Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0042384 (vasculitis)
 20,525 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A patient with sensorimotor mononeuritis multiplex had a type II cryoglobulin with an IgM kappa M-protein that appeared to contain monoclonal anti-MAG antibodies of the same isotype. A sural nerve biopsy demonstrated necrotizing arteritis and features of both axonal degeneration and demyelination. IgM kappa and C3 deposits were present on the myelin sheath of some residual nerve fibers. The findings suggest that the anti-MAG antibodies contributed to the myelin damage, while cryoprecipitates may have caused the vasculitis and axonal degeneration.
 ...
PMID:Vasculitic neuropathy in a patient with cryoglobulinemia and anti-MAG IGM monoclonal gammopathy. 137 93

Monoclonal gammopathies are frequently associated with peripheral neuropathies of which clinical, electrophysiological, pathological and possibly pathogenetical aspects are heterogeneous. Nevertheless some clinico-biological entities, which account for the majority of cases, have been recently recognized: 1) The IgM neuropathy is a chronic demyelinating sensori-motor polyneuropathy with tremor and ataxia as prominent features. It can be either associated with MGUS or Waldenstrom macroglobulinemia. The light chain of the gammopathy is kappa in a majority of cases. Numerous reports have demonstrated specific antibody activities supported by the M-protein and directed against various peripheral nerve antigens, usually myelin components such as the myelin associated glycoprotein (MAG). The ultrastructural evidence of widely spaced myelin is suggestive of the diagnosis but is not consistent. Treatment directed towards the gammopathy is occasionally associated with improvement of the symptoms. 2) The neuropathy of the osteosclerotic myelomas and solitary plasmacytomas present as a chronic sensori-motor polyradiculoneuropathy with conspicuous demyelination and may be associated with one or more of the systemic clinical features of the Crow-Fukase or POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, M-protein, Skin changes). The POEMS syndrome may also be associated with "benign" monoclonal or even polyclonal dysproteinemias. The M-proteins are almost all IgG or IgA with lambda light chains. There are some relations between POEMS syndrome and Castleman's disease. The pathogenesis of both disorders remains obscure. Treatment is most favorable in case of solitary plasmacytomas, which may be completely removed. 3) The neuropathy observed in patients with primary AL amyloidosis or amylosis associated with malignant plasma-cell dyscrasias is rare. Sensory deficit and autonomic dysfunction are related to a prominent involvement of small myelinated and unmyelinated fibers. A clinical and/or electro-physiological carpal tunnel syndrome is frequent. In a majority of cases the light chain of the M-protein is lambda. Amyloid deposits are observed on nerve biopsy. Treatment is inefficient. 4) The neuropathy associated with cryoglobulinemias may be asymmetric, painful, cryosensitive and associated with cutaneous purpura and neuromuscular vasculitis. In fact, in a majority of cases the symptoms are less suggestive raising the problem of an incidental laboratory finding. 5) A motoneuron disease-like syndrome may develop in patients with various types of monoclonal gammopathies.(ABSTRACT TRUNCATED AT 400 WORDS)
 ...
PMID:[Dysglobulinemic neuropathies]. 284 76

The POEMS syndrome is a synopsis of different symptoms such as polyneuropathy, organomegaly, endocrine disturbances, M-protein and skin changes. The leading symptoms are neuropathy and the skin symptoms. Additionally, a monoclonal light chain gammopathy is often found. The administration of immunosuppressive drugs yields a substantial improvement in some cases. We report here about a 72 year old lady who fell ill with a rapidly progressive neuropathy accompanied by hyperpigmentation and a morphea-like induration of the skin. A biopsy of the sural nerve showed a demyelinating axonal neuropathy and a focal vasculitis. Isoelectric focussing revealed oligoclonal bands in cerebrospinal fluid and serum. The cortisol serum level was very low and there were signs of a latent diabetes mellitus. These clinical features correspond to the POEMS syndrome. The prescription of initially 1 mg and later 0.5 mg prednisone improved the patient's condition dramatically.
 ...
PMID:[POEMS syndrome: a contribution to differential diagnosis of polyneuropathy]. 850 14

A 68-year-old-male who was diagnosed as having rheumatoid arthritis (RA) 7 years previously was admitted the Chiba Social Insurance Hospital due to general fatigue, spiking fever, and appetite loss. Blood tests showed extremely high levels of C-reactive protein (CRP, 318.5 mg/dL), and hypergammapathy (IgG 3228 mg/dL, IgA 905 mg/dL, IgM 2537 mg/dL) and high titers of rheumatoid factor (RAPA 40960X). He was diagnosed as having RA with vasculitis, according to interstitial pneumonitis, cutaneous nodules and polyneuropathy. Prednisolone (30 mg/day) was prescribed, however, myeloperoxidase-antineutrophil cytoplasmic antibody proved to be positive (86EU) and cyclophosphamide (50 mg/day) was added one week later. Additionally, IgM K-chain M-protein was revealed and the differentiation between auto-immune and hematologic diseases was required for further drug prescriptions. Therefore, double filtration plasmapheresis (DFPP) was initiated weekly. Hematologic diseases were negated and the hypergammapathy was improved. C-reactive protein and MPO-ANCA decreased to the normal level after three sessions (IgG 1064 mg/dL, IgA 331 mg/dL, IgM 94 mg/dL, CRP 0.04 mg/dL) and the patients symptoms improved. Prednisolone was tapered and he was discharged. It was suggested that the case presented here was quite rare, having an extremely high level of CRP which was successfully managed by DFPP.
 ...
PMID:Double filtration plasmapheresis for the treatment of a rheumatoid arthritis patient with extremely high level of c-reactive protein. 1566 36