multifocal fibrosclerosis

Etiology

• risk factors
  • smoking
  • asbestos exposure
  • aortic atherosclerosis
  • aortic aneurysm
  • systemic inflammation
  • autoimmune disease
  • elevated serum IgG4
  • malignancy
    • metastatic carcinoma
    • lymphoma
    • carcinoid
  • retroperitoneal injury
  • granulomatous diseases
    • tuberculosis
    • sarcoidosis
  • Erdheim-Chester disease
  • biliary disease
  • gonorrhea
  • ascending lymphangitis

Epidemiology

• rare
• 1.3 to 3 per 100,000
• male to female ration 3:1
• mean age of onset 56-64 years of age

Pathology

• multifocal fibrosis
  • large number of spindle cells expressing macrophage markers
  • activated fibroblasts
• inflammatory pseudotumors
• retroperitoneal fibrosis
  • entraps & distorts retroperitoneal structures including
    • great vessels
    • kidney & ureters
    • biliary tree
    • nerves
• mediastinal fibrosis
• orbital pseudotumor
• Dupuytren's contracture
• lymphoid hyperplasia
• Peyronie's disease
• vasculitis
• thyroiditis
• biliary cirrhosis
• testicular fibrosis
• pachymeningitis

Clinical manifestations

• abdominal pain or low back pain
• vomiting or diarrhea
• renal insufficiency, hydronephrosis
• peripheral edema, varicose veins
• nerve entrapment may occur
• deep vein thrombosis, pulmonary embolism
• superior vena cava syndrome
• portal hypertension
  • esophageal varices
• uveitis

Laboratory

• biopsy, CT or ultrasound-guided

Radiology

• computed tomography (CT) or magnetic resonance imaging (MRI)
• urography

Management

• surgical lysis of fibrotic masses
• stenting or catheter drainage of biliary, ureteral, veouns, or arterial obstruction
• lifelong anticoagulation for deep vein thrombosis
• prednisone taper over 8 months for preventing relapse
• role of immunosuppressive agents for maintenance unknown
• statins may be of benefit

More general terms

• fibrosclerosis