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” قالوا سبحانك لا علم لنا إلا ما علمتنا إنك أنت العليم الحكيم “

بسم الله الرحمن الرحيم. ” قالوا سبحانك لا علم لنا إلا ما علمتنا إنك أنت العليم الحكيم “ صدق الله العظيم (البقرة: 32 ). AUTOINFLAMMATORY DISORDERS By DR. RANIA EL-TATAWY Ass. Prof. Dermatology & Venereology.

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” قالوا سبحانك لا علم لنا إلا ما علمتنا إنك أنت العليم الحكيم “

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  1. بسم الله الرحمن الرحيم ” قالوا سبحانك لا علم لنا إلا ما علمتنا إنك أنت العليم الحكيم “ صدق الله العظيم (البقرة:32)

  2. AUTOINFLAMMATORY DISORDERS By DR.RANIA EL-TATAWY Ass. Prof. Dermatology & Venereology.

  3. The autoinflammatory disorders (AIDS) are a new and expanding classification of inflammatory diseases characterized by recurrent episodes of systemic inflammation in the absence of pathogens, autoantibodies or antigen specific T cells.

  4. Autoinflammatory disorders are caused by primary dysfunction of the innate immune system, without evidence of adaptive immune dysregulation. These disorders are all caused by or associated with mutation of gene regulating innate immunity.

  5. Proteins that are mutated in AIDs mediate the regulation of NF kappa-B activation, cell apoptosis, and IL-1beta secretion through cross-regulated and sometimes common signaling pathways.

  6. I-Hereditary periodic fever syndromes It characterized by periodic or recurrent episodes of systemic inflammation causing fever often associated with rash, serositis (peritonitis, pleuritis), lymphadenopathy, arthritis, and other clinical manifestations. Systemic reactive (AA) amyloidosis may be a severe long-term complication.

  7. 1-Familial Mediterranean fever. -Autosomal recessive. -Result from defective in pyrin protein that down regulates inflammation. -Recurrent painful febrile attacks, peritonitis, pleuritis, arthritis. -Skin rash: Eerysiplas like lesion . Polyarteritis nodosa. Henoch-schonlein purpura. -Histopathology :Massive neutrophilic infiltration in dermis.

  8. 2-Tumor necrosis factor receptor-associatedsyndrome (TRAPS). - Caused by a defective membrane receptor for TNF - Fever, myalgia, arthralgia ,bdominal pain, cojunctivitis. - Skin rash : -Migratory erythematous patch overlying the area with myalgia. -Urticarial like plaques -Reccurent panniculitis.

  9. 3-Hyperimmunoglobulin-Dsyndrome with periodic fever(HIDS). - Autosomal recessive . - Geneticaly conferred to deficiency of mevalonte kinase. - Fever, severe abdominal pain, diarrhea ,arthritis, cervical lymphadenopathy. - Skin rash: - Macules ,papules ,nodules, petechiae, purpra. - Sweet syndrome like . - Cellulitis like - Erythema elevatum diutinum. - Elevated Ig D serum

  10. II-Cryopyrinopathies They are a group of conditions, characterized by a chronic or recurrent systemic inflammation variably associated with a number of clinical features, such as urticarial-like rash, arthritis, sensorineural deafness, and central nervous system and bone involvement.

  11. They associated to mutations of the gene encoding cryopyrin → hyperactivation of inflammasome → ↑ caspase-1 generation → hyperactivation of IL-1 . Also activation of IL-6 is mediated via NF kappa B.

  12. 1- Familial cold autoinflammatory syndrome -Autosomal dominant disease. -Early onset cold-induced itchy urticarial rash - Chills, fever, arthralgia, mialgia, headache and conjunctivitis.

  13. 2-Muckle–Wells syndrome(MWS). -Urticarial-deafness-amyloidosis syndrome(UDA). - Famalial urticaria during early childhood . - Characterised by periodic attacks of fever ,limb pain ,urticarial like eruption progressive perceptive deafness . -May be associated with amayloid nephropathy.

  14. 3-Chronic infantile neurological cutaneous and articular syndrome (CINCA). -Neonatal Onset Multisystem Inflammatory Disease (NOMID). -Skin rash, severe arthritis ,chronic meningitis leading to neurological damage.

  15. III_pyogenic disorders These disorders are dominated by the presence of sterile pyogenic abscesses affecting the skin, joints, and bones.

  16. - Caused by alteration of the protein PSTPIP_1 (proline, serine ,threonine, phosphatase, and interactive protein). - Mutation in its encoding gene → hyperphosphorylated products → bind more to pyrin → reduces pyrin’s braking on inflammasome activation.

  17. 1- pyogenic sterile arthritis, pyoderma gangrenosum, and acne syndrome. 2- Chronic recurrent multifocal osteomyelitis (CRMO). 3- Majeed syndrome : -CRMO -Neutrophilic dermatosis. -Sweet syndrome.

  18. VI-Granulomatous disorders Blau’s syndrome : -Autosomal dominant disorder . - it is characterized by familial granulomatous arthritis, iritis, and skin granulomas, (sarcoidosis and granuloma annulare ).

  19. V-Collagen disease-like chronic inflammatory disease. Behcet's disease . Crohn's disease. Sarcoidosis. Psoriatic arthritis. Atopic dermatitis.

  20. TNANK YOU

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