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A. AMC - MCQ MATEO 2024 > Rheumatology > Flashcards

Rheumatology Flashcards

1
Q

Rheumatoid arthritis typically presents:

A

morning stiffness > 1 hour,
symmetric joint swelling of the MCP and PIP joints, and systemic symptoms

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2
Q

Rheumatoid arthritis most common affected joints:

A

wrists, metacarpophalangeal joints, proximal interphalangeal joints and metatarsophalangeal joints

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3
Q

Extra articular manifestations of RA leading cause pf death ?

A

anything with CARDIAC

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4
Q

Extra articular manifestations of RA

A
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5
Q

Extra articular manifestations of RA

A
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6
Q

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

A
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7
Q

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

A
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8
Q

What is Caplan syndrome ?

A

Caplan syndrome, also known as rheumatoid pneumoconiosis, is the combination of seropositive rheumatoid arthritis and a characteristic pattern of fibrosis

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9
Q

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

A

Hepatomegaly

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10
Q

The diagnosis of RA is confirmed ?

A
  1. with imaging,
  2. positive rheumatoid factor (RF) and/or anti-citrullinated peptide antibody (anti-CCP),
  3. 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

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11
Q

The radiographic hallmarks of rheumatoid arthritis are:

A
  1. 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
  3. * 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.

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12
Q

RA treatment?

A

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

  1. CONSERVATIVE: education, exercise, physical therapy, smoking
  2. MEDICAL : metrotexate +- corticoseteroides
  3. OPERATIVE : join replacemente surgery (failed conservative and medical)
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13
Q

ra in pregnancy treatment?

A

SULFAZALAZINE
O
HIDROXYCLOROQUINE

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14
Q

RA TX
METROTEXATE SIDE EFFECTS

A

GI symptoms, oral ulcers, macrocytic anemia, and hepatotoxicity+ add folic acid

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15
Q

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

A

c. methotrexate

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16
Q

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?

A

b) Thiopurine methyltransferas

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17
Q

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

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.

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18
Q

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

A

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).

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19
Q

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

A

2 Juvenile Rheumatoid arthritis

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20
Q

Juvenile Idiopathic Arthritis: clinical definition?

A
  • 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

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21
Q

A boy with joint pains. ANA +veand RF –e. What’s the long-term complications?

A. Cardiomyopathy
B. Uveitis
C. Small bowel ulceration

A

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

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22
Q

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

a) juvenile rheumatoid arthritis

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23
Q

Which is not criteria for juvenile rheumatoid arthritis?

  1. Arthritis
  2. Iridocyclitis
  3. Positive Rf
  4. Unexplained fever for few days
  5. Splenomegal
A

Positive Rf

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24
Q

Rheumatic fever clinic :

A

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.

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25
Rheumatic fever TX :
benzathine benzilpeniciline IM unique dose
26
OA x ray signs: ## Footnote 4
**LOSS**
27
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.
28
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
29
OA - physical exam
bony swelling *swelling of the distal interphalangeal (**Herberdennodes**) *swelling of the proximal interphalangeal **(Bouchard nodes) **
30
OA - tx
**Conservative**= exercise and weight loss*indication*first-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 surgery*indication*in patients with advanced pain who are unresponsive to conservative and pharmacologic therapy
31
# 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
32
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
33
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.
34
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)
35
Pseudogout joint analysis =
caused by deposition of calcium pyrophosphate crystals *crystal analysis will demonstrate weakly **positive** **birefringent** **rhomboid** **crystals** under polarized light
36
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**
37
Clinical presentation pseudogout ?
chondrocalcinosis (cartilage calcification) asyntomatic in most cases syntomatic = acute affects mostly the **knee** joint pain and morning stiffness (pseudo rheumatoid arthritis)
38
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**
39
Treatment Pseudogout =
1. Conservative *observation*indication*in patients with asymptomatic chondrocalcinosis 2. Medical **nonsteroidal anti-inflammatory drugs (NSAIDs) Indomethacin / Paracetamol** *indication= an initial treatment option for pseudogout* **colchicine***indication=*an initial treatment option for pseudogout ***glucocorticoids***indications= *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
40
GOUT VS SEUDOGOUT
41
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
42
SLE antibody used to monitor disease activity?
anti-dsDNA antibody can be used to monitor disease activity
43
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
44
Difference between RA and SLE arthritis ?
joint deformities in SLE are often **reducible** and **infrequently** **erosive** on plain radiographs
45
autoantibodies and disease associated
46
SLE diagnosis **clinical** + lab | clinical?
***diagnosis confirmed with 4 or more criteria from RASHNIA4** ***R**enal disease ***A**rthralgias ***S**erositis ***H**ematologic abnormalities ***N**eurologic abnormalities ***I**mmunologic derangements ***A**ntinuclear antibodies ***4** types of rashes: *Malar *discoid *photosensitive *oral ulcers
47
SLE diagnosis clinical + **lab**? lab=
1. *antinuclear antibody (ANA)***best initial test*** high sensitivity but low specificity 2. ***anti-double-stranded DNA (dsDNA)** antibody*often rises during flares*high specificity but low sensitivity **MONITOR** 3. **anti-Smith antibody (antibody to snRNPs)** *high specificity (more than anti-dsDNA) but low sensitivity 4. LOW complement levels during a flare*↓C3, C4, and CH
48
SLE TX =
**antimalarials** are often used alongside **steroids** for acute flares *management is often dictated by specific organ involvement *Conservative*use sunscreen and avoid sun exposure*indications*for all patients *Medical A =non-steroidal anti-inflammatory drugs (**NSAIDs**) *indications*arthralgias B= antimalarials*indications*dermatologic findings and joint pain***often used in conjunction with other medications, including steroids **hydroxychloroquine*****chloroquine***side effects*risk of retinopathy C=***steroids***indications*acute flares ***prednison**
49
DRUG INDUCED LUPUS
**(mnemonic: Cute CHIMPPP) ** Common drugs that cause drug-induced lupus include **CUTE CHIMPPP** quinidine, chlorpromazine, hydralazine, isoniazid, methyldopa, primaquine, penicillamine, procainamide
50
Antiphospholipid antibody syndrome Prolonged ?
PTT (intrinsico) PROLONGED all factor except VII and VIII | PT extrinsico 1,2,5,7,10
51
Antiphospholipid antibody syndrome LAB
1. Lupus anticoagulant 2. anti b2 glycoprotein (IgG and IgM) 3. anticardiolipin
52
Antiphospholipid antibody syndrome TX
**warfarin** *indications *for non-pregnant patients *prevention of thrombosis *target INR 2-3 **aspirin plus unfractionated** **heparin** *indications*for **pregnant** patients *prevention of pregnancy loss
53
Scleroderma (Systemic sclerosis) arthirtis clinic= ## Footnote 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%
54
Scleroderma (Systemic sclerosis) triad
*autoimmunity *noninflammatory vasculopathy *collagen deposition with fibrosis
55
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**
56
Finger discomfort + arthralgia + GORD (+/-skin tightness) ?
scleroderma
57
Scleroderma (Systemic sclerosis) LAB ->
1. **anti-Scl-70 (anti-DNA topoisomerase I)***associated with systemic scleroderma*in ~ 30% of patients 2. ***anti-centromere autoantibod**y*associated with limited scleroderma (CREST syndrome)*in ~ 50% of patients 3. ***antinuclear antibodies***in ~ 90-95% of affected patients*speckled or centromere pattern*nucleolar pattern is specific for systemic sclerosis **4. *↑ inflammatory markers** *erythrocyte sedimentation rate *C-reactive protein
58
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
59
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 .
60
Sjögren with persisten glandular swelling + weight loss ?
**primarily of B cell origin**; persistent glandular swelling or abnormal weight loss should prompt further investigation
61
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 a**ntinuclear 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
62
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)
63
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
64
Ankylosing spondylitis physical exam ## Footnote Chronic inflammatory disorder that primarily affects the sacroiliac joint and spine
may have stooped posture + Schober test*demonstrates limited spine flexibility* decreased chest wall expansion
65
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)
66
most common extra articular manifestetation -> Ankylosing spondylitis
acute anterior uveitis.
67
Ankylosing spondylitis best initial step in managment (treatment)
** radiograph of the lumbosacral spine** **GOLD STANDAR**to 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
68
ankylosing spondylitis pulmonary function test?
decreased vital capacity and total lung capacity but a normal FEV1/FVC ratio and DLCO RESTRICTIVE DISEASE
69
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) 3. csDMARDS
70
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 ## Footnote he disease is associated with preceding **gastrointestinal** infection with organisms such as **Salmonella, Shigella, Yersinia, or Campylobacte**r 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.
71
Triad of arthritis, conjunctivitis and urethritis. ## Footnote **REITER SYNDROME**
Reactive Arthritis
72
lab Más sensible y más específico para dx RA ?
Más sensible= factor reumatoide Más específico= anti ccp
73
Male patient with Ra Infertility meds
Metrotexate Sulfazalina
74
Cual med de RA puedes dar a largo plazo ? Metrotexate Prednisolona Hidroxicloroquina
Hidroxicloroquina
75
Cuando inicias hidroxicloroquina referir a que especialista ?
Oftalmólogo
76
Fever+ dispnea + joints pain that migrate + eritema marginatum!!
Reumátic fever
77
Investigations in reumátic fever ?
Throat swab culture ASTO
78
Gout prevention Allopurinol pero si es alérgico
Febuxostat
79
Factores precipitantes GOTA
- alcohol - diureticos LOOP y tiazidicos - carnes
80
Systemic esclerodermia antibody
Serum anti-topoisomerase, also known as SCL-7
81
Lupus cual antibody es más específico y Cual más sensible?
Ana es más sensible Anti Dns más específico
82
Esclerodermia CREST autoANTIBODY
Anti centrómero autoaintibody
83
Dermatomiositis vs poliomiositis
Dermato CD4 , niños, gotrons papules. Debilidad proximal Más vasculitis Más cancer Polio cd8 , adultos
84
Poliomiositis da que tipo de cáncer ?
Ovario + pulmón
85
Behcet vs lupus como diferenciar ?
Úlceras en la boca duelen en behcet Hay biopsia con leucoclastic vasculitis.
86
The most common genitourinary pathogen associated with reactive arthritis i
Chlamydia trachomatis
87
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
88
keratoderma blennorrhagica. This is a pustular hyperkeratotic rash typically affecting the palms and soles of the feet 3 DISEASEAS THAT HAVE THIS :
1. REACTIVE ARTHRITIS 2. PSORIASIS 3. SIFILIS
89
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).
90
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
91
Psoriatic arthtitis tx
A) Nonoperative *NSAIDS, methotrexate, sulfasalazine, cyclosporine,TNF-alpha inhibitors *indications*mainstay of treatment*similar to RA* B)Operative *digit fusion vs resection arthroplasty *indications*advanced joint disease
92
The most specific diagnostic test for dermatomyositis?
muscle biopsy showing an inflammatory myopathy with a lymphocytic infiltrate at the periphery of the muscle fascicle
93
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 :
1. **Erythema** of the **eyelids** (heliotrope 2. Thickened, red, scaly **plaques** of the knuckles and dorsal hands (Gottron’spapules; 3. **Erythema** of the **elbows** and knees (Gottron’ssign; 4.**Erythema** of the upper back/chest (shawl sign)
94
pts with dermatomyositis should be screende for malignancy of ?
**colon** and **ovarian** cancers which co-occur frequently.
95
Polymyositis/ Dermatomyositis 1. dx labs = 2. what is gold standar to confirm?
elevada CK, LDH, AST,ALT muscle biopsy
96
Polymyositis/ Dermatomyositis TX
**systemic steroids (high-dose)** + hydroxychloroquine (skin manifestations) recurrent diseasea : **metrotexate**
97
98
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.
99
metacarpophalangeal joint + wrists are warm and tender + swollen
RA
100
Normals ESR
Men < 50: 15 mm/hr. Men > 50: 20 mm/hr Women < 50: 20 mm/hr Women > 50: 30 mm/hr
101
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.
102
medications of choice for lupus nephritis
cyclophosphamide and mycophenolate
103
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.
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Uveoparotid fever or Heerfordt’s syndrome
rare manifestation of sarcoidosis Uveitis Parotiditis Chronic fever Palsy of facial nerves in some cases
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elderly px + elevated ESR + CRP pain and stiffness in hips and shoulders in the morning
Polymyalgia rheumatica
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Polymyalgia rheumatica + headache?
About 15% of patients with polymyalgia rheumatica also have **giant cell arteritis**
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* Polymyalgia rheumatica symptoms
*muscle pain and stiffness in the neck, shoulders, or pelvis for > 2 week Physical exam*normal muscle strength
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Rapid response to corticosteroid therapy at the usual starting dose (egprednis(ol)one 15 mg orally, daily) =
Polymyalgia rheumatica
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Polymyalgia rheumatica tx
Medica **systemic low-dose corticosteroids ***indication*initial therapy for patients with polymyalgia rheumatica*low-dose steroid response is usually rapid*consider supplementing with calcium and vitamin D for prevention of osteoporosis *methotrexate = respuesta inadecuada si no responde a corticoides CONSIDERAR OTRO DX
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Giant cell Arteritis = OLDER PEOPLE >50 (formerly known as temporal arteritis or cranial arteritis) CLASSIC SYMPTOMS ## Footnote 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
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**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
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**Giant cell Arteritis DX
*Labs ***↑ erythematous sedimentation rate ** *↑C-reactive protein ***Temporal artery biopsy*confirms the diagnosis**!!!!!!!
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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
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POLYARTERITIS NODOSA ANCA - ETHIOLOGY ## Footnote not associated with ANCA antibodies
idiopathic (most common) *secondary polyarteritis nodosa can result from: *hepatitis B infection *hepatitis C infection *hairy cell leukemia
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Polyarteritis nodosa (PAN) most common affected organs?
The most commonly affected organs and tissues are the skin, nerves, gastrointestinal tract and **kidneys**. ## Footnote polyneuropathy articular pain purpura **glomerulonephritis** fever weight loss
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PAN confirm dx?
Biopsy -> confirms the diagnosis *must biopsy the clinically affected organ
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PAN SUMMARY
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PAN TX
1. ** high-dose prednis(ol)one** 2. **intravenous** **methylprednisolone** 3. ***Cyclophosphamide** + **prednis(ol)one** and another immunomodulatory drug is almost always required 4. ** high-dose corticosteroids with cyclophosphamide** moderate to severe disease ## Footnote 1. 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. 2. *For diseaseaffecting a major organ or body system (eg patients with polyneuropathy, kidney or liver involvement) **intravenous** **methylprednisolone** is used instead. 3. ***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 4. ** high-dose corticosteroids with cyclophosphamide** *indication*in patients with moderate to severe disease
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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
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Kawasaki symptoms
**CRASH and burn** ## Footnote * conjuntivitis *Rash *Adenopathy *Strawberry tongue *Hand and foot rash *fever (burn) *Making the diagnosis*diagnostic criteria *fever for 5 or more days (burn) *4/5 of CRASH
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Kawasaki IMPORTANT to assess for all patiens
Echocardiography obtained at the time of diagnosis and again at 2 and 6-8 weeks after diagnosis
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Kawasaki Labs
*↑ inflammatory markers *↑ C-reactive protein *↑ erythrocyte sedimentation rate *↑ platelet count (often at weeks 2-3) *↑ white blood cells *↑ liver transaminases
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Kawasaki TX
1. intravenous infusion of immunoglobulin 2. intravenous methylprednisoloneis 3. ** High dose aspirin** until fever resolved -> **Low-dose aspirin is continued** until follow-up echocardiography has been performed ## Footnote 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.
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Henoch-Schonlein purpura (HSP) TRIADA symptoms ## Footnote also known as IgA vasculitis
1. palpable simetric NO TENDER purpura LOWER LIMBS AND BUTTOCKS 2. arthralgias lower extremities 3. abdominal pain (**colickly** + hematuria +
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Henoch-Schonlein purpura (HSP) risk factors ## Footnote 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**
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Henoch-Schonlein purpura (HSP) ASSOCIATED CONDTITIONS?
*IgA nephropathy (Berger disease)
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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
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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. ## Footnote Henoch-Schonleinpurpura (HSP) in children is the same
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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 studies***proteinuria and hematuria indicates renal involvement biopsy most accurate only if not certain (unnecessary)
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Wegner’s granulomatosis (WG) antibodies
antiproteinase-3 (PR3 ANCA) or **c-ANCA**
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Wegner’s granulomatosis (WG) symptoms ## Footnote 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
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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)**
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Wegner’s granulomatosis (WG) TX
Medical **high-dose glucocorticoids** *indication*initial 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
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PAN VS WEGNERS? difference
Polyarteritis nodosa normally involves renal and visceral vessels, but the lungs are spared.
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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.**
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A. AMC - MCQ MATEO 2024 (15 decks)

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