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163 Cards in this Set

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Achondroplasia does not affect which type of bone formation?
Membranous ossification is not affected (skull, facial bones, and axial skeleton are normal). Head and trunk are normal in size, but limbs are much shorter than normal.
What's the classic presentation of osteoarthritis?
Pain in weight bearing joints after use (eg. at the end of the day), improving with rest. No systemic symptomes. Common in older patients.
name 7 mechanical features of osteoarthritis:
-wear and tear of joins leads to destruction of articular cartilage
-subchondral bone formation
-sclerosis
-ostephytes
-eburnation - bone looks shiny because it's lost cartilage
-Heberden's nodes (DIP)
-Bouchard's nodes (PIP)
What's the classic presentation of Rheumatoid Arthritis?
-morning stiffness improving with use
-symmetric joint involvement
-systemic symptomes (fever, fatigue, pleuritis, pericarditis)
Lab test for Rheumatoid Arthritis:
80% of RA patients have positive Rheumatoid Factor (anti-IgG antibody)
Etiology and Pathologies of Rheumatoid Arthritis
-Autoimmune-inflammatory disorder affecting synovial joints with pannus formation in joints (MCP, PIP)
-subcutaneous rheumatoid nodules
-ulnar deviation
-subluxation.
Which arthritis is worse in the morning? which at night?
Rheumatoid arthritis is worse in the morning, osteoarthritis is worse after a long days use.
Which arthritis gets better with use?
Rheumatoid arthritis- morning stiffness improves with use.
what are the 4 systemic symptoms of rheumatoid arthritis?
fever
fatigue
pleuritis
pericarditis
what are the systemic symptoms of osteoarthritis?
there are no systemic symptomes of osteoarthritis
Definition of osteoporosis
reduction of bone mass in spite of normal bone mineralization
Type 1 vs Type 2 osteoporosis
Type 1:
-Postmenopausal
-increased bone resorption due to decreased estrogen levels
-estrogen replacement is controversial as prophylaxis due to side-effects

Type 2: (Senile Osteoperosis)
-affects men and women older than 70
What racial groups does osteoperosis affect?
whites > blacks > asians
3 Common fractures in osteoporosis
-Vertebral crush fractures--acute back pain, loss of height, kyphosis
-Distal radius (Colles') factures
-vertebral wedge fractures
Treatement for osteoperosis
Bisphosphonates or pulsatile PTH for severe cases
Osteopetrosis is also known as
marble bone disease

(is also known as)
Osteopetrosis is caused by
Abnormal function of osteoclasts → Failure of normal bone resorption → thickened, dense bones
blood values in osteopetrosis
Serum Calcium, Phosphate and Alkaline Phosphatase are ALL NORMAL.
In osteomalacia/rickets, vitamin D deficiency leads to...
Vitamin D deficiency → ↓Calcium → ↑PTH secretion, ?serum phosphate

Defective mineralization of osteoid → soft bones
Osteitis Fibrosa Cystica is caused by
Hyperparathyroidism
Osteitis Fibrosa Cystica is characterized by
"brown tumors" (cystic spaces lined by osteoclasts, filled with fibrous stroma and sometimes blood)
what are brown tumors?
cystic spaces lined by osteoclasts, filled with fibrous stroma and sometimes blood - found in Osteitis Fibrosa Cystica
blood values in Osteitis Fibrosa Cystica
High serum calcium
Low serum phosphorus
High alkaline phosphatase
Paget's disease is also known as
osteitis deformans
Paget's disease (osteitis deformans) is caused by
abnormal bone architecture caused by an ↑ in both osteoblastic and osteoclastic activity
blood values in paget's disease (osteitis deformans)
serum calcium, phosphorus and PTH levels are normal

Serum Alkaline Phosphatase is Elevated!
what is Polyostotic Fibrous Dysplasia?
Bone is replaced by fibroblasts, collagen, and irregular bony trabeculae. Affects many bones.
What is Albright's Syndrome?
a form of polyostotic fibrous dyplasia in which there are multiple unilateral bone lesions associated with endocrine abnormalities (precocious puberty) and unilateral pigmented skin lesions
How does Polymyalgia Rheumatic present?
Pain and siffness in shoulders and hips, often with fever, malaise, and weight loss.

Does not cause muscle weakness

Occurs in patients >50 y.o.

*Associated with temporal (giant cell) arteritis
what blood values would you see in Polymyalgia Rheumatica?
↑ ESR
How do you treat Polymyalgia Rheumatica?
with Predisone
Polymyositis - what is it and what's it caused by?
Progressive proximal muscle weakness caused by CD8+ T-cell-induced injury to myofibers.
How do you diagnose Polymyositis?
muscle biopsy with evidence of inflammation is diagnostic

Labs show:
↑CK
↑aldolase
positive ANA
anti-Jo-1
what is dermatomyositis?
similar to polymyositis but also involves skin rash and ↑ risk for malignancy
How do you diagnose dermatomyositis?
Labs show:
↑CK
↑aldolase
positive ANA
anti-Jo-1
6 characteristics of Mixed Connective Tissue Disease
Raynaud's phenomenon
Arthralgias
Myalgias
Fatigue
Esophageal Hypomotility

Antibodies to U1RNP
Classic triad of Sjogren's syndrome
Xerophthalmia - dry eyes, conjunctivitis

Xerostomia - dry mouth, dysphagia

Arthritis

(also see parotid enlargment)
Autoantibody production in Sjogren's syndrome
Autoantibodies to:
ribonucleoprotein antigens
SS-A (Ro)
SS-B (La)
Associated diseases with Sjogren's syndrome
Sjogrens patients have ↑ risk of B-cell lymphoma.

Sjogren's is also associated with rheumatoid arthritis

and Sicca syndrome (a type of sjogren's?)
what is Sicca syndrome? (5 symptomes)
another name for Sjogren's syndrome?

-Dry eyes,
-dry mouth
-nasal and vaginal dryness
-chronic bronchitis
-reflux esophagitis
Sjogren's affects which demographic predominantly?
Females, 40-60 y.o.
Systemic Lupus Erythematosus affects which demographic predominantly?
90% are female between 14 - 45 y.o. Mostly black females.
Mnemonic for Lupus and associated symptoms
I'M DAMN SHARP

Immunoglobulins (anti-dsDNA, anti-Sm, antiphospholipid)
Malar rash

Discoid rash
Antinuclear antibody
Mucositis (oropharyngeal ulcers)
Neurologic disorders

Serositis (pleuritis, pericarditis)
Hematologic disorders
Arthritis
Renal disorders
Photosensativity
7 symptoms for Lupus (not found in IM DAMN SHARP)
fever
fatigue
weight loss
nobacterial verrucous endocarditis
hilar adenopathy
Raynaud's phenomenon
Renal characteristics of Lupus
WIRE LOOP lesions in kidney with immune complex deposition (with nephrotic syndrome)
Death from renal failure and infections
_______ are found in drug-induced lupus
Antihistone antibodies
Lupus can cause false positives on which test? why?
syphilis tests (RPR/VDRL) due to antiphospholipid antibodies
Lab tests for lupus detect which antibodies?
Antinuclear antibodies (ANA) - sensitive, but not specific for SLE
Antbodies to double-stranded DNA (anti-dsDNA) - very specifice, poor prognosis
Anti-Smith antibodies (anti-Sm) - very specific, but not prognostic
Antihistone antibodies - drug-induced lupus
Which antibodies indicate a poor prognosis in lupus?
Anti-dsDNA
what is Celiac sprue caused by?
Autoimmune-mediated intolerance of gliadin (wheat) leading to steatorrhea
what type of diarrhea is found in Celiac sprue
steatorrhea
Celia sprue is associated with which race?
people of northern european descent
3 Findings in Celia sprue
blunting of villi
lymphocytes in the lamina propria
abnormal D-xylose test
what part of the GI tract does celia sprue affect?
tends to affect the Jejunum
what other 2 diseases is celiac sprue associated with?
dermatitis herpetiformis

and 10-15% lead to malignancy (most often T-cell lymphoma)
what crystals precipitate in the joints in gout?
monosodium urate crystals
what is gout?
precipitation of monosodium urate crystals into the joints due to hyperuricemia
5 etiologies of gout
Lesch-Nyhan syndrome
PRPP excess
Decreased excretion of uric acid
glucose-6-phosphatase deficiency
Thiazide Diuretics
What class of drugs can lead to gout? why?
Thiazide diuretics, which competitively inhibit the secretion of uric acid
is the joint distribution in gout symmetric or asymmetric?
asymmetric
2 classic manifestations of gout
Painful MTP joint in the big toe (Podagra)

Tophus formation (often on external ear or Achilles tendon)
Describe gout crystals
needle shaped and negatively birefringent
demographic of gout
more common in men
when do acute attacks of gout usually occur?
after alcohol consumption or a large meal
4 treatments of gout
Allopurinol
Probenecid
colchicine
NSAIDs
describe the crystals found in pseudogout
calcium pyrophosphate crystals found in pseudogout are:
basophilic
rhomboid
weakly positively birefringent
what crystals are found in pseudogout
calcium pyrophosphate crystals
what joints are affected in pseudogout
large joints (especially the knee)
demographics of pseudogout
>50 y.o.
both sexes affected equally
treatment for pseudogout
no treatment
2 major Findings in Goodpasture's Syndrome
pulmonary hemorrhages -> hemoptysis

renal lesions -> hematuria, anemia, crescentic glomerulonephritis
demographics of Goodpasture's syndrome
most common in men 20-40
immonofluorescence pattern seen in Goodpasture's
anti-glomerular basement membrane antibodies produce linear staining on immunofluorescence
Goodpastures is a Type # hypersensitivity?
Type 2
Sarcoidosis is characterized by
immune mediated, widespread noncaseating granulomas and elevated serum ACE levels.
Mnemonic for Sarcoidosis
GRAIN

Gammaglobulinemia
Rheumatoid arthritis
ACE increase
Interstitial fibrosis
Noncaseating granulomas
GRAIN
Associated with:

restrictive lung disease
bilateral hilar lymphadenopathy
erythema nodosum
Bell's palsy
Epithelial granulomas containing microscopic Schaumann and asteroid bodies
uveoparotitis
hypercalcemia (due to elevated conversion of vitamin D to its active form in epitheliod macrophages)
Sarcoidosis
why do you see hypercalcemia in Sarcoidosis
b/c of elevated conversion of vitamin D to its active form in epitheliod macrophages
Epithelial granulomas containing ____________ are found in Sarcoidosis
epithelial granulomas containing microscopic Schaumann and asteroid bodies
name 2 seronegative spondyloarthopathies
Ankylosing spondylitis

Reiter's syndrome
Arthritis without rheumatoid factor (no anti-IgG antobody)
Strong association with HLA-B27 (gene that codes for HLA MHC I)

-describes what group of diseases
seronegative spondyloarthopathies
what does HLA-B27 code for?
gene that codes for HLA MHC I
HLA-B27 is associated with which 4 diseases?
Psoriasis
Ankylosing Sponylitis
Inflammatory bowel disease
Reiter's syndrome

mnemonic: PAIR
PAIR
demographics of seronegative spondyloarthopathies
most often in males
Describe Ankylosing spondylitis
Chronic inflammatory disease of spine and sacroiliac joints → ankylosis (stiff spine), uveitis, and aoritic regurgitation
Classic triad of Reiter's syndrome
Urethritis
Conjunctivitis and anterior uveitis
Arthritis


Can't see, can't pee, can't climb a tree
Can't see, can't pee, can't climb a tree
Reiter's syndrome often occurs post-____ infections?
post-GI or chlamydia infections
another name for Scleroderma?
Progressive systemic sclerosis - PSS
2 major categories of Scleroderma
Diffuse scleroderma
CREST syndrome
Scleroderma is characterized by?

which organs are affected?
Excessive fibrosis and collagen deposition throughout the body.

Commonly sclerosis of skin but also of cardiovascular and GI systems and kidney
demographics of scleroderma
75% female
which antibodies are Diffuse scleroderma and CREST syndrome associated with?
Diffuse scleroderma - anti-Scl-70 antibody

CREST syndrome - anticentromere antibody
describe Diffuse scleroderma
widespread skin involvement,
rapid progression,
early visceral involvement.

Associated with anti-Scl-70 antibody
describe CREST syndrome
Calcinosis
Raynaud's phenomenon
Esophageal dysmotility
Sclerodactyly
Telangiectasia

Limited skin involvement, often confined to fingers and face
More benign clinical course
associated with anti-centromere antibody
mutation in Achondroplasia
FGF receptor 3
pannus formation
Rheumatoid Arthritis
ulnar deviation,
Rheumatoid Arthritis
fever, fatigue, pleuritis, pericarditis
systemic symptomes of Rheumatoid Arthritis
Boutonnière deformity, Swan-neck deformity, Z-thumb deformity
Rheumatoid Arthritis
↑ bone resorption due to ↓estrogen levels.
Type 1 (postmenopausal) osteoporosis
Senile osteoporosis––
Type 2 osteoporosis. >70 y.o.
tx for osteoporosis
Bisphosphonates or pulsatile PTH for severe cases.
Failure of normal bone resorption → thickened, dense bones.
Osteopetrosis (marble bone disease)
Failure of normal bone resorption → thickened, dense bones. Bone defect is due to abnormal function of osteoclasts. Serum calcium, phosphate, and alkaline phosphatase are normal.
Bone defect is due to abnormal function of osteoclasts.
Osteopetrosis (marble bone disease)
Failure of normal bone resorption → thickened, dense bones. Bone defect is due to abnormal function of osteoclasts. Serum calcium, phosphate, and alkaline phosphatase are normal.
Serum calcium, phosphate, and alkaline phosphatase are normal.
Osteopetrosis (marble bone disease)
Failure of normal bone resorption → thickened, dense bones. Bone defect is due to abnormal function of osteoclasts. Serum calcium, phosphate, and alkaline phosphatase are normal.
Defective mineralization of osteoid → soft bones.
Osteomalacia/rickets
Defective mineralization of osteoid → soft bones. Vitamin D deficiency in adults →
↓ calcium levels →↑ secretion of PTH, ↑ in serum phosphate. Reversible when
vitamin D is replaced. Vitamin D deficiency in childhood causes rickets.
Vitamin D deficiency in adults →
↓ calcium levels →↑ secretion of PTH

Osteomalacia/rickets
Defective mineralization of osteoid → soft bones. Vitamin D deficiency in adults →
↓ calcium levels →↑ secretion of PTH, ↑ in serum phosphate. Reversible when
vitamin D is replaced. Vitamin D deficiency in childhood causes rickets.
Caused by hyperparathyroidism.
Osteitis Fibrosa Cystica
Caused by hyperparathyroidism. Characterized by “brown tumors” (cystic spaces lined by osteoclasts, filled with fibrous stroma and sometimes blood). High serum calcium, low serum phosphorus, and high alkaline phosphatase.
Characterized by “brown tumors” (cystic spaces lined by osteoclasts, filled with fibrous stroma and sometimes blood).
Osteitis Fibrosa Cystica
Caused by hyperparathyroidism. Characterized by “brown tumors” (cystic spaces lined by osteoclasts, filled with fibrous stroma and sometimes blood). High serum calcium, low serum phosphorus, and high alkaline phosphatase
High serum calcium, low serum phosphorus, and high alkaline phosphatase
Osteitis Fibrosa Cystica
Caused by hyperparathyroidism. Characterized by “brown tumors” (cystic spaces lined by osteoclasts, filled with fibrous stroma and sometimes blood). High serum calcium, low serum phosphorus, and high alkaline phosphatase
Abnormal bone architecture caused by ↑ in both osteoblastic and osteoclastic activity.
Paget’s disease (osteitis deformans)
Abnormal bone architecture caused by ↑ in both osteoblastic and osteoclastic activity. Serum calcium, phosphorus, and PTH levels are normal. Serum alkaline phosphatase is elevated.
osteitis deformans -- aka?
Paget’s disease
Abnormal bone architecture caused by ↑ in both osteoblastic and osteoclastic activity. Serum calcium, phosphorus, and PTH levels are normal. Serum alkaline phosphatase is elevated.
Serum alkaline phosphatase is elevated.
Paget’s disease (osteitis deformans)

Osteitis Fibrosa Cystica
Abnormal bone architecture caused by ↑ in both osteoblastic and osteoclastic activity. Serum calcium, phosphorus, and PTH levels are normal. Serum alkaline phosphatase is elevated.
Bone is replaced by fibroblasts, collagen, and irregular bony trabeculae.
Polyostotic fibrous Dysplasia
Bone is replaced by fibroblasts, collagen, and irregular bony trabeculae. Affects many bones. Albright’s syndrome is a form of polyostotic fibrous dysplasia in which there
are multiple unilateral bone lesions associated with endocrine abnormalities
(precocious puberty) and unilateral pigmented skin lesions.
unilateral pigmented skin lesions.
Albright’s syndrome

Albright’s syndrome is a form of polyostotic fibrous dysplasia in which there
are multiple unilateral bone lesions associated with endocrine abnormalities
(precocious puberty) and unilateral pigmented skin lesions.
Pain and stiffness in shoulders and hips, often with fever, malaise, and weight loss.
Polymyalgia Rheumatica
Pain and stiffness in shoulders and hips, often with fever, malaise, and weight loss. Does not cause muscular weakness. ↑ ESR. Occurs in patients > 50 years of age; associated with temporal (giant cell) arteritis. Treated with prednisone.
Does Polymyalgia Rheumatica cause muscle weakness?
NO
Pain and stiffness in shoulders and hips, often with fever, malaise, and weight loss. Does not cause muscular weakness. ↑ ESR. Occurs in patients > 50 years of age; associated with temporal (giant cell) arteritis. Treated with prednisone.
↑ ESR. Occurs in patients > 50 years of age
Polymyalgia Rheumatica
Pain and stiffness in shoulders and hips, often with fever, malaise, and weight loss. Does not cause muscular weakness. ↑ ESR. Occurs in patients > 50 years of age; associated with temporal (giant cell) arteritis. Treated with prednisone.
associated with temporal (giant cell) arteritis
Polymyalgia Rheumatica
Pain and stiffness in shoulders and hips, often with fever, malaise, and weight loss. Does not cause muscular weakness. ↑ ESR. Occurs in patients > 50 years of age; associated with temporal (giant cell) arteritis. Treated with prednisone.
How do you treat Polymyalgia Rheumatica?
prednisone
Pain and stiffness in shoulders and hips, often with fever, malaise, and weight loss. Does not cause muscular weakness. ↑ ESR. Occurs in patients > 50 years of age; associated with temporal (giant cell) arteritis. Treated with prednisone.
Does Polymyositis cause muscle weakness?
Yes - progressive proximal muscle weakness caused by CD8+ T-cell-induced injury to myofibers. Muscle biopsy with evidence of inflammation is diagnostic.
Polymyositis— progressive proximal muscle weakness caused by CD8+ T-cell-induced injury to myofibers. Muscle biopsy with evidence of inflammation is diagnostic.
Dermatomyositis—similar to polymyositis but also involves skin rash and ↑ risk of malignancy.
Labs for polymyositis/dermatomyositis show ↑ CK, ↑ aldolase, and positive ANA, anti-Jo-1.
Muscle biopsy with evidence of inflammation is diagnostic.
Polymyositis/dermatomyositis
Polymyositis— progressive proximal muscle weakness caused by CD8+ T-cell-induced injury to myofibers. Muscle biopsy with evidence of inflammation is diagnostic.
Dermatomyositis—similar to polymyositis but also involves skin rash and ↑ risk of malignancy.
Labs for polymyositis/dermatomyositis show ↑ CK, ↑ aldolase, and positive ANA, anti-Jo-1.
involves skin rash and ↑ risk of malignancy.
dermatomyositis
Polymyositis— progressive proximal muscle weakness caused by CD8+ T-cell-induced injury to myofibers. Muscle biopsy with evidence of inflammation is diagnostic.
Dermatomyositis—similar to polymyositis but also involves skin rash and ↑ risk of malignancy.
Labs for polymyositis/dermatomyositis show ↑ CK, ↑ aldolase, and positive ANA, anti-Jo-1.
↑ CK, ↑ aldolase, and positive ANA, anti-Jo-1.
Labs for polymyositis/dermatomyositis
Polymyositis— progressive proximal muscle weakness caused by CD8+ T-cell-induced injury to myofibers. Muscle biopsy with evidence of inflammation is diagnostic.
Dermatomyositis—similar to polymyositis but also involves skin rash and ↑ risk of malignancy.
Labs for polymyositis/dermatomyositis show ↑ CK, ↑ aldolase, and positive ANA, anti-Jo-1.
Raynaud’s phenomenon, arthralgias, myalgias, fatigue, and esophageal hypomotility.
Mixed connective Tissue Disease
Raynaud’s phenomenon, arthralgias, myalgias, fatigue, and esophageal hypomotility. Antibodies to U1RNP.
Antibodies to U1RNP.
Mixed connective Tissue Disease
Raynaud’s phenomenon, arthralgias, myalgias, fatigue, and esophageal hypomotility. Antibodies to U1RNP.
Raynaud's phenomenon AND Esophageal dysmotility/hypomotility are seen in which 2 diseases?
Mixed connective Tissue Disease

CREST Scleroderma
Raynaud's phenomenon is seen in whic 4 diseases?
Mixed connective Tissue Disease

CREST Scleroderma

Systemic Lupus Erythematosus

Buerger's disease (vasculitis)
Parotid enlargement,
Sjögren’s syndrome
Classic triad––xerophthalmia (dry eyes, conjunctivitis), xerostomia (dry mouth, dysphagia), arthritis. Parotid enlargement, ↑ risk of B-cell lymphoma. Autoantibodies to ribonucleoprotein antigens, SS-A (Ro), SS-B (La). Predominantly affects females between 40 and 60 years of age.

Associated with rheumatoid arthritis
↑ risk of B-cell lymphoma
Sjögren’s syndrome
Classic triad––xerophthalmia (dry eyes, conjunctivitis), xerostomia (dry mouth, dysphagia), arthritis. Parotid enlargement, ↑ risk of B-cell lymphoma. Autoantibodies to ribonucleoprotein antigens, SS-A (Ro), SS-B (La). Predominantly affects females between 40 and 60 years of age.

Associated with rheumatoid arthritis
2 diseases associated with rheumatoid arthritis
Sjögren’s syndrome,

Sarcoidosis
Classic triad––xerophthalmia (dry eyes, conjunctivitis), xerostomia (dry mouth, dysphagia), arthritis. Parotid enlargement, ↑ risk of B-cell lymphoma. Autoantibodies to ribonucleoprotein antigens, SS-A (Ro), SS-B (La). Predominantly affects females between 40 and 60 years of age.

Associated with rheumatoid arthritis
-xerophthalmia (dry eyes, conjunctivitis)
-xerostomia (dry mouth, dysphagia)
-arthritis

Parotid enlargement,
Sjögren’s syndrome
Classic triad––xerophthalmia (dry eyes, conjunctivitis), xerostomia (dry mouth, dysphagia), arthritis. Parotid enlargement, ↑ risk of B-cell lymphoma. Autoantibodies to ribonucleoprotein antigens, SS-A (Ro), SS-B (La). Predominantly affects females between 40 and 60 years of age.

Associated with rheumatoid arthritis
dry eyes, dry mouth, nasal and vaginal dryness, chronic bronchitis, reflux esophagitis.
Sicca syndrome
Symptoms include fever, fatigue, weight loss, nonbacterial verrucous endocarditis, hilar adenopathy, and Raynaud’s phenomenon.
Lupus
90% are female and between ages 14 and 45. (Especially black females)
Lupus
lymphocytes in the lamina propria, abnormal D-xylose test, blunting of villi,
Celiac sprue
Autoimmune-mediated intolerance of gliadin (wheat) leading to steatorrhea. Associated
with people of northern European descent. Findings include blunting of villi,
lymphocytes in the lamina propria, and abnormal D-xylose test. Tends to affect
jejunum. Associated with dermatitis herpetiformis. 10–15% lead to malignancy (most
often T-cell lymphoma).
10–15% lead to malignancy (most often T-cell lymphoma)
Celiac sprue
Autoimmune-mediated intolerance of gliadin (wheat) leading to steatorrhea. Associated
with people of northern European descent. Findings include blunting of villi,
lymphocytes in the lamina propria, and abnormal D-xylose test. Tends to affect
jejunum. Associated with dermatitis herpetiformis. 10–15% lead to malignancy (most
often T-cell lymphoma).
Associated with dermatitis herpetiformis
Celiac sprue
Autoimmune-mediated intolerance of gliadin (wheat) leading to steatorrhea. Associated
with people of northern European descent. Findings include blunting of villi,
lymphocytes in the lamina propria, and abnormal D-xylose test. Tends to affect
jejunum. Associated with dermatitis herpetiformis. 10–15% lead to malignancy (most
often T-cell lymphoma).
Autoimmune-mediated intolerance of gliadin (wheat) leading to steatorrhea.
Celiac sprue
Autoimmune-mediated intolerance of gliadin (wheat) leading to steatorrhea. Associated
with people of northern European descent. Findings include blunting of villi,
lymphocytes in the lamina propria, and abnormal D-xylose test. Tends to affect
jejunum. Associated with dermatitis herpetiformis. 10–15% lead to malignancy (most
often T-cell lymphoma).
monosodium urate crystals
Gout
Precipitation of monosodium urate crystals into joints due to hyperuricemia, which can be
caused by Lesch-Nyhan syndrome, PRPP excess, ↓ excretion of uric acid, or glucose-6-
phosphatase deficiency. Also associated with the use of thiazide diuretics, which
competitively inhibit the secretion of uric acid. Asymmetric joint distribution. Classic
manifestation is painful MTP joint in the big toe (podagra). Tophus formation (often
on external ear or Achilles tendon). Crystals are needle shaped and negatively
birefringent. More common in men. Acute attack tends to occur after alcohol
consumption or a large meal (see Color Image 54). Treatment includes allopurinol,
probenecid, colchicine, and NSAIDs.
Gout can be caused by
hyperuricemia <-- Lesch-Nyhan syndrome, PRPP excess, ↓ excretion of uric acid, or glucose-6-phosphatase deficiency.

Also associated with the use of thiazide diuretics, which
competitively inhibit the secretion of uric acid.
Precipitation of monosodium urate crystals into joints due to hyperuricemia, which can be
caused by Lesch-Nyhan syndrome, PRPP excess, ↓ excretion of uric acid, or glucose-6-
phosphatase deficiency. Also associated with the use of thiazide diuretics, which
competitively inhibit the secretion of uric acid. Asymmetric joint distribution. Classic
manifestation is painful MTP joint in the big toe (podagra). Tophus formation (often
on external ear or Achilles tendon). Crystals are needle shaped and negatively
birefringent. More common in men. Acute attack tends to occur after alcohol
consumption or a large meal (see Color Image 54). Treatment includes allopurinol,
probenecid, colchicine, and NSAIDs.
Crystals are needle shaped and negatively
birefringent.
Gout
Precipitation of monosodium urate crystals into joints due to hyperuricemia, which can be
caused by Lesch-Nyhan syndrome, PRPP excess, ↓ excretion of uric acid, or glucose-6-
phosphatase deficiency. Also associated with the use of thiazide diuretics, which
competitively inhibit the secretion of uric acid. Asymmetric joint distribution. Classic
manifestation is painful MTP joint in the big toe (podagra). Tophus formation (often
on external ear or Achilles tendon). Crystals are needle shaped and negatively
birefringent. More common in men. Acute attack tends to occur after alcohol
consumption or a large meal (see Color Image 54). Treatment includes allopurinol,
probenecid, colchicine, and NSAIDs.
Tophus formation often occurs in
external ear or achillis tendon
Precipitation of monosodium urate crystals into joints due to hyperuricemia, which can be
caused by Lesch-Nyhan syndrome, PRPP excess, ↓ excretion of uric acid, or glucose-6-
phosphatase deficiency. Also associated with the use of thiazide diuretics, which
competitively inhibit the secretion of uric acid. Asymmetric joint distribution. Classic
manifestation is painful MTP joint in the big toe (podagra). Tophus formation (often
on external ear or Achilles tendon). Crystals are needle shaped and negatively
birefringent. More common in men. Acute attack tends to occur after alcohol
consumption or a large meal (see Color Image 54). Treatment includes allopurinol,
probenecid, colchicine, and NSAIDs.
Acute Gout attack tends to occur after
alcohol or large meal
Precipitation of monosodium urate crystals into joints due to hyperuricemia, which can be
caused by Lesch-Nyhan syndrome, PRPP excess, ↓ excretion of uric acid, or glucose-6-
phosphatase deficiency. Also associated with the use of thiazide diuretics, which
competitively inhibit the secretion of uric acid. Asymmetric joint distribution. Classic
manifestation is painful MTP joint in the big toe (podagra). Tophus formation (often
on external ear or Achilles tendon). Crystals are needle shaped and negatively
birefringent. More common in men. Acute attack tends to occur after alcohol
consumption or a large meal (see Color Image 54). Treatment includes allopurinol,
probenecid, colchicine, and NSAIDs.
calcium pyrophosphate crystals
Pseudogout
Caused by deposition of calcium pyrophosphate crystals within the joint space. Forms
basophilic, rhomboid crystals that are weakly positively birefringent (as opposed to the
negatively birefringent, needle-shaped crystals in gout). Usually affects large joints
(classically the knee). > 50 years old; both sexes affected equally. No treatment.
basophilic, rhomboid crystals that are weakly positively birefringent
Pseudogout

calcium pyrophosphate crystals
Caused by deposition of calcium pyrophosphate crystals within the joint space. Forms
basophilic, rhomboid crystals that are weakly positively birefringent (as opposed to the
negatively birefringent, needle-shaped crystals in gout). Usually affects large joints
(classically the knee). > 50 years old; both sexes affected equally. No treatment.
Usually affects large joints (classically the knee)
Pseudogout
Caused by deposition of calcium pyrophosphate crystals within the joint space. Forms
basophilic, rhomboid crystals that are weakly positively birefringent (as opposed to the
negatively birefringent, needle-shaped crystals in gout). Usually affects large joints
(classically the knee). > 50 years old; both sexes affected equally. No treatment.
pulmonary hemorrhages → hemoptysis;
renal lesions → hematuria, anemia, crescentic glomerularnephritis
Goodpasture’s
Goodpasture’s is most common in
men 20-40 y.o.
Anti–glomerular basement membrane antibodies produce linear staining on immunofluorescence.
Goodpasture’s
Characterized by immune-mediated, widespread noncaseating granulomas and elevated serum ACE levels. Common in black females.
Sarcoidosis
GRAIN in Sarcoidosis stands for?

BHUSHAN & LE is on hint
Gammaglobulinemia
Rheumatoid arthritis
ACE increase
Interstitial fibrosis
Noncaseating granulomas
-Bell’s palsy
-hilar lymphadenopathy
-uveoparotitis,
-Schaumann bodies (in epithelial granulomas)
-hypercalcemia (due to
elevated conversion of vitamin D to its active
form in epithelioid macrophages)
-asteroid bodies (in epithelial granulomas)
-noncaseating granulomas

-restrictive Lung disease,
-erythema nodosum (inflammation of fat under skin. Tender, red nodules usually on shins)
BHUSHAN & LE in Sarcoidosis stand for?

GRAIN is on hint
-Bell’s palsy
-hilar lymphadenopathy
-uveoparotitis,
-Schaumann bodies (in epithelial granulomas)
-hypercalcemia (due to
elevated conversion of vitamin D to its active
form in epithelioid macrophages)
-asteroid bodies (in epithelial granulomas)
-noncaseating granulomas

-restrictive Lung disease,
-erythema nodosum (inflammation of fat under skin. Tender, red nodules usually on shins)
Gammaglobulinemia
Rheumatoid arthritis
ACE increase
Interstitial fibrosis
Noncaseating granulomas
Arthritis without rheumatoid factor (no anti-IgG antibody).
Seronegative Spondyloarthropathies
-Ankylosing Spondylitis
-Reiters
ankylosis (stiff spine) uveitis
aortic regurgitation
Ankylosing spondylitis

Chronic inflammatory disease of spine and sacroiliac joints → ankylosis (stiff spine), uveitis, and aortic
regurgitation.
Chronic inflammatory disease of spine and sacroiliac joints → ankylosis (stiff spine), uveitis, and aortic
regurgitation.
Urethritis
Conjunctivitis and anterior uveitis
Arthritis
Reiter’s syndrome

Post-GI or chlamydia
infections.
Excessive fibrosis and collagen deposition throughout the body. 75% female.
Scleroderma (Progressive Systemic Sclerosis - PSS)
CREST syndrome
Calcinosis, Raynaud’s phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasia. Limited skin involvement, often confined to fingers and face. More benign clinical course. Associated with anticentromere antibody
Limited skin involvement, often confined to fingers and face
CREST syndrome
Calcinosis, Raynaud’s phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasia. Limited skin involvement, often confined to fingers and face. More benign clinical course. Associated with anticentromere antibody
anticentromere antibody
CREST syndrome
Calcinosis, Raynaud’s phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasia. Limited skin involvement, often confined to fingers and face. More benign clinical course. Associated with anticentromere antibody
anti-Scl-70 antibody
Diffuse scleroderma
Diffuse scleroderma––widespread skin involvement, rapid progression, early visceral involvement. Associated with anti-Scl-70 antibody.
widespread skin involvement, rapid progression, early visceral involvement.
Diffuse scleroderma
Diffuse scleroderma––widespread skin involvement, rapid progression, early visceral involvement. Associated with anti-Scl-70 antibody.
Scleroderma is commonly sclerosis of the skin but also of the
cardiovascular and GI systems and kidney.