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Transcript of CPC
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
Occasionally takes nonsteroidal anti-inflammatory drugs for joint pains
No known drug allergies
Single. Heterosexual male, history of unprotected sex, smokes ½ pack of cigarettes since he was 18years old, drinks alcohol socially, denies drug use.
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.
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.
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
Slight increase in AST and ALT
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
Systolic murmur at the apex
Hypergammaglobulinemia and elevated ESR
Gallium uptake in nose, sternal and costal junctions
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
Polyarticular joint pain
Systemic rheumatologic disorders
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.
Insiduous onset 70%, subacute 20% and acute is about 10%
Symmetric pattern with sweeling, stiffness, erosions
Usually high titers of RF
Choroid and retinal nodules
Interstitial lung disease
Nodules on valves
Crystal induced synovitis
Giant cell arteritis
Inflamatory bowel disease
Adapted from: Tan, E.M., et. al. The 1982 Revised Criteria for the Classification of SLE. Arth Rheum 25: 1271-1277.
Usually short lived and self limited
Symmetrical. No erosions
No chronic disease
No specific therapy
Hepatitis B, C, A
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)
Symmetrical, small and large joints
Tenosynovitis and Carpal tunnel syndrome are common
May occur in 25% of adults after vaccine administration
Direct invasion to the joint
Septic arthritis (usually monoarticular)
Beta hemolytic streptococci
Gram negative bacilli
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
Low grade fever
Painless oral ulcers
Arthritis (oligoarticular, asymmetric)
Enthesitis (Achilles tendon)
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:
Elevated acute phase reactants (ESR, C-reactive protein)
Prolonged PR interval
Bone/joints disease is seen in 1-3% of cases
Risk factors for ostearticular tuberculosis
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
Early: Erythema chronicum migrans
Disseminated: CNS, Cardiac,Musculoskeletal
Late: Episodic attacks of asymmetric oligoarticular arthritis of large joints
Sexually transmitted disease cause by Treponema pallidum
Age group between 15-40 years
"The great impostor"
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
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
Gummatous lesions may be present in cartilage (saddle nose) and trachea
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
Saddle nose deformity
Humoral immunity by Collagen type 2 antibodies
Inflammation leads to cartilage destruction and replacement by fibrotic tissue
Inflammatory bowel disease
Primary biliary cirrhosis
McAdam et al criteria (3 of 6 clinical features are present)
Bilateral auricular chondritis
Nonerosive seronegative inflammatory polyarthritis
Respiratory tract chondritis
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
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.
Generalized gonococcal infection
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
C-ANCA, anti-proteinase 3 antibodies
Non-specific arthralgias and myalgias are common