IgG4-ziekte

• Confirmation diagnosis
  
  • biopsy of an involved organ
    
  • clinical
    
  • serologic: IgG4
    
  • radiologic data
    
• specificity and sensitivity of more than 97 and 82 percent
  

• often subacute development of a mass in affected organ or diffuse enlargement of an organ
  
• more common in middle-aged and older males
  
• lymphadenopathy common
  
• one of the characteristic patterns of organ or tissue involvement
  
  • pancreatitis of unknown origin
    
  • sclerosing cholangitis
    
  • bilateral salivary and/or lacrimal gland enlargement
    
  • retroperitoneal fibrosis (RPF) / chronic sclerosing aortitis and periaortitis
    
  • orbital pseudotumor or proptosis
    
• likelihood increased with
  
  • high serum levels of IgG4
    
  • allergic symptoms or asthma
    
  • other fibrotic processes are also present
    
• ook gezien: Riedel's thyroiditis / Hashimoto, interstitial pneumonitis and pulmonary inflammatory pseudotumors, kidney disease (mn TIN)
  

• pancreatic cancer
  
• cholangiocarcinoma
  
• primary sclerosing cholangitis
  
• Sjögren's syndrome (SS)
  
• anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV)
  
• Castleman disease
  
• idiopathic RPF
  
• infectious aortitis
  

Some of these conditions may also exhibit increased levels of serum IgG4 (table 1). (See 'Differential diagnosis' above.)

• symptomatische actieve ziekte (spoed: pancreatobiliary or renal disease), also identified by biochemical (bv. leverbiochemie, kreatinine) or imaging abnormalities (bv. aortitis/periaortitis, retroperitoneal fibrosis): lacrimal gland swelling, orbital pseudotumors, submandibular or parotid gland swelling (pain or concern about cosmetic issues), renal involvement, type 1 autoimmune pancreatitis (AIP) or retroperitoneal fibrosis (steatorrhea, pain, hydronephrosis, or other manifestations of their organ involvement)
  
• asymptomatic indien progressive disease
  
• niet asymptomatic, nonprogressive, and limited disease, bv. asymptomatic lymphadenopathy, mild submandibular gland enlargement, or incidentally detected lung nodules, do require regular monitoring (eg, every six months) to detect changes in symptoms, signs, or laboratory values that may indicate a need for treatment (eg, the development of pancreatic or renal dysfunction)
  

• zo nodig spoedinterventie: stent ureter of galweg
  
• prednisone (0.6 mg/kg/day), which is then tapered to discontinuation over a two-month period: symptomatic improvement, reductions in size of masses, improvement in organ function, and often a decrease in serum levels of IgG4
  
• no respons or no tapering to <5 mg daily: rituximab
  
• third: antimetabolite immunosuppressive, eg, azathioprine, methotrexate, or mycophenolate mofetil