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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
General
fibrosclerosis
References
- Goldman's Cecil Medicine
Goldman L and Shafer AI (eds)
24th edition. Elsevier
https://www.inkling.com/read/cecil-textbook-of-medicine-goldman-schafer-24th/chapter-284/idiopathic-multifocal