View source for Multifocal fibrosclerosis

{{Infobox medical condition (new)
| name            = Multifocal fibrosclerosis
| synonyms        = Idiopathic fibrosclerosis
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'''Multifocal fibrosclerosis''' and '''idiopathic fibrosclerosis''' are disorders of unknown aetiology, characterised by fibrous [[lesion]]s (co-)occurring at a variety of sites. Known manifestations include [[retroperitoneal fibrosis]], [[mediastinal fibrosis]] and [[Riedel's thyroiditis]].<ref>{{Cite journal| last1 = Brooks| first1 = MS| last2 = Robinson| first2 = JA| title = Idiopathic fibrosclerosis| journal = The Journal of Rheumatology| volume = 12| issue = 3| pages = 575–9| year = 1985| pmid = 4045855}}</ref><ref>{{Cite journal| doi = 10.1507/endocrj.47.335| last1 = Kishimoto | first1 = M.| last2 = Okimura | first2 = Y.| last3 = Kimura | first3 = K.| last4 = Mizuno | first4 = I.| last5 = Iguchi | first5 = G.| last6 = Fumoto | first6 = M.| last7 = Takahashi | first7 = Y.| last8 = Kanda | first8 = F.| last9 = Kaji | first9 = H.| last10 = Abe | first10 = H.| last11 = Hanioka | first11 = K.| last12 = Chihara | first12 = K.| title = Multifocal fibrosclerosis as a possible cause of panhypopituitarism with central diabetes insipidus| journal = Endocrine Journal| volume = 47| issue = 3| pages = 335–342| year = 2000| pmid = 11036878| doi-access = free}}</ref>
They are now considered to be manifestations of [[IgG4-related disease]].<ref name=pmid22736240>{{cite journal |author1=John H. Stone |author2=Arezou Khosroshahi |author3=Vikram Deshpande |title=Recommendations for the nomenclature of IgG4-related disease and its individual organ system manifestations |journal=Arthritis & Rheumatism |date=October 2012 |volume=64 |issue=10 |pages=3061–3067 |doi=10.1002/art.34593 |pmid=22736240|pmc=5963880 |display-authors=etal}}</ref>
==Signs and symptoms==
The illness can present with a variety of symptoms, such as [[cholangitis]], sclerosing [[pancreatitis]], [[Riedel's thyroiditis|Riedel thyroiditis]], and [[retroperitoneal fibrosis]].<ref name="Cutaneous involvement">{{cite journal | last1=Kromer | first1=Courtney | last2=Winfield | first2=Harry | last3=Franklin | first3=Matthew | title=Cutaneous involvement in a case of multifocal idiopathic fibrosclerosis | journal=JAAD Case Reports | publisher=Elsevier BV | volume=6 | issue=4 | year=2020 | issn=2352-5126 | doi=10.1016/j.jdcr.2020.02.020 | pages=357–358| pmid=32258321 | pmc=7103662 }}</ref> Apart from the aforementioned characteristics, the syndrome has also been linked to [[pulmonary fibrosis]], [[parotid gland]] fibrosis, [[lacrimal gland]] fibrosis, kidney fibrosclerosis, pancreatic fibrosis, and testicular fibrosis.<ref name="a new case report and review of the literature">{{cite journal | last1=Oguz | first1=Kader | last2=Kiratlı | first2=Hayyam | last3=Oguz | first3=Oguzhan | last4=Cila | first4=Aysenur | last5=Oto | first5=Aytekin | last6=Gokoz | first6=Aytac | title=Multifocal fibrosclerosis: a new case report and review of the literature | journal=European Radiology | publisher=Springer Science and Business Media LLC | volume=12 | issue=5 | date=December 1, 2001 | issn=0938-7994 | doi=10.1007/s00330-001-1158-4 | pages=1134–1138| pmid=11976858 | s2cid=274033 }}</ref> Neurological manifestations such as pachymeningitis, [[Cranial nerve disease|cranial nerve palsies]], [[hypopituitarism]], and [[central diabetes insipidus]] have also been linked to neurological involvement.<ref name="a new case report">{{cite journal | last1=Sommerfield | first1=A J | last2=Lockman | first2=K A | last3=Bathgate | first3=A J | last4=Patrick | first4=A W | title=Multifocal fibrosclerosis: a new case report | journal=Annals of Clinical Biochemistry: International Journal of Laboratory Medicine | volume=45 | issue=1 | date=2008 | issn=0004-5632 | doi=10.1258/acb.2007.007049 | pages=99–101| pmid=18275684 }}</ref>
==Causes==
Multifocal fibrosclerosis's etiology is unknown, but one theory of the cause suggests that an [[Autoimmunity|autoimmune]] process is to blame. This is corroborated by the pathological features of cellular infiltration, which include [[Plasma cell|plasma cells]] and [[Lymphocyte|lymphocytes]], the commonly observed focal [[vasculitis]] on pathological examination, and the positive response of some multifocal fibrosclerosis patients to systemic [[corticosteroid]] treatment.<ref name="a new case report"/>
==Diagnosis==
The diagnosis cannot be definitively confirmed by a test. However, common clinical findings include multiple areas of disseminated fibrosis, an elevated [[erythrocyte sedimentation rate]] (ESR), and an increased [[White blood cell|white cell]] count, particularly [[eosinophilia]]. High titers of [[Autoantibody|autoantibodies]] are frequently observed, including [[rheumatoid factor]], [[antinuclear antibody]], and [[Antithyroid autoantibodies|antithyroid antibodies]].<ref name="a new case report"/> The fibrotic lesion's histology shows fibrosclerosis with noticeable hyalinizing collagen bundles and [[myofibroblast]] cell proliferation.<ref name="a new case report and review of the literature"/>
==Treatment==
Numerous distinct treatment plans have been documented in the literature. There have been documented cases of treatment with [[Radiation therapy|radiotherapy]], surgery, [[cyclophosphamide]], and [[colchicine]], frequently with only patchy results. Although the response to treatment varies, [[Glucocorticoid|glucocorticoids]] are currently the preferred course of action.<ref name="a new case report"/>
==References==
{{Reflist}}
==Further reading==
* {{cite journal | last=COMINGS | first=DAVID E. | title=Familial Multifocal Fibrosclerosis | journal=Annals of Internal Medicine | publisher=American College of Physicians | volume=66 | issue=5 | date=May 1, 1967 | pages=884–892 | issn=0003-4819 | doi=10.7326/0003-4819-66-5-884 | pmid=6025229 | ref=none}}
* {{cite journal | last1=DRIESKENS | first1=OLIVIER | last2=BLOCKMANS | first2=DANIEL | last3=VAN DEN BRUEL | first3=ANNICK | last4=MORTELMANS | first4=LUC | title=Riedel's Thyroiditis and Retroperitoneal Fibrosis in Multifocal Fibrosclerosis | journal=Clinical Nuclear Medicine | publisher=Ovid Technologies (Wolters Kluwer Health) | volume=27 | issue=6 | year=2002 | issn=0363-9762 | doi=10.1097/00003072-200206000-00005 | pages=413–415 | pmid=12045432 | s2cid=423062 | ref=none}}
==External links==
{{Medical resources
| ICD11           = {{ICD11|FB51.Y}}
| ICD10           = {{ICD10|M35.5}}
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| OMIM            = 228800
| MeshID          = C537375
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[[Category:IgG4-related disease]]
[[Category:Idiopathic diseases]]