Autoimmune Flashcards
A 31 year old female presents with polyarthralgias and a malar rash. What type of hypersensitivity reaction is responsible for her condition?
Type III (immune complex-mediated) hypersensitivity due to Systemic Lupus Erythematosus
A 45 year old woman is referred to a rheumatologist with suspected scleroderma. What is a possible origin of foreign cells found in her vasculature on biopsy?
Fetal cells from one of her sons; this observation has drawn parallels to graft vs host disease (GVHD).
A 49 year old female patient presents at the emergency room with symptoms of PICA infarction. On exam, erythematous lesions of the face and arms are observed, and urinalysis shows hyaline casts and red cells. The PICA infarction is a potentially fatal complication of what disorder?
Systemic Lupus Eythematosus - arthralgias of multiple joints should have been observed in earlier stages
A 38 year old male presents with diffuse symptoms including loss of facial expression, progressive shortness of breath, malignant hypertension, Raynaud’s phenomenon,electrolyte imbalances, and cardiac arrhythmias. What is the most likely disorder? What is the most likely autoantibody found in serum?
Generalized (or Progressive or Diffuse) Scleroderma, remember that 20% of cases are males; SCL-70 antibodies are positive in 60% of cases
A 61 year old male in the hospital for treatment of a non-MDR case of tuberculosis was recovering well but had to be evaluated for sudden onset of cough, fever, dyspnea, and pain in multiple joints. Chest CT shows new onset pleuritis. What is the most likely cause of these symptoms? What is the treatment?
Drug induced lupus from isoniazid treatment of TB; Switch TB drugs to alleviate symptoms. The patient may have an HLA-DR4 phenotype and poor clearance (poor acetylation rate) of the isoniazid
A 44 year old female presents with calcinosis, Raynaud’s phenomenon, esophageal dismotility, sclerodactyly, and telangiectasia. What is the responsible syndrome in this case? What is the most likely autoantibody in serum?
CREST syndrome of limited scleroderma; anticentromere antibodies are seen in a majority of cases
A 29 year old female presents with mixed erythematous and telangiectatic plaques on the face and scalp. The doctor tests ANA, which is postive at 1:1280. What is observed on biopsy of the lesions? What is the diagnosis if uninvolved skin has no visible pathology?
Ig and complement deposition is observed at the dermis-epidermis interface; Chronic discoid lupus. SLE shows skin deposits in areas with or without an overlying rash or lesion.
A 37 year old female’s case history has been brought to Grand Rounds to discuss her complicated case. She has developed polyarthralgias with joint destruction, pulmonary interstitial disease, Raynaud’s phenomenon, malar rash, arthritis, and esophageal dismotility. Autoantibody testing reports that she is ANA+, dsDNA-, U1-RNP+. All other autoantibodies are negative. What is the official name for this disorder? What is the controversy in nomenclature and treatment?
Officially, she fits the criteria for Mixed Connective Tissue Disease (MTCD), a multi-etiology manifestation. There is argument over the possibility that this is merely eary, undifferentiated autoimmune disease that should be followed until it differentiates into a more recognizable case. Also corticosteroid treatment may or may not work depending on the study.
A 42 year old female presents with recurrent pneumonia. A physical exam reveals keratoconjunctivitis sicca (dry eyes) and xerostomia (dry mouth). What blood test and what biopsy would help diagnose the disorder causing the recurrent infections?
A test for Ro (SS-A) and La (SS-B) antibodies. A biopsy of the salivary or lacrimal glands will show acinar/ductal destruction with lymphocytic infiltrate. The glandular stroma is preserved, a key differantial to rule out lymphoma.
Antibodies against dsDNA and Sm antigen
IgG and complement deposition at dermal-epidermal junction
women, child-bearing age
SLE
Autoantibodies against DNA or nuclear nonhistone proteins, especially SS-A (Ro) and SS-B (La)
keratoconjunctivitis sicca; xerostomia
women, ages 30-65
intense lymphocytic infiltrate at salivary, lacrimal glands
Sjogren Syndrome
Scl-70 autoantibody; nucleolar autoantibodies against RNApol; anti-centromere antibodies
widespread collagen deposition, leading to fibrosis and vasculopathy
HLA-DQB1
Scleroderma
anti-U1-RNP antibody
Mixed Connective Tissue Disease
“stone facies”; ischemic ulceration of fingers
esophageal hypomotility & dysphagia
vascular narrowing & fibrosis of kidney
women, ages 25-50
What is CREST?
Scleroderma
C: Calcinosis R: Raynaud's E: Esophageal dysmotility S: Sclerodactyly T: Telangiectasia
medium- to smaller-sized muscular arteries
fibrinoid necrosis + eosinophil involvement
proteinuria, mucle pain, reddish-blue skin lesions
responsive to anti-inflammatories, immunosuppression
Polyarteritis Nodosa
leukocytoclastic vasculitis + palpable purpura on lower extremities + foreign object (drug, bacterial product)
P-ANCA(+)
Hypersensitivity Angitis aka Microscopic Polyarteritis
-may occur as a feature of SLE, RA, Sjogren, HSP
oral apthous ulcers
genital ulcers
ocular inflammation
nonspecific vasculitis of arterioles, capillaries, venules
Behcet Disease
“a systemic vasculitis involving mucous membranes of many organs”
headache; throbbing pain over temporal artery; visual symptoms
dramatic response to corticosteroids
patchy & granulomatous inflammatory process
elderly
Giant Cell Arteritis (Temporal arteritis)
pneumonitis, sinusitis
renal glomerular disease (hematuria, proteinuria)
rash, muscule pains, joint involvement, neuro symptoms
C-ANCA(+)
men, 5th to 6th decades
Wegener’s Granulomatosis (Granulomatosis with Polyangitis)
fever; skin rash; conjunctival, oral, & mucosal inflammation
infancy or early childhood
necrotizing vasculitis that can lead to coronary aneurysms
Kawasaki Disease (mucocutaneous lymph node syndrome)
smoker
intermittent claudication and pains after exercise, quickly relieved by rest
painful ulceration of tips of digits
acute inflammation of medium- to small-sized arteries
Buerger Disease (Thromboangitis obliterans)
young patient
ANCA(+)
late-onset asthma and respiratory distress
granulomatous lesions with intense eosinophilic infiltrates
Churg-Strauss (Allergic Granulomatosis)
“a systemic vasculitis of young patients with late-onset asthma”
intermittent claudication of arms or legs
asymmetric differences in blood pressure
CHF, visual loss
young women
inflammation of large arteries, usually aorta, which has focal, raised plaques
Takayasu Arteritis
aortic branches become occluded
slowly progressive destruction of articular cartilage
deep, achy joint pain following activity; relieved by rest
weight-bearing joints and fingers of older persons; or traumatized joints of younger persons
Heberden nodes
Osteoarthritis
long, needle-shaped crystals that are negatively birefringent under polarized light
punched-out, juxtaarticular lytic lesions
impaired renal excretion of UA; deposition of urate crystals in joints, kidneys
adult men
Gout
“rat-bite” lesions
tophus nodule
vague MSK discomfort that localizes to the involved joints; symmetric & bilateral
pain is most severe after periods of disuse
disease activity waxes & wanes
synovial cell hyperplasia results in pannus formation
women, 3rd-4th decades, HLA2 genes
Rheumatoid Arthritis
Rheumatoid nodules are found in extra-auricular locations and show central necrosis surrounded by palisaded macrophages
inflammatory arthropothy of the vertebral column and sacroiliac joints
young men, HLA-B27(+)
Ankylosing Spondylitis
1) seronegative polyarthritis
2) conjunctivits/uveitis
3) nonspecific urethritis
men, HLA-B27(+)
follows venereal exposure or an episode of bacillary dysentery
Reiter Syndrome
mucocutaneous lesions (keratoderma blennorrhagica) over palms, soles, and trunk
self-limited attacks of acute arthritis
stubby, short, rhomboid (coffin-shaped) crystals with weak positive birefringence under polarized light
old age, often >85
Pseudogout
Calcium Pyrophosphate Dihydrate Deposition
