Connective tissue disorder

Finally, benzpiperylon or l-(l-methyl-4-piperidyl)-3-phenyl-4-benzyl-2- (or 3-) pyrazolin-5-one has been utilized by Sandoz as an investigational agent in connective tissue disorders (B-76MI40404). 

The definition of an overlap syndrome dictates that the criteria for diagnosis of both disorders (in the present context, of PM/DM and of some other connective tissue disorder), are fulfilled. It is not unexpected that those syndromes which overlap with PM/DM are also either known autoimmune conditions or ones in which an autoimmune basis is strongly suspected. The association of these disorders with PM/DM syndromes may not materially alter the basic histopathological featmes expected in PM/DM but some differences may be identifiable. The disorders most frequently associated with an overlap syndrome are rheumatoid arthritis, systemic lupus erythematosis, scleroderma, and mixed connective tissue disease. 

Metabolic defects Connective tissue disorders Environmental factors Diet... 

R3. Robert, L., Mombelloni, P., and Crosti, P., Studies on serum haptoglobin in experimental connective tissue disorders. Proc. Soc. Exptl. Biol. Med. 107, 499 (1961). 

Defects in collagen synthesis, structure, or assembly Into fibers are the principal basis for a group of connective tissue disorders called Ehlers-Danlos syndrome (EDS). 

Marfan syndrome is a connective tissue disorder with manifestations in many organs, but especially the skeleton, blood vessels, eyes, and lungs. 

L3. Liu, W. O., Oefner, P. J., et al. Denaturing HPLC-identified novel FBNl mutations, polymorphisms, and sequence variants in Marfan syndrome and related connective tissue disorders. Genet. Test. 1(4), I il-lAl (1997). 

Persikov, A. V., Pillitteri, R. J., Amin, P., Schwarze, U., Byers, P. H., and Brodsky, B. (2004). Stability related bias in residues replacing glycines within the collagen triple helix (Gly-Xaa-Yaa) in inherited connective tissue disorders. Hum. Mut. 24, 330-337. 

Willow is reputed to have anti-inflammatory, antirheumatic, antipyretic, antihidrotic, analgesic, antiseptic, and astringent properties. Traditionally, it is used for muscular and arthrodial rheumatism with inflammation and pain, influenza, respiratory catarrh, gouty arthritis, and other systemic connective tissue disorders characterized by inflammatory changes. 

M15. Meltzer, M., and Franklin, E. C., Cryoglobulins, rheumatoid factors, and connective tissue disorders. Arthritis Rheum. 10, 489-492 (1967). 

When severe psychosis following treatment with chloroquine and hydroxychloroquine occurs it is usually during treatment for malaria, but it can follow treatment for connective tissue disorders. Hallucinations have been reported after hydroxychloroquine treatment for erosive lichen planus (152). 

Several inherited connective tissue disorders predispose to arterial dissection and other vascular abnormalities, including aneurysms and vasoocclusive disease. 

Schievink WI (2001). Spontaneous dissection of the carotid and vertebral arteries. New England Journal of Medicine 344 898-906 Schievink WI, Michels VV, Piepgras DG (1994). Neurovascular manifestations of heritable connective tissue disorders. A review. Stroke 25 889-903... 

The clinical symptoms of classical Menkes disease can be traced back to developmentaUy important copper enzymes such as lysyl oxidase, tyrosinase (see Copper Hemocyanin/Tyrosinase Models), cytochrome c oxidase (see Cytochrome Oxidase), dopamine -hydroxylase, superoxide dismutase, and amine oxidase (see Superoxide Dismutase). Lysyl oxidase is needed for the cross-linking of connective tissue a deficiency in this enzyme causes weakened connective tissue and connective tissue disorder such as arterial ruptures as observed in these patients. Low levels of cytochrome c oxidase cause temperature instability and the absence of tyrosinase explains the hair depigmentation observed in affected individuals. ... 

In FES it is thought that sleeping with an everted eyelid causes the pretarsal orbicularis oculi and skin to override the lid margins, which causes the eyelashes to point downward (eyelash ptosis). Because some patients with FES also have keratoconus, an underlying connective tissue disorder may be implicated. 

CONNECTIVE TISSUE DISORDERS (COLLAGEN VASCULAR DISEASES) ... 

The connective tissue disorders comprise a unique family of systemic diseases that have distinctive yet nonspecific systemic manifestations associated with organ involvement. Such diseases as rheumatoid arthritis (RA), rheumatic fever, systemic lupus erythematosus (SEE), scleroderma, and periarteritis nodosa all demonstrate the typical histologic and clinical findings characteristic of this category of diseases. 

Hydroxychloroquine is less effective than other DMARDs but it is also less toxic it is best used for for arthralgias associated with connective tissue disorders (e.g. SLE) and achieves a useful response in about 50% of patients after 4 weeks. For rheumatoid arthritis hydroxychloroquine is best combined with another DMARD. 

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