Rheumatology Book

http://www.fpnotebook.com/

Rheumatoid FactorAka: RF

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  1. Normal
    1. Negative
  2. Mechanism
    1. IgM (rarely IgG) Antibody
      1. Binds IgG constant region (crystallizable fragment)
    2. Serum Detection
      1. Latex fixation
        1. Agglutination of latex particles
        2. Latex coated with human IgG
      2. Sheep Cell Agglutination assay indications
        1. Negative latex fixation despite high suspicion
  3. Causes of Rheumatoid factor (RF) presence
    1. Rheumatoid Factor present in normal persons
      1. Very Low titers (<1:80) present in many patients
      2. Normally present in 1-5% of general population
      3. Incidence of RF increases with age
      4. Incidence of RF in ages over 65 years: 10%
    2. Rheumatoid Arthritis
      1. Prognosis worse with high titer at onset
      2. RF Incidence increases with duration of disease
        1. Three months: 33%
        2. Six months: 45%
        3. One year: 75%
        4. Eighteen months: 90%
        5. Up to 20% of RA patients remain seronegative
    3. Other Autoimmune disease
      1. Sjogren's Syndrome (75 to 95%)
      2. Systemic Lupus Erythematosus (15 to 35%)
      3. Scleroderma (20 to 30%)
      4. Polymyositis or Dermatomyositis (10%)
      5. Cryoglobulinemia (40 to 100%)
      6. Mixed connective tissue disease (50 to 60%)
    4. Infections
      1. Bacterial endocarditis
      2. Osteomyelitis
      3. Tuberculosis
      4. Syphilis
      5. Hepatitis (Acute and Chronic)
      6. Mononucleosis
      7. Infection following vaccination
    5. Other Conditions
      1. Diffuse interstitial pulmonary fibrosis
      2. Hepatic Cirrhosis
      3. Primary biliary Cirrhosis
      4. Sarcoidosis
  4. Conditions not associated with elevated RF
    1. Osteoarthritis
    2. Ankylosing Spondylitis
    3. Gout
    4. Chondrocalcinosis
    5. Suppurative Arthritis
    6. Psoriatic Arthritis
    7. Enteropathic Arthritis
    8. Reiter's Syndrome
  5. References
    1. Shmerling (1991) Am J Med 91:528

Fanconi Syndrome (C0015624)

Definition (MSH)A hereditary or acquired form of generalized dysfunction of the PROXIMAL KIDNEY TUBULE without primary involvement of the KIDNEY GLOMERULUS. It is usually characterized by the tubular wasting of nutrients and salts (GLUCOSE; AMINO ACIDS; PHOSPHATES; and BICARBONATES) resulting in HYPOKALEMIA; ACIDOSIS; HYPERCALCIURIA; and PROTEINURIA.
Definition (CSP)constellation of clinical and laboratory manifestations produced by generalized proximal tubular insufficiency in the presence of normal, or nearly normal, glomerular filtration.
ConceptsDisease or Syndrome (T047)
ICD9270.0
MSHD005198
English'De Toni-Fanconi-Debre' syndrome, Congenital Fanconi syndrome, CTNS, De Toni Debre Fanconi Syndrome, de Toni-Debre-Fanconi syndrome, De Toni-Fanconi syndrome, deToni Fanconi syndrome, Fanconi Renotubular Syndrome, Fanconi Syndrome, Fanconi-de Toni syndrome, Fanconi-de Toni-Debre syndrome, Fanconi-de-Toni syndrome, FRTS, Infantile nephropathic cystinosis, Lignac Fanconi Syndrome, Lignac-Fanconi syndrome, Nephropathic cystinosis, Primary Fanconi syndrome, Proximal Renal Tubular Dysfunction, Renal Fanconi Syndrome, RFS
Spanishcistinosis nefropatica, cistinosis nefropatica infantil, sindrome de De Toni - Fanconi, sindrome de Fanconi, sindrome de Fanconi congenito, sindrome de Fanconi primario, sindrome de Fanconi-de Toni-Debre, sindrome de Fanconi-Toro, sindrome de Lignac - Fanconi, sindrome de Lignac-Fanconi
Parent ConceptsAmino Acid Metabolism, Inborn Errors (C0002514), Syndrome (C0039082), inborn biological transport disorder (C0596765), Kidney Diseases (C0022658), Renal Tubular Transport, Inborn Errors (C0035091), Hereditary Diseases (C0019247), Cystinosis (C0010690), Fanconi Syndrome (C0015624), Nephritis, Interstitial (C0027707), Renal tubular disorder (C0151747), Amino acid transport disorder (C0268641), Connective tissue hereditary disorder (C0410787), Congenital connective tissue disorder (C0852085), Duplicate concept (C1274013), Hereditary disorder of the urinary system (C1285467), Ambiguous concept (C1274012)
SourcesAOD, CSP, DXP, MSH, MTH, MTHICD9, NCI, NDFRT, OMIM, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)



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