Biomaterial Database

[Long-term study of diffuse connective tissue disease after methylprednisolone pulsed therapy]. 1991

Z Hrncír, and P Bradna, and H Nozicková, and M Tichý

II. interní klinika lékarské fakulty Univerzity Karlovy, Hradec Králové.

Pulsed i.v. treatment with methylprednisolone (MP) infusions à 1 g 3-5 times on alternate days was administered in 22 diffuse connective tissue diseases: 13X systemic lupus erythematosus (SLE), 1X Sharp's syndrome and 8X polymyositis/dermatomyositis (PM/DM). In all instances active disease was involved with serious organ manifestations; 17 patients had no aimed treatment so far, in 5 previous treatment had failed. A total of 101 pulsed MP was administered. Initial MP treatment was always successful. The patients were then followed up during oral maintenance treatment for 3-60 months (mean 27 months) in a stage of persistent, inconstant or partial remission. In six patients treatment was repeated 8X after reactivation. The response was always favourable and did not differ from that to initial MP treatment, except for one female patient who died from progression of PM/DM (one female patient died from the sequels of oncological disease associated with SLE).

UI	MeSH Term	Description	Entries
D008180	Lupus Erythematosus, Systemic	A chronic, relapsing, inflammatory, and often febrile multisystemic disorder of connective tissue, characterized principally by involvement of the skin, joints, kidneys, and serosal membranes. It is of unknown etiology, but is thought to represent a failure of the regulatory mechanisms of the autoimmune system. The disease is marked by a wide range of system dysfunctions, an elevated erythrocyte sedimentation rate, and the formation of LE cells in the blood or bone marrow.	Libman-Sacks Disease,Lupus Erythematosus Disseminatus,Systemic Lupus Erythematosus,Disease, Libman-Sacks,Libman Sacks Disease
D008297	Male		Males
D008775	Methylprednisolone	A PREDNISOLONE derivative with similar anti-inflammatory action.	6-Methylprednisolone,Medrol,Metipred,Urbason,6 Methylprednisolone
D008875	Middle Aged	An adult aged 45 - 64 years.	Middle Age
D008947	Mixed Connective Tissue Disease	A syndrome with overlapping clinical features of systemic lupus erythematosus, scleroderma, polymyositis, and Raynaud's phenomenon. The disease is differentially characterized by high serum titers of antibodies to ribonuclease-sensitive extractable (saline soluble) nuclear antigen and a "speckled" epidermal nuclear staining pattern on direct immunofluorescence.	Connective Tissue Disease, Mixed,Sharp Syndrome,MCTD,Syndrome, Sharp
D003240	Connective Tissue Diseases	A heterogeneous group of disorders, some hereditary, others acquired, characterized by abnormal structure or function of one or more of the elements of connective tissue, i.e., collagen, elastin, or the mucopolysaccharides.	Connective Tissue Disease,Disease, Connective Tissue,Diseases, Connective Tissue
D003882	Dermatomyositis	A subacute or chronic inflammatory disease of muscle and skin, marked by proximal muscle weakness and a characteristic skin rash. The illness occurs with approximately equal frequency in children and adults. The skin lesions usually take the form of a purplish rash (or less often an exfoliative dermatitis) involving the nose, cheeks, forehead, upper trunk, and arms. The disease is associated with a complement mediated intramuscular microangiopathy, leading to loss of capillaries, muscle ischemia, muscle-fiber necrosis, and perifascicular atrophy. The childhood form of this disease tends to evolve into a systemic vasculitis. Dermatomyositis may occur in association with malignant neoplasms. (From Adams et al., Principles of Neurology, 6th ed, pp1405-6)	Polymyositis-Dermatomyositis,Dermatomyositis, Adult Type,Dermatomyositis, Childhood Type,Dermatopolymyositis,Juvenile Dermatomyositis,Juvenile Myositis,Adult Type Dermatomyositis,Childhood Type Dermatomyositis,Dermatomyositis, Juvenile,Myositis, Juvenile,Polymyositis Dermatomyositis
D005260	Female		Females
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293	Adolescent	A person 13 to 18 years of age.	Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths