Definition of "Autoimmune disease"

Last modified: 6 hours



Autoimmune disease is damage or a pathologic state arising from an abnormal immune response of the body against substances and tissues normally present in the body. Autoimmunity is presence of self-reactive immune responses (e.g. auto-antibodies, self-reactive T cells), with or WITHOUT damage/pathology resulting from it.

Pathophysiology
  • It can be restricted to certain organs (e.g. in autoimmune thyroitiditis), or involve a particular tissue in different places (e.g. Goodpasture's disease, which may affect the basement membrane in both the lung and kidney)
Classification
  • Heart:
    • Myocarditis
    • Postmyocardial infarction syndrome
    • Postpericardiotomy syndrome
    • Subacute bacterial endocarditis
  • Kidney:
    • Anti-glomerular basement membrane nephritis
    • Interstitial cystitis
    • Lupus nephritis
  • Liver:
    • Autoimmune hepatitis
    • Primary biliary cirrhosis
    • Primary sclerosing cholangitis
  • Lung:
    • Antisynthetase syndrome
  • Skin:
    • Alopecia areata
    • Autoimmune angioedema
    • Autoimmune progesterone dermatitis
    • Autoimmune urticaria
    • Bullous pemphigoid
    • Cicatricial pemphigoid
    • Dermatitis herpetiformis
    • Discoid lupus erythematosus
    • Epidermolysis bullosa acquisita
    • Erythema nodosum
    • Gestational pemphigoid
    • Hidradenitis suppurativa
    • Lichen planus
    • Lichen sclerosus
    • Linear IgA disease
    • Morphea
    • Pemphigus vulgaris
    • Pityriasis lichenoides et varioliformis acuta
    • Mucha-Habermann disease
    • Psoriasis
    • Systemic scleroderma
    • Vitiligo
  • Adrenal gland:
    • Addison's disease
  • Multi-glandular:
    • Autoimmune polyendocrine syndrome
    • Autoimmune polyendocrine syndrome type 3
    • Autoimmune polyendocrine syndrome type 3
  • Pancreas:
    • Autoimmune pancreatitis
    • Diabetes mellitus type 1
  • Thyroid gland:
    • Autoimmune thyroiditis
    • Ord's thyroiditis
    • Graves' disease
  • Reproductive organs:
    • Autoimune oophoritis
    • Endometriosis
    • Autoimmune orchitis
  • Salivary glands:
    • Sjogren's syndrome
  • Digestive system:
    • Autoimmune enteropathy
    • Celiac disease
    • Crohn's disease
    • Microscopic colitis
    • Ulcerative colitis
  • Blood:
    • Antiphospholipid syndrome
    • Aplastic anemia
    • Autoimmune hemolytic anemia
    • Automimune lymphoproliferative syndrome
    • Autoimmune neutropenia
    • Autoimmune thrombocytopenic purpura
    • Cold agglutinin disease
    • Essential mixed cryoglobulinemia
    • Evans syndrome
    • IgG4-related systemic disease
    • Paroxysmal nocturnal hemoglobinuria
    • Pernicious anemia
    • Pure red cell aplasia
    • Thrombocytopenia
  • Connective tissue, systemic, and multi-organ:
    • Adiposis dolorosa
    • Adult-onset Still's disease
    • Ankylosing spondylitis
    • CREST syndrome
    • Drug-induced lupus
    • Enthesitis-related arthritis
    • Eosinophilic fasciitis
    • Felty syndrome
    • Juvenile arthritis
    • Lyme disease (chronic)
    • Mixed connective tissue disease
    • Palindromic rheumatism
    • Parry Romberg syndrome
    • Parsonage-Turner syndrome
    • Psoriatic arthritis
    • Reactive arthritis
    • Relapsing polychondritis
    • Retroperitoneal fibrosis
    • Rheumatic fever
    • Rheumatoid arthritis
    • Sarcoidosis
    • Schnitzler syndrome
    • Systemic lupus erythematosus
    • Undifferentiated connective tissue disease
  • Muscle:
    • Dermatomyositis
    • Fibromyalgia
    • Inclusion body myositis
    • Myositis
    • Myasthenia gravis
    • Neuromyotonia
    • Paraneoplastic cerebellar degeneration
    • Polymyositis
  • Nervous system:
    • Acute disseminated encephalomyelitis
    • Acute motor axonal neuropathy
    • Anti-N-Methyl-D-Aspartate receptor encephalitis
    • Balo concentric sclerosis
    • Bickerstaff's encephalitis
    • Chronic inflammatory demyelinating polyneuropathy
    • Guillain-Barre syndrome
    • Hashimoto's encephalopathy
    • Idiopathic inflammatory demyelinating diseases
    • Lambert-Eaton myasthenic syndrome
    • Multiple sclerosis, pattern 2
    • Pediatric autoimmune neuropsychiatric disorder associated with streptococcus
    • Progressive inflammatory neuropathy
    • Restles leg syndrome
    • Stiff person syndrome
    • Sydenham chorea
    • Transverse myelitis
  • Eyes:
    • Autoimmune retinopathy
    • Autoimmune uveitis
    • Cogan syndrome
    • Graves ophthalmopathy
    • Intermediated uveitis
    • Ligneous conjunctivitis
    • Mooren's ulcer
    • Neuromyelitis optica
    • Opsoclonus myoclonus syndrome
    • Optic neuritis
    • Slceritis
    • Susac's syndrome
    • Sympathetic ophthalmia
    • Tolosa-Hunt syndrome
  • Ears:
    • Autoimmune inner ear disease
    • Meniere's disease
  • Vascular system:
    • Anti-neutrophil cystoplasmic antibody-associated vasculitis
    • Behcet's disease
    • Churg-Strauss syndrome
    • Giant cell arteritis
    • Henoch-Schonlein purpura
    • Kawasaki's disease
    • Leukocytoclastic vasculitis
    • Lupus vasculitis
    • Rheumatoid vasculitis
    • Microscopic polyangiitis
    • Polyarteritis nodosa
    • Polymyalgia rheumatica
    • Urticarial vasculitis
    • Vasculitis
Tx
  • Immunosuppression, a Rx that decreases the immune response
  • Cytokine blockade, or the blockade of cytokine signalling pathways
  • Removal of effector T-cells or B-cells, e.g. anti-CD20 therapy can be effective at removing instigating B cells
  • IV immunoglobulin, has been helpful to Tx some antibody mediated autoimmune diseases as well, possibly through negative feedback mechanisms
Epidemiology
  • A large number of autoimmune diseases are recognized

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Definition of Autoimmune disease | Autoprac


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