Relapsing Polychondritis Flashcards
Relapsing polychondritis pathogenesis
Activated lymphocytes and macrophages → release of lysosomal enzymes (eg proteases) against cartilage components (collagen type 2, 9, 11, matrilin 1 - found exclusively in resp tract and ears), and proteoglycans → destruction and attempt at repair by local fibroblasts and chondrocytes → granulation tissue and fibrosis
RPC (relapsing polychondritis) histopath
Cartilage matrix acidophilic (pink) with hamtoxylin and eosin staining (usually basophlic and blue)
-Inflammatory cell infiltrates (PMN, lymph, plasma cells) invade cartilage from periphery
-Granulation tissue and fibrosis adjacent to inflamm infiltrates
-Electron microscopy: increased lipids and lysosomes in chondrocytes on
-Immunofluorscence: Ig and complements
When to bx RPC
- Lack of multiple sites of targeted cartilage involvement
– Failure to resp to pred / dapsone
– Features concerning for alt dx
DIagnostic criteria RPC
McAdam 3/6 OR 1/6 + histology OR 2/6 + response to steroids/dapsone:
– Bilat auricular chondritis
– Nonerosive seroneg inflamm polyarthriits
– Nasal chondritis
– Ocular inflamm
– Resp tract chondritis
– Audiovestibular damage
-Michet: 2maj or 1maj+2minor
-Major:
– Proven inflamm episodes involving auricular cartilage OR
– Nasal cartilage OR
– Laryngotracheal cartilage
-Minor:
– Ocular inflamm (conjunctivitis, keratitis, episcleritis, uveitis)
– Hearing loss
– Vestibular dysfcn
– Seroneg inflamm arthritis
** RPC clinical manifestations**
Auricular chondritis
-Arthritis (oligo/poly, nonerosive, asymmetric)
-Tenosynovitis
-Nasal chondritis
-Ocular inflamm
-Laryngotracheal sx (eg laryngeal, epiglottal edema, choking, stridor, SOB, resp failure,t racheal stenosis)
-Reduced hearing
-Vestibular dysfcn
-Saddle nose
-Cutaneous
-Laryngotracheal stricture
-Vasculitis
-Costochondritis (SC, CC, SM joints; flail chest)
-Recurrent respiratory infxn
** Ocular inflamm RPC**
Episcleritis,
-Scleritis,
-Necrotizing scleritis,
-Lid edema,
-Orbital inflamm dz,
-Conjunctivitis,
-Uveitis,
-Peripheral ulcerative keratitis,
-Retinal vasculitis,
-Optic neuritis
-Cataracts
-Proptosis
-Corneal ulcerations /thinning
-EoM palsy
Audiovestibular Mx RPC
Hearing loss (conductive from inflam edema or collapse of external auditory canal or eustacian tubes or sensorineural from inflam of internal auditory artery)
-Tinnitus
-Vertigo
-Fullness in ear (from serous otitis media)
Cardiac Mx RPC
Aortic insuff from DILATION of root
-Pericarditis
-Myocarditis
-Arrhythmia
-Coronary aneurysm
-Valvulitis
-Conduction defects
DDx valvulitis
Rheum fever
-RA
-AS
-ReA
-Behcet
-Endocarditis
DDx dilation of valve ring
Marfan
-Syphilis
-RPC
-Dissecting aneurysm
-Idiopathic
-Takayasu
-GCA
-Cogan
** RPC Derm Mx**
Oral aphthosis ~Behcet (MAGIC)
-LCV
-Erythema nodosum
-Alopecia
-Abnormal nail growth
-Superficial thrombophlebitis
** RPC Neuro Mx**
Cranial neuropathy
-H/A
-Seizures
-Aseptic meningitis
-Encpahlopath
-Hemiplegia
-Ataxia
** RPC Renal Mx**
Elevated Cr
-Microhematuria
RPC Lab Mx
Inflamm markers
-High WBC, Plt
-Chronic anemia
-Increased alpha & gamma globulins
-Low titre RF, ANA, ANCA
-AB to type 2 collagen
RPC radiographic abN
XR: tracheal air column (tracheal stenosis), periarticular osteopenia (nonerosive)
-CT/MRI: tracheal narrowing nad inflamm
RPC DDX
Cocaine induced vasculitis
-Infectious perichondritis
-Frostbite
-Recurrent trauma (wrestlers)
-Cellulitis
-Things that cause saddles nose: GPA, syphilis, cocaine, lethal midline granuloma
-Things that cause aortitis: Marfan, syphilis, rheum
-Cogan (keratitis, vestibuloauditory dysfxn)
RPC coexisting dz
Vasculitis: Behcet, MAGIC, GPA, PAN, eGPA
-Rheum: SLE, RA, SS, SpA, SSc
-Heme: MDS, lymphoma, ALL
-Autoimmune: Thyroidits, T2DM, IBD, MG, PBC
RPC poor prognostic factors
Nasal chondritis
-Airway involvement
-Renal involvement
-Vasculitis
-Male
-Cardiac involvement
-MDS / heme malignancy
Ix for RPC
CXR, CT/MRI (of tracheobronchial tree)
-TTE (aortic involvement)
-PFT
-BW: CBC, ANCA, metabolic, UA, infalmm markers
-Consult ENT or optho
RPC meds
NSAIDs
-Prednisone
-No organ involvement: Dapsone
-Organ involvement: CYC, MTX, AZA, LFN, MMF, Cyclosporine
-Refractory: biologics: IFX, ADA, ETN, Toci, Anakindra, abatacept (RITUX DOESNT WORK)
-Salvage: PLEX, IVIG, Stem cell
Surgery for RPC
Tracheostomy if unresponsive to positive pressure ventilation
-Resection for tracheaomalacia or tracheal stenosis
-Endoscopic laser ablation for focal lesion tx
-Intrabronchial stent placement for airway collapse
-Valve replacement for aortic dilation
-Cochlear implants for hearing loss
-Reconscrution of nasal septal collapse
** Relapsing polychondritis: List 4-5 clinical manifestations of relapsing polychondritis beside saddle nose?**
Auricular chondritis/deformity
-Arthritis
-Ocular inflammation (uveitis, episcleritis, conjunctivitis)
-Laryngotracheal strictures
-Reduced hearing
-Vestibular dysfunction
-Vasculitis (LCV/Aortitis)
-Aortic insufficiency (most common cardiac manifestation in RPC, and besides resp manifestations is the most serious)
-Oral ulcers
-Glomerulonephritis
** Name 2 diseases (other than RP) that can give saddle nose**
- Granulomatous Disorders:
-a) Granulomatosis Polyangitis (GPA)
-b) Infection (Mycobacterial, Leprosy)
-c) Sarcoidosis
-2. Neoplastic Conditions, i.e. EBV Associated Nasal Lymphomas
–Lymphatomatoid Graulomatosis (B-Cell)
–Extranodal NK/T-Cell Lymphoma, Nasal type (T-Cell)
-*Note: The above conditions are collectively called “Midline Destructive Disease”
-3. Relapsing Polychondritis
-4. Primary Atrophic Rhinosinusitis
-5. Congenital Syphilis
-6. Iatrogenic
–cocaine abuse
–trauma (most common cause)
–surgery
–radiation
