically improved following treatment with oral prednisone; however, he considered undergoing reconstructive surgery for the noticeable nasal septal deformity.     

Sarcoidosis of the paranasal sinuses treated with hydroxychloroquine

A case of sarcoidosis of the paranasal sinuses is reported. Biopsies of the sinus mucosa showed typical noncaseating granulomas. Hydroxychloroquine, which is known to be active on the cutaneous form of sarcoidosis, was used here with success and is proposed as an effective alternative to high-dose systemic steroids.