Multifocal fibrosclerosis
Disorder of unknown cause with fibrous lesions at multiple sites.
Multifocal fibrosclerosis is a condition whose cause remains unknown, marked by fibrous growths that can appear together or separately in various parts of the body. Common sites include the retroperitoneum, the mediastinum, and the thyroid gland (as Riedel's thyroiditis). Today, it is understood as a form of IgG4-related disease.
The illness can show up with a wide range of symptoms, such as inflammation of the bile ducts, sclerosing pancreatitis, Riedel's thyroiditis, and retroperitoneal fibrosis. It has also been linked to fibrosis in the lungs, parotid glands, lacrimal glands, kidneys, pancreas, and testes. When the nervous system is involved, neurological issues like pachymeningitis, cranial nerve palsies, hypopituitarism, and central diabetes insipidus may occur.
The exact cause is unknown, but one leading theory points to an autoimmune process. This idea is supported by the presence of plasma cells and lymphocytes in tissue samples, the frequent finding of focal vasculitis on biopsy, and the fact that some patients improve with systemic corticosteroids.
No single test can definitively confirm the diagnosis. Common clinical findings include multiple areas of widespread fibrosis, a high erythrocyte sedimentation rate (ESR), and an elevated white blood cell count, particularly eosinophilia. High levels of autoantibodies—such as rheumatoid factor, antinuclear antibody, and antithyroid antibodies—are often seen. Under the microscope, the fibrotic tissue shows fibrosclerosis with prominent hyalinizing collagen bundles and proliferation of myofibroblasts.
Various treatment approaches have been reported, including radiotherapy, surgery, cyclophosphamide, and colchicine, though results are often inconsistent. Glucocorticoids are currently the preferred treatment, even though individual responses vary.
- Field
- Medicine
- Known for
- Fibrous lesions at multiple sites; now considered IgG4-related disease
- Symptoms
- Cholangitis, sclerosing pancreatitis, Riedel thyroiditis, retroperitoneal fibrosis, pulmonary fibrosis, parotid gland fibrosis, lacrimal gland fibrosis, kidney fibrosclerosis, pancreatic fibrosis, tes
Lore & Background
Multifocal fibrosclerosis presents with a variety of symptoms, including cholangitis, sclerosing pancreatitis, Riedel thyroiditis, and retroperitoneal fibrosis. It has also been linked to pulmonary fibrosis, parotid gland fibrosis, lacrimal gland fibrosis, kidney fibrosclerosis, pancreatic fibrosis, and testicular fibrosis. Neurological manifestations such as pachymeningitis, cranial nerve palsies, hypopituitarism, and central diabetes insipidus have been reported.
Reader's Guide
The etiology of multifocal fibrosclerosis is unknown, but an autoimmune process is suspected based on pathological features of cellular infiltration with plasma cells and lymphocytes, focal vasculitis, and positive response to systemic corticosteroids in some patients. Diagnosis cannot be definitively confirmed by a single test; common findings include multiple areas of disseminated fibrosis, elevated erythrocyte sedimentation rate, increased white cell count with eosinophilia, and high titers of autoantibodies. Histology shows fibrosclerosis with hyalinizing collagen bundles and myofibroblast proliferation. Treatment varies, with glucocorticoids currently preferred, though radiotherapy, surgery, cyclophosphamide, and colchicine have been used with patchy results.
Did You Know?
- Multifocal fibrosclerosis is now considered a manifestation of IgG4-related disease.
- Histology of fibrotic lesions shows hyalinizing collagen bundles and myofibroblast cell proliferation.
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