Rheumatology Flashcards
Rheumatoid arthritis typically presents:
morning stiffness > 1 hour,
symmetric joint swelling of the MCP and PIP joints, and systemic symptoms
Rheumatoid arthritis most common affected joints:
wrists, metacarpophalangeal joints, proximal interphalangeal joints and metatarsophalangeal joints
Extra articular manifestations of RA leading cause pf death ?
anything with CARDIAC
Extra articular manifestations of RA
Extra articular manifestations of RA
A 39-year-old woman presents to the emergency department with** fever, cough, and shortness of breath. **She reports developing flu-like symptoms 7 days ago but progressively worsened to the point where she experiences dyspnoea on exertion. Her cough is accompanied by a mild amount of yellow sputum. Past medical history is notable for a previous admission to the hospital for pneumonia 4 months ago and an admission for bacteremia6 weeks ago. She additionally has a history of IV heroin abuse, but her last use of heroin was 3 years ago. Temperature is 101.2°F (38.4°C), blood pressure is 104/70 mmHg, pulse is 102/min, and respirations are 20/min. Physical examination demonstrates coarse upper airway breath sounds over the right lower lung field. A faint 1/6 non-radiating systolic flow murmur is auscultated at the first right intercostal space. Abdominal examination is significant for moderate splenomegaly. Tenderness of the wrists and fingers is elicited on palpation, and range of motion is restricted. The patient comments that her range of motion and pain usually improve as the day goes on. Which of the following laboratory abnormalities is most likely to be found in this patient?
1Decreased anion gap
2Flow cytometry positive for CD11c and CD2 3Leukocytosis with left-shift
4Neutropenia
5Positive HIV serology Rheumatoid Arthritis
45 year oldcoal mine worker presents with cutaneous nodules, joint pain and occasional cough with dyspnoea. His chest radiograph shows multiple small (1-4cm) nodules in bilateral lung fields Some of the nodules show cavitation and specks of calcification. Most likely these features are diagnostic of
:A)Sjogren syndrome
B)Caplan syndrome (ra+pneumoconiosis)
C)Silicosis D)Wegenersgranulamatosis(lung+renal+eye+joint) Rheumatoid Arthritis
What is Caplan syndrome ?
Caplan syndrome, also known as rheumatoid pneumoconiosis, is the combination of seropositive rheumatoid arthritis and a characteristic pattern of fibrosis
Which of the following is not considered an extra-articular manifestation of Rheumatoid Arthritis?
a) Osteoporosis
b) Peripheral neuropathy
c) Cutaneous nodules
d) Pericardial effusions
e) Hepatomegaly
Hepatomegaly
The diagnosis of RA is confirmed ?
- with imaging,
- positive rheumatoid factor (RF) and/or anti-citrullinated peptide antibody (anti-CCP),
- elevated C-reative protein or erythrocyte sedimentation rate
clinical presentation + autoantibodies + systemic inflammation
RF is present in about 70% of patients with established rheumatoid arthritis, it is detected less frequently in early disease.
*Antibodies toCCP are usually present before the development of symptoms and have a 96% specificity for rheumatoid arthritis.
*However, up to 30% of patients with rheumatoid arthritis never develop RF or antibodies to CCP and are said to have seronegative disease
The radiographic hallmarks of rheumatoid arthritis are:
- soft tissue swelling o fusiform and periarticular; it represents a combination of joint effusion, oedema and tenosynovitis o this can be an early/only radiographic finding
- osteoporosis: initially juxta-articular, and later generalised; compounded by corticosteroid therapy and disuse
-
* joint space narrowing: symmetrical or concentric
4.** * marginal erosions**: due to erosion by pannus of the bony “bare areas” periarticular bony erosions
There is a predilection for:
* PIP and MCP joints (especially 2nd and 3rd MCP)* ulnar styloid* triquetrum
As a rule, the DIP joints are spared.
RA treatment?
Therapy may be with either a single csDMARDor a combination of csDMARDs, with or without a corticosteroid. Methotrexate is the drug of choice for most patients,andshould form the backbone of the regimen
- CONSERVATIVE: education, exercise, physical therapy, smoking
- MEDICAL : metrotexate +- corticoseteroides
- OPERATIVE : join replacemente surgery (failed conservative and medical)
ra in pregnancy treatment?
SULFAZALAZINE
O
HIDROXYCLOROQUINE
RA TX
METROTEXATE SIDE EFFECTS
GI symptoms, oral ulcers, macrocytic anemia, and hepatotoxicity+ add folic acid
32 year old woman presented with bilateral joint swellings of her proximal interphalangeal joints associated with a 45 minutes period of early morning stiffness of joints. She has obvious rheumatoid nodules and a high rheumatoid factor assay. Which of the following is the best treatment of choice
a. infliximab
b. sulfasalazine
c. methotrexate
d. prednisolone
e. Hydroxyurea
c. methotrexate
A patient with rheumatoid arthritis got a flare up and doctor wants to administer azathioprine. which screening test should be done before starting this drug?
b) Thiopurine methyltransferas
Male patient with RA on methotrexate and hydroxychloroquine, planning to start a family, how to manage:
a. stop methotrexate and continue hydroxychloroquine
b. stop hydroxychloroquine and continue methotrexate
c. stop both
d. shift to leflunomide
a. stop methotrexate and continue hydroxychloroquine
Despite the lack of data, most societies recommend thatmenstopmethotrexate3 to 4 months before fathering children because of the potential for impairedfertility. 4 Given the therapeutic benefit ofmethotrexatein IBD, more data are needed regarding its effect onmale fertility.
Raynaud phenomenon is characterized by discoloration of the extremities as a physiologic response to cold temperatures or emotional stress. Initially, digits turn white (indicating ischemia), blue (indicating hypoxia and cyanosis), and finally pink with re-warming (indicating reperfusion). Raynaud can be idiopathic or associated with conditions such as systemic sclerosis/limited scleroderma, systemic lupus erythematosus, mixed connective tissue disease, and cryoglobulinemia
Conservative management involves avoidance of cold temperatures and smoking cessation.
First-line pharmacologic management is dihydropyridine-type calcium channel blockers (i.e., nifedipine, nicardipine, or amlodipine).
A 3-year-old boy presents to the emergency department due to worsening joint pain, fever, and a new rash. He is accompanied by his mother who reports that he has been pointing at his knee and hip while crying. She believes he has been having this symptom for approximately 2 months. He has been having 1-2 fevers a day, and she reports seeing a “pink-like” rash. On physical exam, there is an evanescent salmon-colored rash on the left thigh. There is tenderness to palpation of the left knee and hip with limited range of motion. Lab results show an elevated ESR, CRP, and negative RF, POSITIVE ANA. Most probable diagnosis?
1 Septicarthritis
2 Juvenile Rheumatoid arthritis
3Psoriatic arthritis
4Systemic lupus erythematosus (SLE)
5 Viral Arthritis
2 Juvenile Rheumatoid arthritis
Juvenile Idiopathic Arthritis: clinical definition?
- 1 or more joints are involved for atleast 6 weeks in patients< 16 years of age
- morning stiffness and joint pain*
*** visual changes -uveiti
** MOST COMMON
evanescent and salmon-colored macular ras
ANA ->typically, in systemic and polyarticular JIA positive ANA
*RF and CCP -> normally negative
A boy with joint pains. ANA +veand RF –e. What’s the long-term complications?
A. Cardiomyopathy
B. Uveitis
C. Small bowel ulceration
Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease of childhood, with JIA-associated uveitis its most common extra-articular manifestation. JIA-associated uveitis is a potentially sight-threatening condition and thus carries a considerable risk of morbidity
A child presents to ursurgery with c/o joint pains. You find out that he also suffers from iron deficiency anaemia. what could be the most appropriate diagnosis?
a) juvenile rheumatoid arthritis
b) HSP
c)haemophilia
d)thrombocytopenia
a) juvenile rheumatoid arthritis
Which is not criteria for juvenile rheumatoid arthritis?
- Arthritis
- Iridocyclitis
- Positive Rf
- Unexplained fever for few days
- Splenomegal
Positive Rf
Rheumatic fever clinic :
Migratory joint pains, especially in the knees, ankles, and elbows / chest discomfort / heart failures (rare) / fever/ dyspnea/ new murmur firm and painless subcutaneous nodules over bony prominences/ erythema marginatum/ chorea.
Rheumatic fever TX :
benzathine benzilpeniciline IM unique dose
OA x ray signs:
4
LOSS
OA Affects what parts ?
The proximal interphalangeal joint (PIP)/DIP, foot, knee,and hip joints are commonly affected, while the metacarpophalangeal (MCP), shoulder, wrist, and elbow joints are rarely implicated.
OA - arthritis characteristics
- Symmetrical and poly-oligo articular.
- Worsens on initiating movement and loading the joint and eased by rest.
- Stiffness after activity (Mechanical pain)
- Affected joints: CMC of thumb, MTP great toe, DIP hands/ also Knee, hip, PIP, Cervical and lumbar Spine
OA - physical exam
bony swelling
*swelling of the distal interphalangeal (Herberdennodes)
*swelling of the proximal interphalangeal **(Bouchard nodes) **
OA - tx
Conservative= exercise and weight lossindicationfirst-line in the management of osteoarthritis*
Medical=
*Topical capsaicin or oral nonsteroidal antiinflammatorydrugs (NSAIDs)
*Paracetamol 1 gr PRN
*Duloxetine sss
*Intra-articular corticosteroid injection
Operative=orthopedic surgeryindicationin patients with advanced pain who are unresponsive to conservative and pharmacologic therapy
Gout
Gout occurs when the serum uric acid concentration is sufficiently elevated (usually greater than 0.42 mmol/L [7 mg/dL])
Physical exam ?
acute gout
** **typicallymono-articular
*e.g.,involvement of the first metatarsophalangeal joint(podagra)
** chronic tophaceous gout
** *subcutaneous nodules
*typically non-tender
*overlying skin can be taut
*abnormal color
*white or yellow deposits
Gout (Monosodium urate crystal disorder) diagnosis?
Aspiration of an affected joint, bursa or tophus is required to confirm the diagnosis of gout.
Synovial fluid analysis joint fluid aspiration and crystal analysis is the gold-standard negatively birefringent needle-shaped crystals under polarized light
Serum uric concentration should be measured in all patients with suspected gout. However, the presence of hyperuricaemiaalone is insufficient to diagnose gout. Monosodium urate crystals are not seen on plain X-ray because they are not radiopaque; however, a plain X-ray may be useful to identify joint damage due to goutXray findings: joint effusion (earliest sign)/ preservation of joint space until late stages of the disease/ an absence of periarticular osteopenia/ eccentric erosions the typical appearance is the presence of well-defined “punched-out” erosions with sclerotic margins in a marginal and juxta-articular distribution, with overhanging edges , also known as rat bite erosions
GOUT DX?
Making the diagnosis
*demonstrating monosodium urate crystals in an affected joint via polarizing light microscopy
*when this is not possible, the diagnosis can be clinically made.
GOUT TX
ACUTE =
local corticosesteorid inyection
colchicina NOT in kidney or heptatic disfunction
not allopurinol in acute
CHRONIC =
allopurinol (yes for renal impairment , just modify dose)
Pseudogout joint analysis =
caused by deposition of calcium pyrophosphate crystals
*crystal analysis will demonstrate weakly positive birefringent rhomboid crystals under polarized light
Pseudogout associated with other disease ?
Pseudogout is associated with hemochromatosis which presents with bronzing of the skin and diabetes from pancreatic damage.
** Bronzing of the skin and elevated blood glucose levels**
Clinical presentation pseudogout ?
chondrocalcinosis (cartilage calcification)
asyntomatic in most cases
syntomatic = acute
affects mostly the knee
joint pain and morning stiffness (pseudo rheumatoid arthritis)
Diagnosis Pseudogout=
A definitive diagnosis of calcium pyrophosphate deposition is made by identifying calcium pyrophosphate dihydrate crystals in synovial fluid.
*Radiography = to assess the affected joint: chondrocalcinosisand degenerative changes
*Arthrocentesis *confirms the diagnosis
*Gram stain and culture should always be performed since infection could co-exist
*leukocyte count is 2,000-100,000/mm3
*> 50% polymorphonuclear cells
*polarized microscopy demonstrates weakly positively birefringent rhomboid crystals
Treatment Pseudogout =
- Conservative
observationindication*in patients with asymptomatic chondrocalcinosis - Medical
nonsteroidal anti-inflammatory drugs (NSAIDs) Indomethacin / Paracetamol indication=
an initial treatment option for pseudogout
colchicineindication=an initial treatment option for pseudogout
glucocorticoidsindications=
*injections of the affected joint is typically used in patients with < 2 involved joints
*oral medications are typically used in patients with > 2 involved joints
GOUT VS SEUDOGOUT
SLE symptoms
serositis, oropharyngeal ulcers, anti-nuclear antibodies (ANA), photosensitivity (including a malar or discoid rash), hemolytic anemia, kidney disease among many other possible presenting symptoms
SLE antibody used to monitor disease activity?
anti-dsDNA antibody can be used to monitor disease activity
Major cause of death in SLE ?
with atherosclerotic cardiovascular disease the major cause of mortality in patients with SLE
same as RA, SLE, AND squizofrenia
Difference between RA and SLE arthritis ?
joint deformities in SLE are often reducible and infrequently erosive on plain radiographs
autoantibodies and disease associated
SLE diagnosis clinical + lab
clinical?
diagnosis confirmed with 4 or more criteria from RASHNIA4**
**Renal disease
A**rthralgias
**Serositis
H**ematologic abnormalities
**Neurologic abnormalities
I**mmunologic derangements
**Antinuclear antibodies
*4 types of rashes:
*Malar
*discoid
*photosensitive
*oral ulcers
SLE diagnosis clinical + lab?
lab=
-
antinuclear antibody (ANA)best initial test*
high sensitivity but low specificity -
anti-double-stranded DNA (dsDNA) antibodyoften rises during flares*high specificity but low sensitivity
MONITOR -
anti-Smith antibody (antibody to snRNPs)
*high specificity (more than anti-dsDNA) but low sensitivity - LOW complement levels during a flare*↓C3, C4, and CH
SLE TX =
antimalarials are often used alongside steroids for acute flares
*management is often dictated by specific organ involvement
Conservativeuse sunscreen and avoid sun exposureindicationsfor all patients
*Medical
A =non-steroidal anti-inflammatory drugs (NSAIDs)
indicationsarthralgias
B= antimalarialsindicationsdermatologic findings and joint painoften used in conjunction with other medications, including steroids
**hydroxychloroquinechloroquineside effectsrisk of retinopathy
C=steroidsindications*acute flares *prednison
DRUG INDUCED LUPUS
**(mnemonic: Cute CHIMPPP)
**
Common drugs that cause drug-induced lupus include
CUTE CHIMPPP
quinidine,
chlorpromazine,
hydralazine,
isoniazid,
methyldopa,
primaquine,
penicillamine,
procainamide
Antiphospholipid antibody syndrome
Prolonged ?
PTT (intrinsico) PROLONGED
all factor except VII and VIII
PT extrinsico 1,2,5,7,10
Antiphospholipid antibody syndrome
LAB
- Lupus anticoagulant
- anti b2 glycoprotein (IgG and IgM)
- anticardiolipin
Antiphospholipid antibody syndrome
TX
warfarin
*indications
*for non-pregnant patients
*prevention of thrombosis
*target INR 2-3
aspirin plus unfractionated heparin
indicationsfor pregnant patients
*prevention of pregnancy loss
Scleroderma (Systemic sclerosis)
arthirtis clinic=
utoimmune skin diseasecharacterized byprogressive hardening and induration of the skin and/or other structures, such as the subcutaneous tissues, muscles, andinternal organs.
It can present as a polyarthritis affecting the fingers in 25%
Scleroderma (Systemic sclerosis) triad
*autoimmunity
*noninflammatory vasculopathy
*collagen deposition with fibrosis
Scleroderma (Systemic sclerosis)
limited cutaneos scleroderma -> subtype is CREST syndrome=
Calcinosis cutis
*anti-Centromere antibody
*Raynaud
*↓ blood flow to skin from either cold temperatures or stress, which causes vasospasms
*colors of affected area, commonly the digits, change from white (ischemia) to blue (hypoxia) to red (re-perfusion)
*Esophageal
dysmotility
*Sclerodactyly
*Telangiectasia
Finger discomfort + arthralgia + GORD (+/-skin tightness) ?
scleroderma
Scleroderma (Systemic sclerosis)
LAB ->
- anti-Scl-70 (anti-DNA topoisomerase I)associated with systemic sclerodermain ~ 30% of patients
- anti-centromere autoantibodyassociated with limited scleroderma (CREST syndrome)*in ~ 50% of patients
- antinuclear antibodiesin ~ 90-95% of affected patientsspeckled or centromere patternnucleolar pattern is specific for systemic sclerosis
4. *↑ inflammatory markers
*erythrocyte sedimentation rate
*C-reactive protein
Scleroderma (Systemic sclerosis)
TX
BASED ON SYMPTOMATIC RELIEF
**
inmunosupressive = pulmonary system involved = METROTEXATE/CICLOFOSFAMIDA (refractory to metrotexate)**
ace inhibitor = captopril (scleroderma renal crisis)
antihistamines - prurito
calcium channel blockers - reynaud
ambrisentan and taladafil - pulmonary hipertension
Sjogren’s syndrome (SS) definition
Sjögren syndrome is a chronic autoimmune disease associated with lymphoid infiltration of the exocrine glands, particularly the salivary and lacrimal glands, leading to secretory gland dysfunction .
Sjögren with persisten glandular swelling + weight loss ?
primarily of B cell origin; persistent glandular swelling or abnormal weight loss should prompt further investigation
Sjogren’s syndrome (SS) dx clinic + gold standar?
**Dry eyes **necessitating the use of ocular lubricants several times a day), in association with polyclonal hypergammaglobulinaemia,
a positive antinuclear antibody (ANA), and the presence of antibodies to Ro (SS-A) and La (SS-B)
BIOPSY GLANDS = Lymphocytic infiltrate in the minor salivary glands on lip biopsy
DRY EYES + RO + LA + ANA
Sjogren’s syndrome (SS) TX
Medical artificial tears
*indication= dry eyes
*pilocarpine or cevimeline
*indication= dry eyes or dry mouth
*vitamin D supplementation
*indications=
for all patients (vitamin D deficiency may increase risk of neuropathy and lymphoma)
Cause of fetal heart block in pregnant women?
Circulating anti-Ro (SSA) and anti-La (SSB) antibodies in pregnant women with Sjögren’ssyndrome .
Cross PLACENTA
Ankylosing spondylitis
physical exam
Chronic inflammatory disorder that primarily affects the sacroiliac joint and spine
may have stooped posture
+ Schober testdemonstrates limited spine flexibility
decreased chest wall expansion
Ankylosing spondylitis characteristic symptoms
a gradual onset before the age of 40 years
*a duration of symptoms of longer than 3 months
*prolonged morning stiffness and night pain
improvement with excersice (because inflamatory)
most common extra articular manifestetation -> Ankylosing spondylitis
acute anterior uveitis.
Ankylosing spondylitis
best initial step in managment (treatment)
** radiograph of the lumbosacral spine** GOLD STANDARto look for signs of sacroiliitis which is typically the first radiographical finding.
RX -> “BAMBOO SPINE”
**The most accurate test is an MRI **of the sacroiliac join
ankylosing spondylitis pulmonary function test?
decreased vital capacity and total lung capacity but a normal FEV1/FVC ratio and DLCO
RESTRICTIVE DISEASE
ankylosing spondylitis
TX
ALL stop smoking, exercise and use nonsteroidal anti-inflammatory drugs (NSAIDs)
MEDICAL
1. FIRST LINE NSAIDS
2. TUMOR NECROSIS FACTOR TFN -> INHIBITORS
ADALIMUMAB
INFLIXIMAB
(for those who dont respond to NSAIDs)
- csDMARDS
non infectious urethritis,+ arthritis + conjunctivitis ?
**Reactive arthritis **is an autoimmune response to infection that classically involves a triad of non infectious urethritis, arthritis, and conjunctivitis
he disease is associated with preceding gastrointestinal infection with organisms such as Salmonella, Shigella, Yersinia, or Campylobacter as well as preceding genitourinary infection with the most common causative organism being Chlamydia. Diagnosis is primarily based on history and clinical presentation. Treatment involves treating any underlying infections and NSAIDs for symptom control.
Triad of arthritis, conjunctivitis and urethritis.
REITER SYNDROME
Reactive Arthritis
lab
Más sensible y más específico para dx RA ?
Más sensible= factor reumatoide
Más específico= anti ccp
Male patient with Ra
Infertility meds
Metrotexate
Sulfazalina
Cual med de RA puedes dar a largo plazo ?
Metrotexate
Prednisolona
Hidroxicloroquina
Hidroxicloroquina
Cuando inicias hidroxicloroquina referir a que especialista ?
Oftalmólogo
Fever+ dispnea + joints pain that migrate + eritema marginatum!!
Reumátic fever
Investigations in reumátic fever ?
Throat swab culture
ASTO
Gout prevention
Allopurinol pero si es alérgico
Febuxostat
Factores precipitantes GOTA
- alcohol
- diureticos LOOP y tiazidicos
- carnes
Systemic esclerodermia antibody
Serum anti-topoisomerase, also known as SCL-7
Lupus cual antibody es más específico y Cual más sensible?
Ana es más sensible
Anti Dns más específico
Esclerodermia CREST autoANTIBODY
Anti centrómero autoaintibody
Dermatomiositis vs poliomiositis
Dermato CD4 , niños, gotrons papules.
Debilidad proximal
Más vasculitis
Más cancer
Polio cd8 , adultos
Poliomiositis da que tipo de cáncer ?
Ovario + pulmón
Behcet vs lupus como diferenciar ?
Úlceras en la boca duelen en behcet
Hay biopsia con leucoclastic vasculitis.
The most common genitourinary pathogen associated with reactive arthritis i
Chlamydia trachomatis
Reactive arthritis following gastrointestinal infection affects males and females equally. The most common enteric pathogens are:
Salmonella typhimurium, Shigella flexneri, Yersinia enterocolitica and Campylobacter jejuni
keratoderma blennorrhagica. This is a pustular hyperkeratotic rash typically affecting the palms and soles of the feet
3 DISEASEAS THAT HAVE THIS :
- REACTIVE ARTHRITIS
- PSORIASIS
- SIFILIS
migratory arthritis that has localized suggesting a diagnosis of reactive arthritis, gonococcal arthritis, or septic arthritis. what to do first?
Prior to treatment, any patient with a red and hot joint should have arthrocentesis and fluid analysis performed and have a swab for gonorrhoaeand chlamydia sent off. Subsequently, the patient can be treated with broad-spectrum antibiotics and surgical washout (if indicated).
The radiograph of both hands demonstrates pencil-in-cup deformities of both thumbs and erosion of the DIP joint of left middle finger, making** psoriatic arthritis** (in the context of the scaly rashes on the bilateral elbows) the most likely diagnosis.
**acro-osteolysis **(resorption of the distal phalanx tuf
Psoriatic arthtitis tx
A) Nonoperative
NSAIDS, methotrexate, sulfasalazine, cyclosporine,TNF-alpha inhibitors
indicationsmainstay of treatmentsimilar to RA*
B)Operative
*digit fusion vs resection arthroplasty
indicationsadvanced joint disease
The most specific diagnostic test for dermatomyositis?
muscle biopsy showing an inflammatory myopathy with a lymphocytic infiltrate at the periphery of the muscle fascicle
Dermatomyositis is an autoimmune disorder that is 2-3 times more common in females. The classic presentation is proximal muscle weakness in the setting of a characteristic rash. The most commonly seen cutaneous features include :
- Erythema of the eyelids (heliotrope
- Thickened, red, scaly plaques of the knuckles and dorsal hands (Gottron’spapules;
-
Erythema of the elbows and knees (Gottron’ssign;
4.Erythema of the upper back/chest (shawl sign)
pts with dermatomyositis should be screende for malignancy of ?
colon and ovarian cancers which co-occur frequently.
Polymyositis/ Dermatomyositis
1. dx labs =
2. what is gold standar to confirm?
elevada CK, LDH, AST,ALT
muscle biopsy
Polymyositis/ Dermatomyositis TX
systemic steroids (high-dose)
+
hydroxychloroquine (skin manifestations)
recurrent diseasea : metrotexate
Chronic use of methotrexate
1-Hepatotoxicity
2-Folic acid deficiency
3-Myelosuppression with low platelet and white cell count.
4-Interstitial pneumonitis
5-Fatigue
6-Nausea and vomiting
7-Stomatitis or mouth ulcers.
metacarpophalangeal joint + wrists are warm and tender + swollen
RA
Normals ESR
Men < 50: 15 mm/hr.
Men > 50: 20 mm/hr
Women < 50: 20 mm/hr
Women > 50: 30 mm/hr
patients who are steroid dependent for surgery
1-Give intravenous 100 mg hydrocortisone pre-operatively and 100 mg IV hydrocortisone intra-operatively.
2-During first 24 hours post-op give intravenously 100 mg hydrocortisone 6-8 hourly. This can be reduced by half over the next 48 hours.
3-Then change it to oral when patient tolerates oral diet.
medications of
choice for lupus nephritis
cyclophosphamide and mycophenolate
precipitating factors for acute attack of gout
- Alcoholic Binge
– Drugs ( furosemide, thiazides, Low dose aspirin )
– Starvation
– Chronic kidney disease
– Myeloproliferative disorders
– Lymphoproliferative disorders
– Cytotoxic drugs
– Sugary soft drinks.
Uveoparotid fever or Heerfordt’s syndrome
rare manifestation of sarcoidosis
Uveitis
Parotiditis
Chronic fever
Palsy of facial nerves in some cases
elderly px + elevated ESR + CRP pain and stiffness in hips and shoulders in the morning
Polymyalgia rheumatica
Polymyalgia rheumatica + headache?
About 15% of patients with polymyalgia rheumatica also have giant cell arteritis
- Polymyalgia rheumatica symptoms
*muscle pain and stiffness in the neck, shoulders, or pelvis for > 2 week
Physical exam*normal muscle strength
Rapid response to corticosteroid therapy at the usual starting dose (egprednis(ol)one 15 mg orally, daily) =
Polymyalgia rheumatica
Polymyalgia rheumatica tx
Medica
systemic low-dose corticosteroids
**indicationinitial therapy for patients with polymyalgia rheumaticalow-dose steroid response is usually rapidconsider supplementing with calcium and vitamin D for prevention of osteoporosis
*methotrexate = respuesta inadecuada
si no responde a corticoides CONSIDERAR OTRO DX
Giant cell Arteritis = OLDER PEOPLE >50
(formerly known as temporal arteritis or cranial arteritis)
CLASSIC SYMPTOMS
systemic vasculitis that typically affects the cranial arteries, including the ophthalmic artery.
jaw claudication, severe headache, visual symptoms, scalp tenderness, or malaise) develop in a patient with polymyalgia rheumatica or a history of polymyalgia rheumatica
**Giant cell Arteritis
TX
Urgently refer any patient with suspected giant cell arteritis to a specialist because a delay in treatment can result in serious consequences such as blindness or ischaemicevent
*high-dose systemic glucocorticoids should be promptly administered even before the diagnosis is established
To prevent ischaemicevents, including ophthalmic vascular thrombosis, use aspirin concurrently with corticosteroid therapy
**Giant cell Arteritis
DX
Labs
**↑ erythematous sedimentation rate
**
*↑C-reactive protein
Temporal artery biopsyconfirms the diagnosis!!!!!!!
Behcet’sdisease
SYMPTOMS
PHYSYCAL EXAM
Arthritis / enthesitis/ can have vascular thrombosis or aneurysms from vasculitis
**recurrent oral aphthous ulcers
**
genital ulcers**
**bilateral ocular lesions
** *slit lamp testing
non granulomatous uveitis
**anterior uveitis
skin lesions = papulas and nodules
POLYARTERITIS NODOSA
ANCA -
ETHIOLOGY
not associated with ANCA antibodies
idiopathic (most common)
*secondary polyarteritis nodosa can result from:
*hepatitis B infection
*hepatitis C infection
*hairy cell leukemia
Polyarteritis nodosa (PAN)
most common affected organs?
The most commonly affected organs and tissues are the skin, nerves, gastrointestinal tract and kidneys.
polyneuropathy
articular pain
purpura
glomerulonephritis
fever
weight loss
PAN confirm dx?
Biopsy -> confirms the diagnosis
*must biopsy the clinically affected organ
PAN SUMMARY
PAN TX
- ** high-dose prednis(ol)one**
- intravenous methylprednisolone
- *Cyclophosphamide + prednis(ol)one and another immunomodulatory drug is almost always required
- ** high-dose corticosteroids with cyclophosphamide**
moderate to severe disease
- without evidence of major organ or body system involvement (eg disease affecting the skin only, disease characterised by systemic symptoms only) the standard treatment is** high-dose prednis(ol)one** as recommended for patients with disease not affecting a major organ or body system.
- *For diseaseaffecting a major organ or body system (eg patients with polyneuropathy, kidney or liver involvement) intravenous methylprednisolone is used instead.
- *Cyclophosphamide is added if a visceral organ is affected or if disease is progressive despite adequate corticosteroid therapy. In the maintenance phase, combination therapy with prednis(ol)one and another immunomodulatory drug is almost always required
- ** high-dose corticosteroids with cyclophosphamide**
indicationin patients with moderate to severe disease
Kawasaki
AGE GROUP
Kawasaki disease is a systemic vasculitis that typically affects the coronary arteries.
It occurs almost exclusively in children and is usually seen before 5 years of age
Kawasaki symptoms
CRASH and burn
- conjuntivitis
*Rash
*Adenopathy
*Strawberry tongue
*Hand and foot rash
*fever (burn)
Making the diagnosisdiagnostic criteria
*fever for 5 or more days (burn)
*4/5 of CRASH
Kawasaki IMPORTANT to assess for all patiens
Echocardiography
obtained at the time of diagnosis and again at 2 and 6-8 weeks after diagnosis
Kawasaki
Labs
*↑ inflammatory markers
*↑ C-reactive protein
*↑ erythrocyte sedimentation rate
*↑ platelet count (often at weeks 2-3)
*↑ white blood cells
*↑ liver transaminases
Kawasaki TX
- intravenous infusion of immunoglobulin
- intravenous methylprednisoloneis
- ** High dose aspirin** until fever resolved -> Low-dose aspirin is continued until follow-up echocardiography has been performed
The risk of coronary aneurysms in patients with Kawasaki disease is reduced (from around 30% to around 3%) by** intravenous infusion of immunoglobulin.
If **fever persists o**r recurs following a second dose of intravenous immunoglobulin,** intravenous methylprednisoloneis** used.
**High dose aspirin followed by Low-dose aspirin once the fever has resolved, when marked thrombocytosis is typical.*Low-dose aspirin is continued until follow-up echocardiography has been performed, typically 6 weeks after the resolution of fever. Aspirin may be stopped if no coronary artery lesions are detected. However, if lesions are detected, aspirin should be continued, and ongoing surveillance of the coronary arteries is necessary.
Henoch-Schonlein purpura (HSP)
TRIADA symptoms
also known as IgA vasculitis
- palpable simetric NO TENDER purpura LOWER LIMBS AND BUTTOCKS
- arthralgias lower extremities
- abdominal pain (colickly
+ hematuria +
Henoch-Schonlein purpura (HSP)
risk factors
also known as IgA vasculitis
risk factors
*fall and winter months
*family history
*upper respiratory tract infection
*especially with group A streptococcus or parvovirus B19
Henoch-Schonlein purpura (HSP)
ASSOCIATED CONDTITIONS?
*IgA nephropathy (Berger disease)
Henoch-Schonlein purpura (HSP)
TX
resolves spontaneously over time*typically lasts 4 weeks
SUPPORTIVE: hydratation, pain managment
proteinuria = ACE inhibitor
corticosteroids = persisten proteinuria despite ACE inhibitor
leucocytoclastic vasculitis in adults?
classic triad of non thrombocytopenic purpura (typically on the lower limbs extending to the buttocks), colicky abdominal pain and large-joint arthritis.
The arthritis can cause significant pain and swelling, but can also resolve relatively rapidly.
Henoch-Schonleinpurpura (HSP) in children is the same
Henoch-Schonleinpurpura (HSP)
dx
is clinical
but labs
normal platelet count*presence of thrombocytopenia should prompt investigation into other causes of palpable purpura
*normal coagulation profile
urine studiesproteinuria and hematuria indicates renal involvement
biopsy most accurate only if not certain (unnecessary)
Wegner’s granulomatosis (WG)
antibodies
antiproteinase-3 (PR3 ANCA)
or
c-ANCA
Wegner’s granulomatosis (WG)
symptoms
vasculitis + granulomatosis
upper and lower respiratory tract and kidneys
UPPER
sinusitis,otitis , epistaxis
perforation nasal septum
LOWER
hemoptysis, dyspnea, cough
RENAL
hematuria
SKIN
leucocytoclastic vasculitis
Wegner’s granulomatosis (WG)
DX
RX - if pulmonary involvment + CT
LABS : ESR , C REACTIVE
+ PR3 ANCA / C - ANCA
BIOPSY OF SITE OF ACTIVE DISEASE CONFIRMS (LUNG)
Wegner’s granulomatosis (WG)
TX
Medical
high-dose glucocorticoids indicationinitial treatment option in patients with non-life or organ-threateningdisease*typically given with methotrexate
high-dose glucocorticoids withcyclophosphamide or rituximab !!!!!!!!!
indication*initial treatment option in patients with life-or organ-threatening disease
PAN VS WEGNERS? difference
Polyarteritis nodosa normally involves renal and visceral vessels, but the lungs are spared.
WEGNERS vs microscopic polyangitis (churg-strauss)
Microscopic polyangiitis =
Tests for anti-myeloperoxidase or p-ANCA would be positive
Microscopic polyangiitis is NOT associated with nasopharyngeal symptoms.
Microscopic polyangiitis is characteristically associated with asthma, skin nodules/purpura, and eosinophilia.
