Medicine Marrow Pg No 547-556 (Rheumatology)

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Questions and Answers

Which area of the frontal lobe is primarily responsible for speech production?

  • Area 44 (correct)
  • Area 10
  • Area 4
  • Area 6

The lateral sulcus separates the temporal lobe from the frontal and parietal lobes.

True (A)

What is the function of Area 6 in the frontal lobe?

Premotor area

The __________ is the primary area for motor control in the frontal lobe.

<p>Primary motor area</p> Signup and view all the answers

Match the following areas of the frontal lobe with their functions:

<p>Area 8 = Frontal eye field Area 9 = Prefrontal area Area 44 = Motor speech area Area 4 = Primary motor area</p> Signup and view all the answers

Which of the following functions is NOT assessed by the mini-mental state examination (MMSE)?

<p>Vision (B)</p> Signup and view all the answers

The motor cortex is located in the parietal lobe.

<p>False (B)</p> Signup and view all the answers

What is the primary responsibility of the somatosensory cortex?

<p>Processing touch sensations</p> Signup and view all the answers

The _________ lobe is located at the back of the brain and is responsible for processing visual information.

<p>occipital</p> Signup and view all the answers

Match the following brain lobes with their respective characteristics:

<p>Frontal lobe = Largest lobe at the front of the brain Temporal lobe = Located below the frontal lobe Parietal lobe = Located behind the frontal lobe Occipital lobe = Located at the back of the brain</p> Signup and view all the answers

What is the typical white blood cell (WBC) count in crystal arthritis?

<p>10,000 - 50,000 cells/mD (D)</p> Signup and view all the answers

Presence of needle-shaped crystals is indicative of septic arthritis.

<p>False (B)</p> Signup and view all the answers

What microscopy technique is used to identify needle-shaped crystals in crystal arthritis?

<p>Polarized light microscopy</p> Signup and view all the answers

In gout, the baseline condition is typically _____ in nature.

<p>symptom-free</p> Signup and view all the answers

Match the condition to its feature:

<p>Crystal Arthritis = Presence of crystals Septic Arthritis = Positive culture &amp; gram stain Gout = Symptom-free baseline Palindromic Rheumatism = Variable course</p> Signup and view all the answers

What is a common treatment for mild Adult-Onset Still's Disease (AOSD)?

<p>NSAID (B)</p> Signup and view all the answers

Pancytopenia is a feature associated with Hemophagocytic Lymphohistiocytosis (HLH).

<p>True (A)</p> Signup and view all the answers

What condition is indicated by an elevated ferritin level in the context of Adult-Onset Still's Disease?

<p>Hemophagocytic Lymphohistiocytosis (HLH)</p> Signup and view all the answers

Septic arthritis is most commonly associated with _____ pain and limited range of motion.

<p>joint</p> Signup and view all the answers

Match the following causes of septic arthritis with their descriptions:

<p>Staphylococcus aureus = Most common bacterial cause IV drug abuse = Oligo/polyarticular pattern Gram-negative bacillus = Associated with pneumonia Immunocompromised = Higher risk for infections</p> Signup and view all the answers

What age range is most commonly affected by Adult Onset Still's Disease (AOSD)?

<p>25-45 years (D)</p> Signup and view all the answers

AOSD is characterized by a clinical triad of fever, rash, and arthritis.

<p>True (A)</p> Signup and view all the answers

What color and type of rash is associated with AOSD?

<p>Salmon-colored, transient, maculopapular</p> Signup and view all the answers

Increased levels of _____ is found in AOSD, indicating an inflammatory response.

<p>Ferritin</p> Signup and view all the answers

Match the findings of Adult Onset Still's Disease (AOSD) with their descriptions:

<p>Leukocytosis = Increased white blood cell count Serositis = Inflammation of serous membranes Hepatosplenomegaly = Enlargement of liver and spleen Elevated CRP = Indicator of inflammation</p> Signup and view all the answers

What is the first-line treatment for gout pain relief?

<p>Colchicine (C), Indomethacin (D)</p> Signup and view all the answers

Hyperuricemia is one of the ACR criteria for diagnosing gout.

<p>True (A)</p> Signup and view all the answers

What does the term 'intercritical period' refer to in gout?

<p>Symptom-free period</p> Signup and view all the answers

A significant characteristic seen in X-ray of chronic gout lesions includes _____ margins, known as Rat bite erosions.

<p>sclerotic</p> Signup and view all the answers

Match the following treatments with their respective classifications:

<p>Colchicine = First line treatment Anakinra = Second line treatment IV Pegloticase = Second line treatment NSAIDs = First line treatment</p> Signup and view all the answers

What type of crystals are deposited in Basic Calcium Phosphate disease?

<p>Calcium hydroxyapatite crystals (A)</p> Signup and view all the answers

Calcium oxalate crystals display strong negative birefringence.

<p>False (B)</p> Signup and view all the answers

What condition is associated with calcinosis cutis?

<p>CREST syndrome or juvenile dermatomyositis</p> Signup and view all the answers

The Milwaukee shoulder condition involves rotator cuff arthropathy and __________ destruction.

<p>glenohumeral</p> Signup and view all the answers

Match the following types of calcium oxalate to their classifications:

<p>Primary = Seen in hyperoxaluria Secondary = Seen in chronic kidney disease (CKD)</p> Signup and view all the answers

Which of the following is a common characteristic of septic arthritis?

<p>High WBC count (A), Thick pus in joint fluid (B)</p> Signup and view all the answers

Gonococcal arthritis typically affects older adults.

<p>False (B)</p> Signup and view all the answers

What is the gold standard for diagnosing septic arthritis?

<p>Synovial fluid analysis</p> Signup and view all the answers

Chronic monoarticular arthritis can be caused by _____ disease such as tuberculosis.

<p>infectious</p> Signup and view all the answers

Match the type of arthritis with its characteristic:

<p>Septic Arthritis = Monoarticular and high WBC count Gonococcal Arthritis = Migratory polyarthralgia Chronic Monoarticular Arthritis = TB or fungal disease Polyarticular Arthritis = Could be rheumatoid arthritis or SLE</p> Signup and view all the answers

What is typically observed in a presentation of acute monoarticular arthritis associated with CPPD?

<p>Rhomboid crystals with weak positive birefringence (A)</p> Signup and view all the answers

Chondrocalcinosis is symptomatic and usually causes significant pain in affected joints.

<p>False (B)</p> Signup and view all the answers

Which gene is associated with Calcium Pyrophosphate Dihydrate (CPPD)?

<p>ANKH</p> Signup and view all the answers

Pseudo-gout is associated with the presence of ______ crystals in joint fluid.

<p>rhomboid</p> Signup and view all the answers

Match the following presentations with their associated characteristics:

<p>Chondrocalcinosis = Asymptomatic calcification of articular cartilage Acute Monoarticular Arthritis = Rhomboid crystals with weak positive birefringence Chronic Inflammatory Polyarthritis = Waxing and waning progression Spondyloarthritis = Crowned dens syndrome</p> Signup and view all the answers

Which site is the most common for tophi deposition?

<p>Olecranon (B)</p> Signup and view all the answers

Tohpi formation is usually accompanied by acute pain and inflammation.

<p>False (B)</p> Signup and view all the answers

What are xanthine oxidase inhibitors used for in the treatment of crystal arthropathies?

<p>Uric acid lowering agents</p> Signup and view all the answers

The rate of tophus formation correlates with degree and duration of __________.

<p>hyperuricemia</p> Signup and view all the answers

Match the treatment type with its corresponding agents:

<p>Xanthine oxidase inhibitors = Allopurinol, Febuxostat Uricosuric agents = Probenecid, Sulfinpyrazone, Benzbromarone Recombinant uricase = Rasburicase, Pegloticase</p> Signup and view all the answers

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Study Notes

Areas on Frontal Lobe

  • Area 6: Premotor area
  • Area 8 (Medially): Supplementary motor area
  • Area 8: Frontal eye field
  • Area 9, 10, 11, 12: Prefrontal area
  • Area 44, 45: Motor speech area (Broca's area) located in the inferior frontal gyrus
  • Area 4: Primary motor area (Precentral gyrus)

Medial Surface

  • Precuneus
  • Parieto-occipital fissure
  • Cuneus
  • Calcarine fissure
  • Lingual gyrus
  • Paracentral Lobule
  • Superior Frontal Gyrus
  • Cingulate Gyrus
  • Cingulate sulcus
  • Corpus callosum
  • Fornix
  • Hippocampal gyrus
  • Fusiform gyrus
  • Inferior temporal gyrus

Inferior Surface

  • Gyrus rectus
  • Olfactory sulcus
  • Medial orbital gyrus
  • Anterior orbital gyrus
  • Post.orbital gyrus
  • Lateral orbital gyrus
  • Orbital sulcus (H-shaped)

Functional Frontal Lobe Anatomy

  • Lateral sulcus/Sylvian fissure: A fissure separating the frontal and parietal lobes from the temporal lobe

mini-mental State Examination (mmse)

  • Assesses higher mental functions/cognitive status
  • Based on: Orientation, Registration, Attention, Recall, Language, Copying

Superolateral surface

  • Frontal lobe: The largest lobe at the front of the brain.
  • Temporal lobe: Located below the frontal lobe.
  • Parietal lobe: Located behind the frontal lobe.
  • Occipital lobe: Located at the back of the brain.
  • Central sulcus: A groove separating the frontal and parietal lobes.
  • Sylvian fissure: A deep groove that separates the frontal and temporal lobes.
  • Motor cortex: Located in the frontal lobe, responsible for voluntary movement.
  • Somatosensory cortex: Located in the parietal lobe, responsible for processing touch sensations.
  • Visual cortex: Located in the occipital lobe, responsible for processing visual information.
  • Auditory cortex: (Partially visible) responsible for processing auditory information.

Adult-Onset Still's Disease & Septic Arthritis

Minor Findings:

  • Sore throat
  • Negative ANA, RF

Hemophagocytic Lymphohistiocytosis (HLH)

  • Rheumatological emergency
  • Seen in 10% of AOSD
  • Hepatosplenomegaly, lymphadenopathy
  • Abnormal ALT/AST

Features:

  • Pancytopenia
  • Unsubsiding fever
  • Ferritin ↑

Complications:

  • Bleeding (due to thrombocytopenia)
  • Disseminated Intravascular Coagulation (DIC) with consumptive coagulopathy

Treatment:

  • Steroid
  • Pulse therapy → Oral therapy
  • ESR ↓
  • Fibrinogen ↓
  • Triglycerides ↑
  • Transient thrombocytopenic purpura
  • AA Amyloidosis

TREATMENT OF AOSD:

  • Mild: NSAID
  • No response: Steroid + methotrexate
  • Anti IL-1, IL-6 (Note: Sulfasalazine is avoided)

Septic Arthritis

Acute Monoarticular Arthritis

Susceptible Host:

  • Children
  • Elderly
  • Immunocompromised

Causes:

  • Infection (20%)
    • Staphylococcus aureus > Streptococcus
    • Pneumonia > Gram-negative bacillus
  • IV drug abuse (oligo/polyarticular)
  • Spread: Hematogenous

Clinical Presentation:

  • Fever
  • Pain + Limited range of motion (most common in the knee)
  • No history of similar episode
  • Trauma
  • Tumor
  • Osteoarthritis of a single joint
  • Sarcoidosis

Crystal Arthropathies

Subsequent Attacks:

  • Involvement of ankle, knee, or wrist.
  • Variable course.

Investigation: Synovial Study

Definitive Diagnosis (a.Cytology, Culture & Gram Stain):
Feature Crystal Arthritis Septic Arthritis
WBC (cells/mD) 10,000 - 50,000 >50,000
Focus of infection - +
Culture & gram stain Negative Positive
Other features Presence of crystals Immunosuppression +
b.Polarised light microscopy:
  • Needle-shaped crystals with strong negative birefringence.
  • Polarising light microscopy: Needle-shaped crystals kept parallel to light ray.

Adult Onset Still's Disease (AOSD)

Age:

  • 25-45 years

Presentation:

  • Systemic

Features:

  • Prodromal Phase: Sore throat
  • Clinical Triad:
    • Fever: Persistent high spiking that reaches subnormal levels during the day (quotidian)
    • Rash: Salmon-colored, transient, maculopapular
    • Arthritis: Symmetric, polyarticular (knee > wrist > ankle > elbow). Progress to severe/destructive arthritis in 25% of cases.

Additional Features:

  • Joint Erosions: Most common in children
  • Serositis: Inflammation of serous membranes
  • Lymphadenopathy: Enlarged lymph nodes
  • Hepatosplenomegaly: Enlargement of the liver and spleen
  • Blood Parameters:
    • Leukocytosis: Increased white blood cell count
    • ESR (Erythrocyte Sedimentation Rate) ↑ : Elevated
    • CRP (C-reactive protein) ↑: Elevated
  • Yamaguchi Criteria (>5):
    • Fever: Greater than 39°C for 7 days
    • Arthritis/Arthralgia: Joint pain/inflammation for 2 weeks

Prognosis:

  • Bad prognosis.

Other Findings

  • Ferritin: Increased
  • Serum Albumin: Decreased
  • Rash: Present
  • Leukocytosis

Rheumatology and Immunology

Gout

Treatment:
First Line:
  • NSAIDs: Indomethacin 50mg TDS.
  • Colchicine: Prevents neutrophil migration & chemotaxis. 1.2mg Stat → 0.6mg after 1 hr → 0.6mg BD × 1 week → 0.6mg OD × 3-6 months.
  • Intra-articular steroids
Second Line:
  • Anakinra: Anti IL-1 injection (Single dose).
  • IM ACTH: Injection (Single dose).
  • IV Pegloticase: (Recombinant uricase).
ACR Criteria of Gout:
  • Acute monoarticular arthritis
  • Hyperuricemia
  • Dramatic response to colchicine
Intercritical Period:
  • Symptom-free period
  • Classical feature of crystal arthropathy
Chronic Gout Investigation:
  • X-ray of lesion:
    • Asymmetric
    • Punched out lytic
    • Overhanging edges: Martel sign / G sign
    • Sclerotic margins: Rat bite erosions
    • Joint space maintained
Ultrasound of Knee:
  • Uric acid crystals:
  • Hypoechoic hyaline cartilage:
  • Femoral cortex:
Additional Information:
  • 60%: Second flare within a year.
  • Continued deposition: of tophaceous gout & erosion.

Tophi and Crystal Arthropathies

Basic Calcium Phosphate (BCP)
  • Deposition of calcium hydroxyapatite crystals.
  • Non-birefringent.
Presentation:
  • Chronic calcific periarthritis: most common in the supraspinatus tendon.
  • Milwaukee shoulder: Rotator cuff arthropathy + Glenohumeral destruction.
  • Calcinosis cutis: Subcutaneous calcification.
  • Seen in: 1.CREST syndrome. 2.Juvenile dermatomyositis (Lipodystrophy, myofascial involvement).
Calcium Oxalate
  • Strong positive birefringence.
  • Pyramidal, envelope shaped crystals.

Types:

  • Primary: Seen in hyperoxaluria.
  • Secondary: Seen in CKD.
  • Note: mixture of hyperoxaluria → Combined kidney & liver transplantation.

Rheumatology and Immunology

Differential Diagnosis (D/d)

  • Tendinitis, bursitis (to rule out)
  • Hemophilia (similar presentation)

Gonococcal Arthritis

  • Young, healthy individuals
  • Migratory polyarthralgia/arthritis

Septic Arthritis

  • Immunocompromised individuals
  • Monoarticular arthritis

Diagnosis

  • Synovial fluid analysis (gold standard)
    • WBC > 50000 /μL
  • Treatment (Rx):
    • Antibiotics: vancomycin + ceftriaxone
    • Drainage

Indications for drainage:

  • Thick pus
  • Shoulder, hip joint involvement

Chronic Monoarticular Arthritis

Causes:

  • TB
  • Fungal disease
  • Lymes
  • Sarcoidosis
  • Spondyloarthritis
  • Non-inflammatory

Approach to Arthritis

Monoarticular Arthritis:
  • Septic:
    • High WBC
  • Crystal:
    • Crystal analysis
  • Inflammatory:
    • Elevated inflammation markers
  • Infectious:
    • Infectious disease evaluation
  • Undifferentiated:
Polyarticular Arthritis
  • Acute:
  • Post viral:
  • Chronic Inflammatory (e.g., RA):
    • Rheumatoid arthritis (RA)
    • Systemic lupus erythematosus (SLE)
    • Psoriatic arthritis

Calcium Pyrophosphate Dihydrate (CPPD)

Age:

  • Elderly.

Symptoms:

  • Asymptomatic.

Inflammation:

  • Non-inflammatory.

Gene:

  • ANKH

Presentations:

  1. Chondrocalcinosis: Asymptomatic calcification of articular cartilage. Predominantly affects the knee.
  2. Acute Monoarticular Arthritis:
    • Bloody aspiration
    • No hematogenous source of foci
    • No response to antibiotics and colchicine
    • Rhomboid crystals with weak positive birefringence
    • Pseudo-gout
  3. Chronic Inflammatory Polyarthritis:
    • Waxing and waning progression
    • Non-erosive
    • Pseudo RA
  4. Involvement of MCP (2nd, 3rd), wrist:
    • Hook-like osteophytes (Seen in hemochromatosis)
    • Pseudo-OA
  5. Pseudo neuropathic disease: Presents like Charcot's disease.
  6. Spondyloarthritis: Crowned dens syndrome (Dens in atlas)
  7. Septic Arthritis (m/c knee): Fever

Associations:

  • Hyperparathyroidism
  • Hypophosphatasia (differential diagnosis of rickets with decreased alkaline phosphatase)
  • Hypomagnesemia (Seen in Gitelman syndrome)
  • Hemochromatosis

Crystal Arthropathies

Tophi

  • Irregular asymmetric moderately discrete tumescence of fingers d/t s/c deposition of MSU crystals.
  • Painless.
  • Acute on chronic: Acute inflammation surrounding tophi.

Sites:

  • Olecranon (most common)
  • Prepatellar bursa
  • Ulnar surface
  • Achilles surface
  • Myocardium
  • Heart valves

Treatment

Xanthine oxidase inhibitors: Uric acid lowering agents
  • Febuxostat (S/E: Cardiotoxicity)
  • Allopurinol 300mg daily.
    • S/E: Hypersensitivity in HLA B58-01 susceptibility.
    • Hairfall.
    • Liver & renal disease.
Uricosuric agents: Enhance uric acid excretion
  • Probenecid
  • Sulfinpyrazone
  • Benzbromarone
  • Losartan (ACE inhibitor)
  • Lesinurad
Recombinant uricase:
  • Rasburicase
  • Pegloticase Also used in acute gout & TLS.

Features:

  • 12 years between first attack & chronic gout.
  • Rate of tophus formation correlates with degree & duration of hyperuricemia.

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