Relapsing polychondritis: bone marrow and circular fibrous nodules in the aorta

Article Abstract:

Relapsing polychondritis is a degenerative disease of cartilage characterized by arthritis and involvement of the nose, ears, joints, bronchi (lung airways) and trachea (windpipe). Death may result from repeated infections following collapse of the bronchial walls. A case is reported of a 40-year-old woman with relapsing polychondritis plus an unusual involvement of the aorta. She sought medical attention for episodic swelling of the face and tongue and for conjunctivitis associated with certain foods. No allergens were identified. In subsequent years she developed inflammation of the iris, deafness, and swollen and painful ears. Facial swelling then recurred. As a collagen disease was suspected, she was given steroid therapy, to which she initially responded. During the next 10 years, she developed skin rashes and papules (pimples) which were inflammatory, but no diagnosis was made. The patient gradually developed muscle aching and weakness and heart dysfunction. Twenty-three years after the initial medical examination, she had left-sided muscle weakness, from which she recovered. Subsequent exams showed further heart problems and calcification of the aortic valve, which controls blood flow from the heart into the aorta, and the aorta itself. Aortic dysfunction is frequently related to rheumatic fever or syphilis, but history and tests were negative for these. The patient died from heart failure. Autopsy showed that the aorta and aortic valve were heavily calcified and contained authentic bone cells, including marrow. Fibrotic tissue, deposited in the walls of the aorta, contained nodules, some of which were ''clock face,'' having fibrotic strands radiating outward from the center. This is a case of relapsing polychondritis which was unusual in that diagnosis was not made until autopsy and it featured involvement of the aorta and an unusual pattern of fibrous tissue deposition. (Consumer Summary produced by Reliance Medical Information, Inc.)

Heart diseases

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Late BCNU lung: a light and ultrastructural study on the delayed effect of BCNU on the lung parenchyma

Article Abstract:

Many of the drugs that are used to treat cancer are nonselective in the sense that they not only destroy the cancerous tissue but they also destroy healthy tissue. Therefore, many anti-cancer drugs can produce undesirable toxic effects. Carmustine, also called BCNU, is a drug that was commonly used to treat brain tumors. However, this drug is very toxic to the lungs. It can cause pulmonary fibrosis, in which dense tissue, similar to scar tissue, develops in the lungs. This reduces the elasticity of the lungs and makes it difficult to breathe. Previous studies have reported that the younger the patient and the higher the dose of BCNU, the greater the chance of developing pulmonary fibrosis. It is estimated that BCNU causes lung damage in 20 to 30 percent of the patients treated with this drug. In most cases, symptoms of lung damage begin to appear within several months to three years after treatment with BCNU has been completed. It is rare for symptoms of lung damage to begin three or more years after treatment with BCNU. This article describes the case reports of eight patients who developed pulmonary fibrosis between 12 and 17 years after treatment with BCNU for brain tumors. These findings indicate that patients who are treated with BCNU should be followed closely for many years after treatment with BCNU has been completed. (Consumer Summary produced by Reliance Medical Information, Inc.)

Causes of, Complications and side effects, Lungs, Lung, Chemotherapy, Brain tumors, Pulmonary fibrosis, Carmustine

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Relapsing polychondritis

Article Abstract:

The outlook for survival from relapsing polychondritis may be much better than previously believed if awareness of the symptoms allows earlier diagnosis. Because this disorder, most frequently appearing as intermittent episodes of inflammation of cartilage, is not easily diagnosed, the average time elapsed between a patient seeking help and a physician making a conclusive diagnosis has been 2.9 years. With awareness, earlier diagnosis and treatment with prednisone and methotrexate will be successful.

Diagnosis, Connective tissue diseases

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