Behcet's disease: Difference between revisions
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==Background== | ==Background== | ||
* Chronic small-vessel vasculitis | *Chronic small-vessel vasculitis | ||
* Mucocutaneous, ocular, CV, renal, GI, pulmonary, urologic, musculoskeletal, CNS involvement | *Mucocutaneous, ocular, CV, renal, GI, pulmonary, urologic, musculoskeletal, CNS involvement | ||
* Aphthous oral ulcers + ≥ 2 of following: Genital aphthae, cutaneous lesions, and neurological, oral or rheumatologic manifestations | *Aphthous oral ulcers + ≥ 2 of following: Genital aphthae, cutaneous lesions, and neurological, oral or rheumatologic manifestations | ||
==Clinical Features== | ==Clinical Features== | ||
* Age 20-30s MC | *Age 20-30s MC | ||
* Turkey, Japan, Middle East | *Turkey, Japan, Middle East | ||
* Triad: Recurrent oral aphthous ulcers, genital ulcers, and uveitis | *Triad: Recurrent oral aphthous ulcers, genital ulcers, and uveitis | ||
* Classically painful ulcer with necrotic center and red rim | *Classically painful ulcer with necrotic center and red rim | ||
* Skin: Subcutaneous nodules, pyoderma gangrenosum, cutaneous thrombophlebitis, pustular acne-like folliculitis | *Skin: Subcutaneous nodules, pyoderma gangrenosum, cutaneous thrombophlebitis, pustular acne-like folliculitis | ||
* Ocular: Uveitis, iritis, optic neuritis, vision loss, hypopyon | *Ocular: Uveitis, iritis, optic neuritis, vision loss, hypopyon | ||
* Neurologic: Brainstem and corticospinal tract syndromes, aseptic meningoencephalitis, increased ICP, cerebral sinus thrombosis, optic nerve ischemia | *Neurologic: Brainstem and corticospinal tract syndromes, aseptic meningoencephalitis, increased ICP, cerebral sinus thrombosis, optic nerve ischemia | ||
* GI: Ulcers, obstruction, ileocecal perforation | *GI: Ulcers, obstruction, ileocecal perforation | ||
* Inflammatory oligoarthritis | *Inflammatory oligoarthritis | ||
* Vasculopathy: Aneurysm, vasculitis, superficial thrombophlebitis, thrombosis | *Vasculopathy: Aneurysm, vasculitis, superficial thrombophlebitis, thrombosis | ||
* Cardiac: Myocarditis, endocarditis, pericarditis | *Cardiac: Myocarditis, endocarditis, pericarditis | ||
* Renal: Glomerulonephritis, amyloidosis | *Renal: Glomerulonephritis, amyloidosis | ||
* Pulmonary: Pleural effusions, pulmonary HTN, pulmonary aneurysm | *Pulmonary: Pleural effusions, pulmonary HTN, pulmonary aneurysm | ||
==Differential Diagnosis== | ==Differential Diagnosis== | ||
* [[HSV]] | *[[HSV]] | ||
*[[lichen planus]] | *[[lichen planus]] | ||
*[[pemphigus vulgaris]] | *[[pemphigus vulgaris]] | ||
| Line 40: | Line 40: | ||
==Diagnosis== | ==Diagnosis== | ||
* Clinical diagnosis | *Clinical diagnosis | ||
* ESR/CRP elevated | *ESR/CRP elevated | ||
===Classification=== | ===Classification=== | ||
* International Study Group Criteria | *International Study Group Criteria | ||
* Recurrent oral aphthae (at least 3 times in 1 year) + 2 of the following in absence of other systemic diseases: | *Recurrent oral aphthae (at least 3 times in 1 year) + 2 of the following in absence of other systemic diseases: | ||
** Recurrent genital aphthae | **Recurrent genital aphthae | ||
** Eye lesions | **Eye lesions | ||
** Skin lesions | **Skin lesions | ||
** A positive pathery test | **A positive pathery test | ||
==Management== | ==Management== | ||
* Rheumatology consult +/- multidisciplinary consults | *Rheumatology consult +/- multidisciplinary consults | ||
* Oral and genital ulcers: Topical steroid | *Oral and genital ulcers: Topical steroid | ||
* Severe mucocutaneous disease: [[Prednisone]] 1mg/kg, low-dose thalidomide, or methotrexate | *Severe mucocutaneous disease: [[Prednisone]] 1mg/kg, low-dose thalidomide, or methotrexate | ||
* Systemic disease: Steroid +/- cyclophosphamide or azathioprine | *Systemic disease: Steroid +/- cyclophosphamide or azathioprine | ||
* Ocular: Prednisone + azathioprine and rapid ophthalmologist referral | *Ocular: Prednisone + azathioprine and rapid ophthalmologist referral | ||
* Cerebral [[venous sinus thrombosis]]- Heparinization | *Cerebral [[venous sinus thrombosis]]- Heparinization | ||
==References== | ==References== | ||
| Line 63: | Line 63: | ||
*Rosen's Emergency Medicine 8th edition. 2013. Chapter: Erythematosus and the Vasculitides. p1540. | *Rosen's Emergency Medicine 8th edition. 2013. Chapter: Erythematosus and the Vasculitides. p1540. | ||
*Smith EL, et al. Clinical manifestations and diagnosis of Behcet's disease. In: Post T, ed. UpToDate. Waltham, Mass.: UpToDate; 2014. www.uptodate.com. Accessed December 22, 2014. | *Smith EL, et al. Clinical manifestations and diagnosis of Behcet's disease. In: Post T, ed. UpToDate. Waltham, Mass.: UpToDate; 2014. www.uptodate.com. Accessed December 22, 2014. | ||
* Alnaimat FA, et al. (2014, Dec 16). Behcet Disease. eMedicine. Retrieved 12/22/2014 from http://emedicine.medscape.com/article/329099-overview | *Alnaimat FA, et al. (2014, Dec 16). Behcet Disease. eMedicine. Retrieved 12/22/2014 from http://emedicine.medscape.com/article/329099-overview | ||
[[Category:Rheumatology]] | [[Category:Rheumatology]] | ||
Revision as of 17:56, 4 July 2016
Background
- Chronic small-vessel vasculitis
- Mucocutaneous, ocular, CV, renal, GI, pulmonary, urologic, musculoskeletal, CNS involvement
- Aphthous oral ulcers + ≥ 2 of following: Genital aphthae, cutaneous lesions, and neurological, oral or rheumatologic manifestations
Clinical Features
- Age 20-30s MC
- Turkey, Japan, Middle East
- Triad: Recurrent oral aphthous ulcers, genital ulcers, and uveitis
- Classically painful ulcer with necrotic center and red rim
- Skin: Subcutaneous nodules, pyoderma gangrenosum, cutaneous thrombophlebitis, pustular acne-like folliculitis
- Ocular: Uveitis, iritis, optic neuritis, vision loss, hypopyon
- Neurologic: Brainstem and corticospinal tract syndromes, aseptic meningoencephalitis, increased ICP, cerebral sinus thrombosis, optic nerve ischemia
- GI: Ulcers, obstruction, ileocecal perforation
- Inflammatory oligoarthritis
- Vasculopathy: Aneurysm, vasculitis, superficial thrombophlebitis, thrombosis
- Cardiac: Myocarditis, endocarditis, pericarditis
- Renal: Glomerulonephritis, amyloidosis
- Pulmonary: Pleural effusions, pulmonary HTN, pulmonary aneurysm
Differential Diagnosis
- HSV
- lichen planus
- pemphigus vulgaris
- pemphigoid
- IVD
- SJS
- celiac dz
- SLE
- auto-inflammatory disorders
- Sjogren's
- MS
- sarcoidosis
- syphillis
- TB
- malignancy
- reactive arthritis
- HIV
- vasculitides
Diagnosis
- Clinical diagnosis
- ESR/CRP elevated
Classification
- International Study Group Criteria
- Recurrent oral aphthae (at least 3 times in 1 year) + 2 of the following in absence of other systemic diseases:
- Recurrent genital aphthae
- Eye lesions
- Skin lesions
- A positive pathery test
Management
- Rheumatology consult +/- multidisciplinary consults
- Oral and genital ulcers: Topical steroid
- Severe mucocutaneous disease: Prednisone 1mg/kg, low-dose thalidomide, or methotrexate
- Systemic disease: Steroid +/- cyclophosphamide or azathioprine
- Ocular: Prednisone + azathioprine and rapid ophthalmologist referral
- Cerebral venous sinus thrombosis- Heparinization
References
- Rosen's Emergency Medicine 8th edition. 2013. Chapter: Erythematosus and the Vasculitides. p1540.
- Smith EL, et al. Clinical manifestations and diagnosis of Behcet's disease. In: Post T, ed. UpToDate. Waltham, Mass.: UpToDate; 2014. www.uptodate.com. Accessed December 22, 2014.
- Alnaimat FA, et al. (2014, Dec 16). Behcet Disease. eMedicine. Retrieved 12/22/2014 from http://emedicine.medscape.com/article/329099-overview