Disease Information for Hereditary inflammatory periodic fevers group

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Clinical Manifestations
Signs & Symptoms
Fever Non Infection
Abdominal Pain
Acute abdomen with no signs
Recurrent Abdominal Pain
Cervical adenopathy
Lymphadenopathy
Muscle Pain
Myalgias
Pain in Extremities/Melalgias
Arthralgias Polyarthralgias
Joint Pains
Sore throat/Pharyngitis
Cyclic Periodic Recurrent Fevers
Episodic symptoms/events
Fever
Fever and Normal Sed Rate
Fever, intermittant/recurrent
High body temperature
Pain without findings
Periodic fever
Quotidian Fever
Total body pain/truncal/extremities
Eye Pains, Bilateral
Clinical Presentation & Variations
Presentation/Arthritis Fever Rash
Disease Progression
Course/Periodic Episodic
Demographics & Risk Factors
Ethnic or Racial Factors
Northern European population/extraction
Laboratory Tests
Abnormal Lab Findings (Non Measured)
Acute phase reactants increased (Lab)
Abnormal Lab Findings - Increased
Amyloid A serum (Lab)
C-reactive protein (Lab)
Serum Amyloid A (SSA)
Diagnostic Test Results
Pathology
BX/Liver biopsy/Amyloid Kupfer cells/basement membrane
BX/Renal Amyloid deposit
Associated Diseases & Rule outs
Associated Disease & Complications
Amyloidosis, hepatosplenic
Amyloidosis, renal
Amyloidosis, secondary
Amyloidosis, systemic
Aphthous stomatitis Aphthous oral lesions
Disease Mechanism & Classification
Class
CLASS/Acronymic term disorder (ex)
CLASS/Pediatric disorders (ex)
Pathophysiology
Pathophysiology/Autoinflammatory (non-immune)
Pathophysiology/Auto-Inflammatory Disorder
Process
PROCESS/Episodic disorder (ex)
PROCESS/Hereditary inflammatory period fevers
PROCESS/Hereditofamilial (category)
Treatment
Drug Therapy - Indication
RX/Colchicine (Generic)
RX/Corticosteroid (Cortisone)
RX/Rilonacept IL-1 Blocker (Arcalyst)
Definition

very rare four types of non mediterreanean periodic fever with inflammatory periodic fever; non immune inflammatory response not immune response but all go to amyloidosis with type A amyloid so treatment is indicated with daily colchicine; examples IL1 receptor; TNF release; Tumor necrosis factor receptor-associated periodic syndrome (TRAPS); Tumor necrosis factor receptor-associated periodic syndrome (TRAPS) is an autosomal dominant inherited condition of periodic fever and pain; Most patients are of northern European descent; The attacks manifest as fever and pain in the joints, abdomen, muscles, skin, or eyes, with variations across patients; An acute-phase response occurs during the attacks; Patients with TRAPS are at risk for AA amyloidosis, the most common targets being the kidneys and liver; TRAPS is the first condition for which naturally occurring mutations in a TNF receptor were found; the mutations affect the soluble TNFRSF1A gene in the 12p13 region; In some patients, the pathogenesis involves defective TNFRSF1A shedding from cell membranes in response to a given stimulus; other inherited periodic febrile conditions are familial Mediterranean fever, Muckle-Wells syndrome, cold urticaria, and hyper-IgD syndrome; TRAPS Colchicine may prevent expected Amyloidosis but neither relieves nor prevents the attacks, whereas oral glucocorticoid therapy is effective when used in dosages greater than 20 mg/day [traps]

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Harrison's Online (accessmedicine)
NEJM (The New England Journal of Medicine)
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