Pathology Associates Of Lexington, P.A.

Classification of Autoimmune Disease

This is by no means a complete listing, and whether certain diseases are strictly, primarily, or partially autoimmune is always arguable. Most of those below either are associated with host serum antibody elevations, immunoprotein deposits in host tissue, prominently elevated numbers of tissue eosinophiles, or increased infiltration of target tissues by lymphocytes. By these host reactions, target tissues undergo injury which may or may not be stoppable and from which they may or may not be able to recover. [about autoimmune disease]

Endocrine organ specific autoimmune diseases:
- adrenal gland: Addison's disease
- broncho-pulmonary airways: allergic asthma
- ovaries: premature ovarian failure
- pancreatic islets of Langerhans:
  - acanthosis nigricans associated insulin-resistant diabetes
  - ataxia telangiectasia associated insulin-resistant diabetes
- parathyroid glands: hypoparathyroidism
- thyroid gland: Hashimoto's autoimmune thyroiditis; Graves disease; primary myxedema

There is a definite interrelationship between the various endocrine organs and pernicious anemia with atrophic gastritis. Patients with autoimmune thyroiditis have a 10% incidence of pernicious anemia, compared with 0.2% in the general population. The incidence of antiparietal cell antibodies in patients with autoimmune thyroiditis is 30%, and 50% of patients with pernicious anemia have thyroid antibodies. The antibody reactivity in this group is organ specific. The lesions are localized to the specific organ, as in Hashimoto's thyroiditis.

Systemic autoimmune diseases (the "lupus group"):
- Lupus erythematosus
- Mixed connective tissue disease
- Drug-induced lupus
- Sjogren's syndrome
- Scleroderma/CREST syndrome
- Dermatomyositis/polymyositis and here
- Rheumatoid arthritis

Of the many autoimmune disease, there are remarkable overlaps at each end of the spectrum. For example, thyroid autoantibodies occur with high frequency in patients with pernicious anemia who have stomach autoimmunity. These patients have a higher incidence of thyroid autoimmunity than the normal population. The cluster of rheumatologic diseases at the other end of the spectrum also shows significant overlap. Features of rheumatoid arthritis may be associated with the clinical picture of SLE. In these diseases, immune complexes are deposited systemically in the kidneys, joints, and skin. This second group of diseases induces autoantibodies directed against nuclear and cytoplasmic antigens that are neither organ nor species specific.

Organ system autoimmune diseases:
- Liver:
  - autoimmune chronic active hepatitis
  - primary biliary cirrhosis
- Muscle:
  - myasthenia gravis
- Blood:
  - autoimmune hemolytic anemia
  - autoimmune leukopenia
  - autoimmune thrombocytopenia
- Gastrointestinal:
  - atrophic gastritis of autoimmune type (leads to pernicious anemia)
  - ulcerative colitis (IBD-UC)
  - Crohn's disease (IBD-C)
  - food protein intolerance enteropathies (such as gluten sensitive enteropathy [GSE, celiac disease, celiac sprue]
- Nervous system:
  - demyelinating diseases (eg, multiple sclerosis), amyotrophic lateral sclerosis, sensor motor neuropathy
  - myasthenic syndromes
    1. occular myasthenia
    2. myasthenia gravis
    3. Lambert-Easton paraneoplastic myasthenic syndrome
  1. stiff person (stiff man) syndrome: antibody mediated chronic near total body cramp.
  - Guillain-Barre syndrome
  - Progressive multifocal leukoencephalopathy
  - paraneoplastic syndromes
  - transverse myelitis
  - Bickerstaff's postinfectious brainstem encephalitis
- Kidney:
  - Goodpasture's syndrome
  - Immune complex glomerulonephritis (many different types, including poststreptococcal, cryoglobulin, DNA-anti-DNA in SLE, etc)
- Skin:
  - Pemphigus vulgaris
  - Bullous pemphigoid
  - Cicatricial pem
  - Dermatitis herpetiformis
  - Vitiligo
Other autoimmune diseases:
- Wegener's granulomatosis
- Spontaneous male infertility

References:

Nakamura RM, et. al., Clinical and Laboratory Evaluation of Human Autoimmune Disease, ASCP Press 2002, 450 pages...p. 101 (EBS's office).

(posted 31 August 2003; latest update 30 October 2010)