Relapsing polychondritis

WikiDoc Resources for Relapsing polychondritis
Articles
Most recent articles on Relapsing polychondritis Most cited articles on Relapsing polychondritis Review articles on Relapsing polychondritis Articles on Relapsing polychondritis in N Eng J Med, Lancet, BMJ
Media
Powerpoint slides on Relapsing polychondritis Images of Relapsing polychondritis Photos of Relapsing polychondritis Podcasts & MP3s on Relapsing polychondritis Videos on Relapsing polychondritis
Evidence Based Medicine
Cochrane Collaboration on Relapsing polychondritis Bandolier on Relapsing polychondritis TRIP on Relapsing polychondritis
Clinical Trials
Ongoing Trials on Relapsing polychondritis at Clinical Trials.gov Trial results on Relapsing polychondritis Clinical Trials on Relapsing polychondritis at Google
Guidelines / Policies / Govt
US National Guidelines Clearinghouse on Relapsing polychondritis NICE Guidance on Relapsing polychondritis NHS PRODIGY Guidance FDA on Relapsing polychondritis CDC on Relapsing polychondritis
Books
Books on Relapsing polychondritis
News
Relapsing polychondritis in the news Be alerted to news on Relapsing polychondritis News trends on Relapsing polychondritis
Commentary
Blogs on Relapsing polychondritis
Definitions
Definitions of Relapsing polychondritis
Patient Resources / Community
Patient resources on Relapsing polychondritis Discussion groups on Relapsing polychondritis Patient Handouts on Relapsing polychondritis Directions to Hospitals Treating Relapsing polychondritis Risk calculators and risk factors for Relapsing polychondritis
Healthcare Provider Resources
Symptoms of Relapsing polychondritis Causes & Risk Factors for Relapsing polychondritis Diagnostic studies for Relapsing polychondritis Treatment of Relapsing polychondritis
Continuing Medical Education (CME)
CME Programs on Relapsing polychondritis
International
Relapsing polychondritis en Espanol Relapsing polychondritis en Francais
Business
Relapsing polychondritis in the Marketplace Patents on Relapsing polychondritis
Experimental / Informatics
List of terms related to Relapsing polychondritis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] ,Associate Editor(s)-in-Chief: Manpreet Kaur, MD [2]

Overview

Relapsing polychondritis is an immune-mediated chronic disease which involves the cartilaginous structure of different parts of the body, most commonly those of the ears, nose, and laryngotracheobronchial tree. The term relapsing polychondritis was first coined by Pearson in 1960. The majority of patients with relapsing polychondritis remain asymptomatic for several years. Early clinical features include ear pain, polyarthritis and other non-specific symptoms such as fever, weight loss and skin rash. The pathogenesis of relapsing polychondritis is unknown, however several studies have suggested the role of both cellular immunity and abnormal autoantibody in the pathogenesis of relapsing polychondritis. There are two different criteria to diagnose relapsing polychondritis which include Mc Adam criteria and modified Damiani criteria. There are no specific laboratory findings associated with relapsing polychondritis but certain antibodies are raised such as anti-type II collagen antibodies and antineutrophil cytoplasmic antibodies. Other abnormal laboratory findings include low haemoglobin, leukocytosis, eosinophilia, increased ESR, CRP, urinary glycosaminoglycans levels and urinary collagen type II neoepitope levels in active inflammation. Imaging such as chest x-ray is helpful to find out tracheal stenosis, however, CT scan is more useful to diagnose subglottic stenosis. MRI is useful to see the difference between edema, fibrosis, and inflammation. The mainstay of therapy for relapsing polychondritis is medical therapy. NSAIDs are used as an initial treatment of relapsing polychondritis. If NSAIDs are contraindicated or resistant, then steroids or dapsone can be used. Prednisone is used in the treatment of relapse. Treatment of life-threatening organ damage include prednisone and cyclophosphamide. Various surgical options include tracheostomy, cardiac valve replacement, aortic aneurysm repair, and saddle-nose deformity repair.

Historical Perspective

• In 1923, Jaksch-Wartenhorst used the term polychondropathia to describe his first case about relapsing polychondritis.
• In 1960, the term relapsing polychondritis was first coined by Pearson.

Classification

• There is no established classification of relapsing polychondritis.

Pathophysiology

• The pathogenesis of relapsing polychondritis is unknown.
• Several studies have suggested the role of both cellular immunity and abnormal autoantibody in the pathogenesis of relapsing polychondritis.
• The HLA class II has been associated with the development of relapsing polychondritis, there is increase susceptibility with HLA-DR4.
• On gross pathology, thickening of cartilage of ear, nose, epiglottis, and cricoid and tracheal rings are characteristic findings of relapsing polychondritis.
• On microscopic histopathological analysis of relapsing polychondritis, following features are seen:
  • Lymphocytes, plasma cells, neutrophils, and eosinophils are seen in the cartilage.
  • Cartilage has lost the chondrocytes and normal architecture at the later stages of inflammation.
  • Cartilage is replaced by fibrous tissue in the end.

Differentiating Relapsing Polychondritis from other Diseases

• Chondritis
• Chondromdermatitis
• Hansen's disease
• Nodularis helicis
• Perichondritis
• Rhinophyma
• Skin cancer
• Syphilis

Also, Relapsing Polychondritis should be differenttieted form other causes of small-sized vessel vasculitis. Abbreviations: ABG= Arterial blood gas, ANA= Antinuclear antibody, ANP= Atrial natriuretic peptide, ASO= Antistreptolysin O antibody, BNP= Brain natriuretic peptide, CBC= Complete blood count, COPD= Chronic obstructive pulmonary disease, CRP= C-reactive protein, CT= Computed tomography, CXR= Chest X-ray, DVT= Deep vein thrombosis, ESR= Erythrocyte sedimentation rate, HRCT= High Resolution CT, IgE= Immunoglobulin E, LDH= Lactate dehydrogenase, PCWP= Pulmonary capillary wedge pressure, PCR= Polymerase chain reaction, PFT= Pulmonary function test.

Diseases			Clinical manifestations								Para-clinical findings									Gold standard	Additional findings
			Symptoms					Physical examination
											Lab Findings				Imaging				Histopathology
			Headache	Fever	Weight loss	Arthralgia	Claudication	Bruit	HTN	Focal neurological disorder	Biomarker	CBC	ESR	Other	CT scan	Angiography	Ultrasound/ Echocardiography	Other
Small-Vessel Vasculitis	ANCA-associated vasculitis	Microscopic polyangiitis	+	-	-	+/-	-	-	-	+	Anti-PR3 antibody (C-ANCA) (40%), Anti-MPO antibody (P-ANCA) (60%)	Leukocytosis, Normocytic anemia	↑	Proteinuria, Erythrocyte casts	Suspected pancreatitis	Mesenteric angiography for differentiating from polyarteritis nodosa	-	Bilateral nodular, and patchy opacities in CXR	Glomerulonephritis with focal necrosis, crescent formation, and lack or paucity of immunoglobulin deposits	Histological confirmation	Rash, Hemoptysis
		Granulomatosis with polyangiitis (Wegener’s)	+	+/-	+/-	-	-	-	-	+	Anti-PR3 antibody (C-ANCA) (90%), Anti-MPO antibody (P-ANCA) (10%)	Leukocytosis, Normochromic normocytic anemia	↑	↑Cr or BUN, Hypoalbuminemia	Consolidation, Patchy or diffuse ground-glass opacities	Occlusion or stenosis of LAD and RCA in coronary angiography	-	Single or multiple nodules and masses with cavitation in CXR	Parenchymal necrosis, Granulomatous inflammation	Histological confirmation	Conjunctivitis, Episcleritis, Uveitis, Optic nerve vasculitis
		Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)	+/-	-	-	+	-	-	-	+	Anti-MPO antibody (P-ANCA) (40%), Eotaxin-3	Eosinophilia, Anemia	↑	↑Cr or BUN, Proteinuria, Erythrocyte casts, ↑IgE levels	Significant enlargement of peripheral pulmonary arteries	Myocardial ischemia and infarction in coronary angiography	Congestive heart failure (CHF) in echocardiography	Extensive air-space opacities in CXR	Small necrotizing granulomas with eosinophilic core surrounded by macrophages and epithelioid giant cells	Histological confirmation	Allergic rhinitis, Asthma, Urticarial rash
		Hydralazine-associated ANCA-associated vasculitis	-	-	-	+	-	-	-	+/-	Anti-MPO antibody (P-ANCA), Anti-histone antibodies	Anemia	-	↑Cr or BUN, Hypoalbuminemia	Bilateral pulmonary infiltrates	Aneurysms or occlusions of the visceral arteries	-	-	Pauci-immune necrotizing and crescentic glomerulonephritis	Histological confirmation	Sinusitis, Hemoptysis
	Immune complex small-vessel vasculitis	Anti-glomerular basement membrane disease	+	+/-	-	-	-	-	+	-	Anti-GBM antibodies	Hypochromic microcytic anemia, Thrombocytopenia	-	↓C3 level	Pulmonary hemorrhage	-	Normal kidneys	Alveolar infiltrates spreading from the hilum in CXR	Cellular crescents in the glomeruli, Intra-alveolar hemorrhages	Anti-GBM antibodies	Hemoptysis, Hematuria
		Cryoglobulinemic vasculitis	+/-	+/-	-	+	+/-	-	-	-	C4 component	Leukocytosis, Anemia	↑	ANA, hypocomplementemia	R/O underlying malignancy	Stenosis or occlusions of the visceral arteries	Bacterial endocarditis in echocardiography	Interstitial involvement or pleural effusions in CXR	HCV-associated proteins in vasculitic skin, Intraluminal cryoglobulin deposits	Histological confirmation	Acrocyanosis, Retinal hemorrhage, Purpura
		Hepatitis C virus-associated cryoglobulinemic vasculitis	+/-	+/-	+	+	-	-	+	+/-	HCV RNA, Cryoglobulins	Leukocytosis, Anemia	↑	↓Serum C4, Positive RF	Increased hepatic echogenicity	-	Hepatomegaly, Splenomegaly	Increased hepatic echogenicity in MRI	Vasculitic skin, Antigen infilteration in lesions	HCV RNA, Histological confirmation	Palpable purpura, Microscopic hematuria
		IgA vasculitis (Henoch-Schönlein purpura)	-	-	+	+	-	-	-	-	IgA	Normochromic anemia, Leukocytosis	↑	Stool OB, ↓C3, ↓C4	-	-	Increased bowel wall thickness, hematomas, peritoneal fluid, and intussusception	Dilated loops of bowel consistent in abdominal X-ray	Leukocytoclastic vasculitis in postcapillary venules with IgA deposition	History and physical examination	Hematuria, Palpable purpura
		Hypocomplementemic urticarial vasculitis (anti-C1q vasculitis)	-	-	+/-	+	-	-	-	-	C1q	Mild anemia	↑	ANA, ↓C1q, ↓C3, ↓C4	-	-	Hepatomegaly, Splenomegaly	-	Deposits of immunoglobulins, complement, or fibrin around blood vessels	Urticaria, Histological confirmation	Urticaria, Hematuria
	Gastrointestinal disease	Acute mesenteric ischemia	-	+/-	-	-	-	-	-	-	I-FABP, Alpha-GST, Ischemia-modified albumin (IMA)	Leukocytosis, ↑HCT	-	↑Amylase	Bowel wall thickening, Intestinal pneumatosis, Portomesenteric thrombosis	Mesenteric venous thrombosis	Arterial stenosis or occlusion of the celiac or superior mesenteric arteries in duplex ultrasound	Ileus with distended loops of bowel, Bowel wall thickening in abdominal X-ray	Superficial mucosal hemorrhage, edema and necrosis	History and physical examination	Abdominal pain, Distension, Absent bowel sounds
	Cardiovascular disease	Infective Endocarditis	+	+	+	-	-	-	-	+	NT-proBNP	Normochromic-normocytic anemia	↑	Hyperglobulinemia, Cryoglobulinemia	Metastatic infections, such as splenic infarct, renal infarcts, or psoas abscess	-	Vegetation, abscess, or new dehiscence of a prosthetic valvein echocardiography	Vertebral osteomyelitis in MRI	Vegetation or intracardiac abscess demonstrating active endocarditis	Echocardiography (TTE)	Janeway lesions, Osler nodes, Roth spots, Vertebral osteomyelitis
		Leukocytoclastic Vasculitis	+	-	-	+	-	-	-	+	IgM, IgA, IgG	Leukocytosis, Anemia	↑	Hypocomplementemia	-	Vascular stenosis and obstruction in visceral angiography	-	-	Perivascular inflammatory infiltrate of neutrophils with leukocytoclasia (releasing nuclear debris)	Histological confirmation	Palpable purpura, Petechiae
	Pulmonary disease	Langerhans Cell Histiocytosis	+/-	-	-	+	-	-	-	-	CD1a, CD207,BRAF-V600E	Anemia	↑	Hypercalcemia	Pulmonary cysts and nodules, Bone lytic lesions	-	Hepatomegaly, Splenomegaly	Cerebellum and pons hyperintensity in MRI	Birbeck granules by electron microscopy	Histological confirmation	Brown to purplish papules, Eczematous rash
		Non-Small Cell Lung Cancer	-	+/-	+	-	-	-	-	+/-	EGFR, ROS1, EML4-ALK,  PD-L1	Leukocytosis, Anemia	↑	Hypercalcemia, Hyponatremia	Pulmonary lesion or mass	-	Pulmonary marginal lesions	Staging and response to treatment in PET-CT	Adenocarcinoma, Squamous cell carcinoma	High resolution CT-scan	Cough, Hemoptysis
		Small Cell Lung Cancer	-	+/-	+	-	-	-	-	+/-	p53, Thyroid transcription factor-1 (TTF1)	Anemia	↑	Hyponatremia	Large hilar mass with bulky mediastinal adenopathy	-	Endobronchial ultrasound (EBUS)	Standard staging	Spindled cells with dark nuclei, scant cytoplasm, and fine, granular nuclear chromatin	High resolution CT-scan	Cough, Hemoptysis
		Pulmonary Infarction	+/-	-	-	-	-	-	+	-	D-dimer	Mild leukocytosis, Mild anemia	-	Hypoxemia, Hypocarbia or Hypercarbia, Respiratory alkalosis	Pulmonary embolism	Low-density filling defect within the pulmonary artery	Pericardial effusion in echocardiography	Pulmonary infiltrates, atelectasis, and pleural effusions in CXR	Infarct induced apoptosis	Pulmonary artery angiography	Cough, Hemoptysis
	Renal disease	Acute Poststreptococcal Glomerulonephritis	-	-	-	+	-	-	+	+/-	Antistreptolysin-O (ASO) titers	Leukocytosis	↑	Hypocomplementemia	-	-	Normal to slightly enlarged kidneys	Central venous congestion in a hilar pattern in CXR	Hypercellularity of endothelial and mesangial cells, Infiltration of the glomerular tuft with polymorphonuclear cells	Histological confirmation	Hematuria
	Hematologic disease	Hemolytic-Uremic Syndrome	+	+	-	+	-	-	+	+	C5b-9, ADAMTS13	Anemia, Thrombocytopenia, Reticulocytosis	↑	↑Lactate dehydrogenase (LDH), Hypercalcemia	Thalami, brainstem, or cerebellum abnormality	Cerebral microangiopathy or hypertension	Hypoechoic kidney	Abnormal hyperintensity in the brain cisterns in MRI	Microthromboses include fibrin thrombi that may occlude the glomerular tuft	Clinical findings coupled with laboratory abnormalities	Hematuria, Proteinuria
		Chronic Lymphocytic Leukemia (CLL)	+	+	+	+	+/-	-	-	+/-	CD5, CD19, CD20, IgVH	Absolute lymphocytosis, Smudge cells	↑	Flow cytometry	Staging	-	-	-	Large atypical cells, cleaved cells, and prolymphocytes	Chromosomal and genetic testing	Easy bruising
		Multiple Myeloma	+	-	+	+	+	+	+/-	+/-	Ig light chain	Anemia, Thrombocytopenia, Leukopenia	↑	Bone marrow aspiration and biopsy, ↑Cr	Osseous involvement and lytic lesions	Peripheral zone of increased vascularity in lesions	-	Punched-out lesion in skull X-ray	Clonal proliferation of plasma cells	Protein electrophoresis plus conventional X-rays	Constipation
		Hypereosinophilic Syndrome	+/-	+/-	-	-	-	-	-	-	IgE, CD117 with CD2	Eosinophilia	-	↑Serum tryptase	Lymphadenopathy and splenomegaly	-	Intracardiac thrombi in echocardiography	-	Reticulin stain for myelofibrosis and tryptase staining for mast cells	Clinical findings coupled with laboratory abnormalities	Splinter hemorrhages, Raynaud phenomenon
		Non-Hodgkin Lymphoma	+	+	+	+	+/-	+/-	+/-	+/-	MYC, BCL2, BCL6, and TP53	Lymphocytosis, Anemia, Thrombocytopenia	↑	↑Lactate dehydrogenase (LDH), Hypercalcemia	Enlarged lymph nodes, Hepatosplenomegaly, Filling defects in the liver and spleen	-	Hepatosplenomegaly	Mediastinal lymphadenopathy	Small cleaved or noncleaved, intermediate, or large cell with a follicular or diffuse pattern	Surgically excised tissue biopsy	Easy bruising, Testicular mass, Skin lesion
		Serum Sickness	+	+	-	+/-	+/-	-	-	+/-	IL-1, IL-6, TNF	Leukopenia	↑	Polyclonal gammopathy, ↑Cr, Cryoglobulinemia	-	-	-	-	Arteritic lesions are focal, necrotizing, and inflammatory involving all layers of the artery	Clinical findings coupled with laboratory abnormalities	Hematuria, Skin rash
		Disseminated Intravascular Coagulation	+/-	+	-	+/-	-	-	+	+	Fibrin degradation product (FDP)	Thrombocytopenia, Schistocytes	↑	↑D-dimer, aPTT and PT	Intracranial hemorrhage	-	-	-	Ischemia and necrosis due to fibrin deposition in small and medium-sized vessels	Clinical findings coupled with laboratory abnormalities	Acral cyanosis, Hemorrhagic skin infarctions
		Idiopathic Thrombocytopenic Purpura	+	+/-	-	+	-	-	-	+	FC gamma receptors (FCGR) IIb	Anemia, Thrombocytopenia	-	HIV, ANA	R/O other causes	-	R/O splenomegaly	-	Increased number of normal morphologic megakaryocytes	Clinical findings coupled with thrombocytopenia	Easy bruising, Purpura
	Systemic disease	Sarcoidosis	+	+	+	+	-	-	-	+/-	IL-2 and IFN-γ	Mild anemia	↑	↑ACE, ↑1, 25-dihydroxyvitamin D	Active alveolitis or fibrosis	-	Hepatosplenomegaly	Bilateral hilar adenopathy	Noncaseating granulomas (NCGs)	Histological confirmation	Heart block, Ocular lesion
		Legionella Infection	+	+	+	+	-	-	-	+/-	Inflammatory cytokines	Leukocytosis with left shift, Thrombocytosis	↑	↑D-dimer, FDP, Hyponatremia	Pleural effusion	-	-	Nonspecific and indistinguishable CXR	Intra-alveolar inflammation, Microabscesses in the parenchyma	Sputum culture	Cough, Diarrhea
		Systemic lupus erythematosus	+	+	+	+	-	-	+	+	Anti dsDNA, ANA	Leukopenia, Lymphopenia, Anemia, Thrombocytopenia	↑	↑Cr or BUN, ↑ALT or AST, Proteinuria	Interstitial lung disease, Pneumonitis, Pulmonary emboli, Alveolar hemorrhage	Aneurysms	Pericardial effusion, pulmonary hypertension, or verrucous Libman-Sacks endocarditis in echocardiography	Central nervous system (CNS) lupus white-matter changes in MRI	Staging lupus nephritis	Anti-dsDNA antibody test	Skin rashes or photosensitivity
		Rheumatoid arthritis	-	-	+	+	+	+	-	-	RF, Anti-CCP antibody	Anemia	↑	↑Cr or BUN, ↑ALT or AST, ANA	Microfractures	Aneurysms	Effusions in joints	Basilar invagination with cranial migration of an eroded odontoid peg in MRI	Influx of inflammatory cells into the synovial membrane, with angiogenesis, proliferation of chronic inflammatory cells	Clinical findings coupled anti-CCP antibody	Rheumatoid nodules
		Relapsing polychondritis	-	+/-	+/-	+	+	-	-	-	-	Leukocytosis, Anemia	-	Cryoglobulins, ANA, C-ANCA	Calcification of cartilaginous structures	Aortic root dilatation	Aortic root dilatation and degree of aortic regurgitation in echocardiography	Tracheal stenosis in CXR	Chondrolysis, Chondritis, Perichondritis	Clinical findings coupled with imaging	Ear pain and redness, Polyarthritis

Epidemiology and Demographics

• The incidence of relapsing polychondritis is approximately 4 per 100,000 individuals in Minnesota.

Age

• Relapsing polychondritis is more commonly observed among patients aged 40 to 60 years but it can also occur in childhood.

Gender

• Relapsing polychondritis affects men and women equally.

Race

• Relapsing polychondritis usually affects Caucasian.

Risk Factors

Common risk factors in the development of relapsing polychondritis include:

• Genetic susceptibility:
  • Increased risk with HLA DR4
• History of other connective tissue disorders

Natural History, Complications and Prognosis

• The majority of patients with relapsing polychondritis remain asymptomatic for several years.
• Early clinical features include ear pain, polyarthritis and other non-specific symptoms such as fever, weight loss and skin rash.
• Common complications of relapsing polychondritis include collapsing of cartilage which causes facial abnormalities, deformities of the ear, eye, and destruction of the thyroid gland.
• Relapsing polychondritis has the relapsing and remitting course, Prognosis depends upon the type of organ involvement, stage of the disease.

Diagnosis

Diagnostic Criteria

Mc Adam diagnostic criteria for relapsing polychondritis
Criteria	Requirement
Recurrent chondritis of both auricles	3 out of 6 features are required for definitive diagnosis of relapsing polychondritis
Non-erosive inflammatory polyarthritis
Nasal chondritis
Inflammation of auricular structures
Chondritis of the respiratory tract
Cochlear and/or vestibular damage

According to modified Damiani criteria, there should be one of the following findings to diagnose relapsing polychondritis:

• There should be three of McAdam's diagnostic criteria.
• One or more of the clinical findings of McAdam's diagnostic criteria and positive histologic confirmation.
• Chondritis at two or more separate anatomic locations with a response to glucocorticoids or dapsone.

Symptoms

Symptoms of relapsing polychondritis may include the following:

• Ear pain
• Eye pain, redness, and increased lacrimation
• Decreased visual acuity
• Proptosis
• Rhinorrhea
• Epistaxis
• Pain on the movement of joint
• Joint swelling
• Cough
• Shortness of breath
• Dysphagia
• Chest pain
• Headache
• Confusion

Physical Examination

• Patients with relapsing polychondritis usually appear lethargic.
• Physical examination may be remarkable for:
• Ear examination:
  • On inspection, there is inflammation and swelling
  • On palpation, there is tenderness
• Eye examination:
  • On inspection, lid retraction, redness of the eye, ptosis, and proptosis
  • Visual acuity is decreased
• Lung examination:
  • On auscultation, wheezing and expiratory stridor is found
• Heart examination:
  • S1, S2 is normal, the murmur can be heard depending on the involvement of valve
• Joint examination:
  • On inspection, there is redness and swelling
  • On palpation, there is tenderness

Laboratory Findings

• There are no specific laboratory findings associated with relapsing polychondritis.
• Abnormal laboratory findings consistent with the diagnosis of relapsing polychondritis include:
  • CBC with differentials
    • Low hemoglobin
    • Leukocytosis
    • Eosinophilia
  • ESR and CRP are usually raised
  • Antibody testing
    • Anti-type II collagen antibodies is usually raised in early phase of disease
    • Antineutrophil cytoplasmic antibodies is found in patients who has vasculitis
  • Urinary glycosaminoglycans levels may be elevated
  • Urinary collagen type II neoepitope levels is elevated in active inflammation
    • Levels of urinary collagen type II neoepitope is used to assess response to treatment
  • Serum levels of cartilage oligomeric matrix protein is elevated and used as a marker of disease activity

Imaging Findings

• X-ray of the chest shows tracheal stenosis.
• CT scan of the chest shows following findings:
  • Subglottic stenosis
  • Thickening of anterior and lateral tracheal wall with sparing of the posterior membranous wall

Relapsing polychondritis
Source: Case courtesy of Dr Charlie Chia-Tsong Hsu

• MRI is better than CT scanning to see the difference between edema, fibrosis, and inflammation

Other Diagnostic studies

• ECG and Echocardiography are done in the patient who has cardiac involvement.
• Bronchoscopy used to visualize extensive disease involving the lungs.

Treatment

Medical Therapy

• The mainstay of therapy for relapsing polychondritis is medical therapy.
• NSAIDs are used as an initial treatment of relapsing polychondritis.
• Preferred regimen: Naproxen 500 mg PO q12h or ibuprofen 800 mg PO q6h x 7 to 10 days.
• If NSAIDs are contraindicated or resistant then following regimens are used:
  • Preferred regimen: Dapsone 50 to 100 mg PO q24h x 4 months depending on the severity of the disease.
  • Preferred regimen: Prednisone 30 to 60 mg PO q24h in divided dose.

Treatment of relapse:

• Preferred regimen: Prednisone 5 to 7.5 mg PO q24h

Treatment of life-threatening organ damage:

• Preferred regimen: Initial therapy is the combination of prednisone 1 mg/kg PO q24h and cyclophosphamide 2 mg/kg PO q24h.
• Maintenance therapy depends on the renal function of a patient.
• Patient without nephritis, Preferred regimen: Methotrexate 15mg PO once weekly.
• Patient with nephritis, Preferred regimen: Azathioprine - dose is adjused according to GFR.

Surgery

Medical therapy is the mainstay of therapy for relapsing polychondritis, however various surgical options include:

• Tracheostomy
• Cardiac valve replacement
• Aortic aneurysm repair
• Saddle-nose deformity repair

Prevention

There are no primary preventive measures available for relapsing polychondritis.

External links

• Polychondritis Educational Society, Ltd. (PES)
• Dr. D. E. Trentham research paper
• MedicineNet.com
• The Polychondritis Group - Support Group

Template:Osteochondropathy Template:Diseases of the musculoskeletal system and connective tissue