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CPC

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Luis Arias

on 19 September 2015

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Transcript of CPC

Fungi
Fever
Lymphadenopathy
Weight loss
Fatigue
General
Pulmonary
Ileitis
Colitis
Cardiac
CPC
Case
Discussion
Final 3...
References
presentation...
Luis Arias PGY3
38-year-old Caucasian man with an uneventful medical history was referred from his primary care physician for fever and joint pains.
About 5 months before referral, he developed a cough, sore throat, hoarseness, nasal congestion, red eyes, redness of his right ear, and slightly enlarged submandibular glands.
He also had temperatures as high as 103.1°F. He complained of pain in his hands, wrists, shoulders, and knees. The pain would resolve within days with anti-inflammatory medications or spontaneously over about a week without medication but would return a few weeks later. When it occurred it was mildly painful without erythema.
A few days prior to admission he developed mild to moderate sternal and anterior chest pains associated with mild shortness of breath. He was referred to Mount Sinai Medical Center for further workup. He brought a copy of reports from his primary care physician done 4 months ago, which included a chest radiograph and plain CT scan that were interpreted as normal.
Past Medical and Surgical History:
No known medical problems and no past surgical procedures
Medications:
Occasionally takes nonsteroidal anti-inflammatory drugs for joint pains
Allergies:
No known drug allergies
Family History:
Unremarkable
Social History
Single. Heterosexual male, history of unprotected sex, smokes ½ pack of cigarettes since he was 18years old, drinks alcohol socially, denies drug use.
Ocupation
Civil engineer – He currently spends most of his days working on a water source project underground in caissons
Review of systems
As per the HPI and a 15lb weight loss over 5 months.
Physical examination:
VITALS: Temperature was 100.4°F; Saturation 92% on RA; other vital signs were normal.

GENERAL: Revealed a man with a good general appearance.

HEENT: Erythematous conjunctivae. Questionable appearance of a saddle shaped deformity of the nose (the intern says it is, the resident says it’s not).

CARDIAC: Heart sounds were normal, II/VI holosystolic murmur at the apex.

LUNGS: Breath sounds were normal at quiet breathing, but at forced inspiration, there was a discrete stridulous sound.

ABDOMEN: No hepatosplenomegaly, benign

EXTREMITIES: Normal. No rashes.

JOINTS: Mild tenderness in the wrists, knees, and shoulders, mild edema, no erythema.

CNS: Negative
Labs:
Hb: 11.1 g/dl
WBC: 14 x 10 cells/ml with normal differential
PLT: 480.000 cells/ml
ESR: 147 mm/hr
Viral serology was negative except for + Epstein Barr IgG
Cultures from sputum, feces, urine and blood were negative
Normal BMP
Slight increase in AST and ALT
Polyclonal Hypergammaglobulinemia
Imaging
Chest radiograph was normal

CT scan of the chest showed what appeared to be a thickened tracheal wall starting from glottis and continued to the main bronchi
Gallium scan showed increased uptake at the nose and in the areas of the sternal and costal junctions
36 yo man with joint pains and fever
Had "flu-like symptoms" with a red ear? along with lymphadenopathies
Also had chest pains with shortness of breath and thickening of the trachea
Saddle nose
Systolic murmur at the apex
Hypergammaglobulinemia and elevated ESR
Gallium uptake in nose, sternal and costal junctions
In sumary...
Useful tools to approach polyarticular disease
Age and sex of the patient
Onset of symptoms
Degree and of inflammation of the joints
Temporal pattern of joint involvement
Distribution of joints affected
Asociated clinical manifestations
Migratory
Additive
Intermittent
Polyarticular joint pain
Systemic rheumatologic disorders
RA
SLE
Polymyositis/Dermatomyositis
Scleroderma
Behcet disease
Polymyalgia rheumatica
Sgojren's syndrome
Relapsing polychondritis
1. Malar rash: butterfly-shaped rash across cheeks and nose
2. Discoid (skin) rash: raised red patches
3. Photosensitivity: skin rash as result of unusual reaction to sunlight
4. Mouth or nose ulcers: usually painless
5. Arthritis (nonerosive) in two or more joints, along with tenderness, swelling, or effusion. With nonerosive arthritis, the bones around joints don’t get destroyed.
6. Cardio-pulmonary involvement: inflammation of the lining around the heart (pericarditis) and/or lungs (pleuritis)
7 Neurologic disorder: seizures and/or psychosis
8. Renal (kidney) disorder: excessive protein in the urine, or cellular casts in the urine
9. Hematologic (blood) disorder: hemolytic anemia, low white blood cell count, or low platelet count
10. Immunologic disorder: antibodies to double stranded DNA, antibodies to Sm, or antibodies to cardiolipin
11. Antinuclear antibodies (ANA): a positive test in the absence of drugs known to induce it.
SLE Criteria
Insiduous onset 70%, subacute 20% and acute is about 10%
Symmetric pattern with sweeling, stiffness, erosions
Usually high titers of RF

Extraarticular manifestations:
Dermatologic
Palmar erythema
Subcutaneous nodules
Vasculitis
Ocular
Episcleritis
Scleritis
Choroid and retinal nodules
Pleuritis
Nodules
Interstitial lung disease
Broncheolitis obliterans
Arteritis
Cardiac
Pericarditis
Myocarditis
Coronary vasculitis
Nodules on valves
Neuromuscular
Entrapment neuropathy
Peripheral neuropathy
Mononeuritis multiplex
Hematologic
Felty's syndrome
LGLS
Lymphomas
Other
Amyloidosis
Sjogren's syndrome
Crystal induced synovitis
Others
Systemic vasculitis
Henoch-Schonlein purpura
Wegener's granulomatosis
Hypersensitivity vasculitis
Polyarteritis nodosa
Giant cell arteritis
Spondyloarthropathies
Ankylosing spondylitis
Psoriatric arthritis
Inflamatory bowel disease
Reactive arthritis
Endocrine disorders
Hyperparathyroidsm
Hypothyroidsm
Hyperthyroidsm
Malignancy
Metastatic cancer
Multiple myeloma
Infections
Adapted from: Tan, E.M., et. al. The 1982 Revised Criteria for the Classification of SLE. Arth Rheum 25: 1271-1277.
General
Gout
Pseudogout
Viral
Usually short lived and self limited
Symmetrical. No erosions
No chronic disease
No specific therapy
Coxsackievirus
Enterovirus
Adenovirus
Epstein-Barr
Parvovirus B-19
Hepatitis B, C, A
Cytomegalovirus
Rubella
Mumps
HIV
HBV is primarily transmitted parenterally. Less commonly sexually

Arthralgias are estimated to occur in 20% of patients

Joint symptoms typically are present during prodromal phase and precede clinical jaundice by days or weeks

Usually of rapid onset. Pattern is symmetrical additive or migratory

Commonly involve small joints of the hands and knees

Early morning stiffness is common

Believed to be caused by immune complexes deposition in synovial tissue, leading to a secondary nonspecific inflammatory response
Usually transmitted by respiratory secretions

Up to 60% of adult patients develop joint symptoms

Preceded by a "flu-like illness"

Presents as a rapid onset symmetrical polyarthritis involving small joint of hands and wrists

May mimic RA and SLE

Diagnosis is made with serology (IgM)
Centrifugal maculopapular rash starting on the face (sparing palms and soles)

Significant lymphadenopathy

Symmetrical, small and large joints

Tenosynovitis and Carpal tunnel syndrome are common

May occur in 25% of adults after vaccine administration
Bacterial
Direct invasion to the joint
Septic arthritis (usually monoarticular)

Gonococcal

Non gonococcal
S. Aureus
Beta hemolytic streptococci
Gram negative bacilli
Streptococcus pneumoniae
Polymicrobial
Salmonella enteritidis
Salmonella typhimurium
Yersinia enterocolitica
Campylobacter jejuni
Chlamydia trachomatis
Clostridium difficile
Shigella sonnei
Entamoeba histolytica
Cryptosporidium
N. gonorrheae
Indirect through induced inflammation
Reactive arthritis (usually polyarticular and asymmetrical)
Acute Rheumatic Fever
Primarily affects young adults (20-40 yo)
Sterile inflammatory synovitis following an infection of a surface mucose
Classical triad of urethritis, conjunctivitis and arhtritis
Susceptibility may be conferred by HLA-B27
Symptoms usually start within 1-4 week of inital infection
General
Low grade fever
Weight loss
Ocular
Sterile conjunctivitis
Anterior uveitis
Gastrointestinal
Heart block
Aortic regirgitation
Aortitis
Peicarditis
Genitourinary
Urethritis
Prostatitis
Hemorrhagic cystitis
Salpingitis, vulvovaginitis
Mucocutaneous
Circinate balanitis
Keratoderma blennorrhagicum
Painless oral ulcers
Musculoskeletal
Arthritis (oligoarticular, asymmetric)
Enthesitis (Achilles tendon)
Spondilitis
Acute Rheumatic Fever
Systemic inflammatory disease occuring as a delayed complication of pharyngeal infection with group A streptococci

Multiple organ system involved
Supporting evidence of GAS
Major manifestations are:
Carditis
Polyarthritis
Chorea
Erythema marginatum
Subcutaneous nodules
Minor manifestations 
Arthralgia        
Fever
Laboratory findings:
Elevated acute phase reactants (ESR, C-reactive protein)        
 Prolonged PR interval
Other
Spirochettes
M. tuberculosis
Borrelia burgdorferi
Treponema pallidum
Bone/joints disease is seen in 1-3% of cases
Risk factors for ostearticular tuberculosis
Alcoholics
Drug abusers
HIV
Immunosupressed patients
Spine involvement (Pott's disease) accounts for 50%
Peripheral joint involvement accounts for 30%
Arthritis is monoarticular, can lead to joint destruction
Typically affects weight bearing joints like hip, knee, ankle
adjacent osteomyelitis is common
Diagnosis made by sinovial biopsy and culture
Lyme Disease
Tick-borne infection
3 stages:
Early: Erythema chronicum migrans

Disseminated: CNS, Cardiac,Musculoskeletal

Late: Episodic attacks of asymmetric oligoarticular arthritis of large joints
Saddle nose
Wegener's granulomatosis
Syphilis
Relapsing polychondritis
Sexually transmitted disease cause by Treponema pallidum
Age group between 15-40 years
"The great impostor"
Primary syphilis
Chancre: single, nontender papule progressing to a eroded indurated lesion
Heals spontaneously after 3-7 weeks
Usually located in penis and labia, but can also be found in mouth and anal canal
Associated lymphadenopathy
Secondary syphilis
Develops after 4-10 weeks of initial lesion
May present in many different ways, usually with mucocutaneous features and lymphadenopathy
Rash may be papular, macular, pustular or mixed involving palm and soles
Constitutional symptoms as headaches, fever, sore throat are common
Tertiary syphilis
Gummatous lesions may be present in cartilage (saddle nose) and trachea
Cardiovascular
Neurosyphilis
Inflammatory disorder characterized by episodic and progressive destruction of cartilage in ears, nose and tracheobronchial tree
Peak age of onset is between 40-50 years
No sex or racial predominance
HLA-DR4 is more frequent in patients with RP than in normal individuals
Abrupt onset of cartilagenous inflammation in one or two sites
Fever, fatigue and weight loss may precede clinical features by several weeks
Pattern of episodic attacks and affected sites vary widely among patients
Clinical Manifestations
Auricular chondritis
Hearing loss
Nasal chondritis
Saddle nose deformity
Ocular disease
Respiratory disease
Arthritis
Aortic regurgitation
Vasculitis
40 85
10 30
25 55
20 30
20 50
25 50
35 50
- 5
3 10
Clinical features
Frequency %
Presenting Cumulative
Pathophysiology
Autoimmune etiology
Cellular mediated
Humoral immunity by Collagen type 2 antibodies
Inflammation leads to cartilage destruction and replacement by fibrotic tissue
Associated disorders
Systemic vasculitis
Rheumatoid arthritis
SLE
Sjogren's syndrome
Spondyloarthropathies
Behcet's syndrome
Inflammatory bowel disease
Primary biliary cirrhosis
Myelodisplastic syndrome
Internal Medicine
McAdam et al criteria (3 of 6 clinical features are present)


Bilateral auricular chondritis
Nonerosive seronegative inflammatory polyarthritis
Nasal chondritis
Ocular inflammation
Respiratory tract chondritis
Audiovestibular damage


Damiani and Levine criteria (1 of 3 conditions is met)


Three McAdam et al criteria
One McAdam et al criterion plus positive histology
Two McAdam et al criteria plus therapeutic response to corticosteroid or dapsone administration


Michet et al criteria (1 of 2 conditions is met)


Proven inflammation in 2 of 3 of the auricular, nasal, or laryngotracheal cartilages
Proven inflammation in 1 of 3 of the auricular, nasal, or laryngotracheal cartilages plus 2 other signs including ocular inflammation, vestibular dysfunction, seronegative inflammatory arthritis, and hearing loss
Parvovirus B19
Hepatitis B
Rubella
Fungi
Rheumatoid arthritis
http://emedicine.medscape.com/article/331475-overview

Relapsing polychondritis: reversible airway obstruction or asthma. Mohammad A, Ambrose N, Tuohy M, Conway R, Costello R, Kearns G.
Clin Exp Rheumatol. 2008 Sep-Oct;26(5):938-40.

Relapsing polychondritis-case series from South India. Ananthakrishna R, Goel R, Padhan P, Mathew J, Danda D.
Clin Rheumatol. 2008 Oct 23.

Relapsing polychondritis: a rare disease of multisystem involvement.
Paudyal BP, Karki A, Zimmerman M. JNMA J Nepal Med Assoc. 2007 Apr-Jun;46(166):81-3.
3
Generalized gonococcal infection
Urethritis

Maculopapular or vesicular rash

Arthritis may involve a single joint or multiple joints in a migratory pattern

Tenosynovitis is very common
Multisystem disease characterized by granulomatous vasculitis and inflammation of the upper and lower respiratory tract

Pauci-immune glomerulonephritis

C-ANCA, anti-proteinase 3 antibodies

Non-specific arthralgias and myalgias are common
Corticosteroids

Methrotexate

TNF inhbitors

IL-6 inhibitors
Treatment
Chondritis
Infectious perichondritis
Frostbite
Trauma
Final Diagnosis
Relapsing polychondritis
Thank you
M. Tuberculosis
Reactive arthritis
Thyroid acropachy
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