Subject(s)
Antiphospholipid Syndrome/etiology , Calcinosis/etiology , Lupus Erythematosus, Systemic/complications , Splenic Diseases/etiology , Adult , Antibodies, Anti-Idiotypic/blood , Antiphospholipid Syndrome/blood , Antiphospholipid Syndrome/immunology , Calcinosis/diagnostic imaging , Cardiolipins/immunology , Female , Humans , Splenic Diseases/diagnostic imaging , Tomography, X-Ray Computed , beta 2-Glycoprotein I/immunologyABSTRACT
Here we described an autopsy case of intestinal Behcet's disease with sacroiliitis associated with myelodysplastic syndrome (RAEB-t, 8+). Over twenty cases of Behcet's disease associated with myelodysplastic syndrome have been reported in preliterature so far. The majority of them are incomplete type of Behcet's disease having intestinal ulceration. Of those, trisomy 8 is the most common chromosomal abnormality. We reviewed similar cases reported and investigated the association of intestinal Behcet's disease with trisomy 8. The association of sacroiliitis with Behcet's disease is also studied.
Subject(s)
Anemia, Refractory, with Excess of Blasts/complications , Behcet Syndrome/complications , Chromosomes, Human, Pair 8 , Ileal Diseases/complications , Sacrum , Spondylitis/complications , Trisomy , Ulcer/complications , Aged , Anemia, Refractory, with Excess of Blasts/genetics , Humans , MaleABSTRACT
A 36-year-old man was admitted to a hospital with complaints of fever, polyarthralgia and dyspnea. Erythema was observed on his face, extensor surface of the fingers and extremities, and a chest X-ray revealed massive bilateral pleural effusion. He had no sign of myopathy at this point. Pleural fluid was proved to be exudative and contained extremely high levels of hyaluronic acid. He was also complicated with interstitial pneumonitis and was given a pulse therapy with methyl prednisolone followed by daily administration of 55 mg prednisolone (PSL). Twenty days after the commencement of the therapy, pleural effusion decreased but muscle weakness gradually appeared, accompanied by elevation of myogenic enzymes. Myogenic changes on electromyogram, and irregularity of the muscle fibers with slight inflammatory cell infiltrates in a biopsy specimen were demonstrated. He was transferred to our hospital, and a diagnosis of dermatomyositis was made. Later, pleural effusion waxed and waned depending on the dosage of PSL, but no other causative disorder was demonstrated by extensive examinations. This case indicates that the pleuritis could be one of the vasculitic manifestations of dermatomyositis.
