Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0039483 (giant cell arteritis)
 3,204 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Polymyalgia rheumatica is a clinical syndrome which appears after the age of 50 and is characterized by pain and stiffness of proximal muscles, rapid sedimentation rate and a dramatic response to small doses of corticosteroids. The linkage between polymyalgia rheumatica and temporal arteritis is well-known, but the connection with monoclonal gammopathy and lympho-proliferative disorders has rarely been reported. We present a 74-year-old man admitted with the typical clinical picture of polymyalgia rheumatica, which was the presenting symptom of monoclonal gammopathy and subsequently-diagnosed multiple myeloma.
 ...
PMID:[Polymyalgia rheumatica as the presenting symptom of multiple myeloma]. 779 54

We report a patient who presented with clinical symptoms suggesting giant cell arteritis. A diagnosis of AL amyloidosis was made on temporal artery biopsy. Temporal artery involvement is unusual in AL amyloidosis, but its frequency may be underestimated. Another uncommon finding was the presence of an IgD monoclonal gammopathy without diagnostic features of multiple myeloma.
 ...
PMID:Temporal artery involvement revealing AL amyloidosis and IgD monoclonal gammopathy. 883 33

Fever of unknown origin (FUO) is a common clinical diagnostic dilemma. In the elderly, causes of FUO most commonly include malignancy or infection, and less commonly include collagen vascular diseases. Among the collagen vascular diseases causing FUO in the elderly, polymyalgia rheumatica/temporal arteritis, and adult Still's disease (adult juvenile rheumatoid arthritis) are difficult diagnoses to prove. Among the infectious causes of FUO in the elderly are subacute bacterial endocarditis, intra-abdominal abscesses, and extrapulmonary tuberculosis. In the elderly, neoplastic causes of FUO include lymphomas, hepatomas, renal cell carcinomas, and hepatic or central nervous system metastases. Acute leukemias, particularly during "blast" transformation, may present as acute fevers in the absence of infection, but are rare causes of FUO. Preleukemia/myelodysplastic syndromes are exceedingly rare causes of FUO. We present a case of an elderly man who presented with findings that initially suggested adult Still's disease. Prolonged and profound monocytosis provided the key clue to his subsequent diagnosis of preleukemia/myelodysplastic syndrome. In this patient, a positive Naprosyn test result also suggested a neoplastic cause for his FUO. After months of prolonged fevers, myelocytes/metamyelocytes were eventually demonstrated in his peripheral smear during hospital evaluation. These findings, in concert with the persistent monocytosis, highly elevated ferritin levels, polyclonal gammopathy on serum protein electrophoresis, and eventual presence of myelocytes/metamyelocytes on peripheral smear, prompted a bone marrow test that demonstrated blast cells confirming the diagnosis of preleukemia myelodysplastic syndrome as the cause of this patient's FUO.
 ...
PMID:Fever of unknown origin due to preleukemia/myelodysplastic syndrome: the diagnostic importance of monocytosis with elevated serum ferritin levels. 1686

This case report describes a patient who presented with severe anemia, monoclonal gammopathy, a high erythrocyte sedimentation rate and significant weight loss. These features were highly suggestive of multiple myeloma. Bone marrow aspiration was negative for myeloma on two occasions. A positron emission tomography (PET) scan showed extensive 2-flourodeoxy-glucose uptake in the vascular tree consistent with arteritis. A temporal artery biopsy established the diagnosis of giant cell arteritis (GCA). There were no typical symptoms of GCA, such as headache, visual disturbance, or polymyalgia rheumatica. The patient was treated with steroids, which resulted in the resolution of anemia, monoclonal gammapathy, and other symptoms.
 ...
PMID:Giant cell arteritis mimicking multiple myeloma; diagnosed by PET scan. 1719 11

Monoclonal gammopathies complicated by AL amyloidosis can mimic giant cell arteritis (GCA). We hereby present the case of a 63 year old woman in whom symptoms consistent with GCA were the first manifestations of a monoclonal gammopathy of unknown significance (MGUS) associated with amyloidosis. A 63 year old woman was admitted for temporal headache, maseterine claudication, neck and shoulder stiffness. She was recently diagnosed with carpal tunnel syndrome. On physical examination she had prominent temporal arteries, macroglosia and orthostatic hypotension. Muscular strength was normal. She had high ESR and CRP; in this clinical context, GCA was suspected. A gamma spike on serum protein electrophoresis raised the suspicion of monoclonal gammopathy (MG). Immunoelectrophoresis revealed monoclonal bands for IgG and kappa chains. Massive deposits of amyloid and no inflammation were found on temporal artery biopsy. Multiple myeloma and lymphoma were ruled out. A diagnosis of AL amyloidosis complicating MGUS was formulated. She did well on therapy with bortezomib, cyclophosphamide and dexamethasone. Cases published in medical literature reveal amyloidosis mimicking GCA in the setting of established MGUS. As far as we know, this is the first case of MGUS with IgG and kappa chains in which a GCA-like picture induced by amyloidosis was present from the very onset.
 ...
PMID:IgG,kappa monoclonal gammopathy of unknown significance with AL amyloidosis simulating giant cell arteritis. 2846 13