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HMS IDD > Granulomatous Diseases > Flashcards

Granulomatous Diseases Flashcards

1
Q

Liver TRMs

A

Kupffer cells

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2
Q

Spleen and secondary lymphoid organ TRMs

A

Sinus histiocytes

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3
Q

CNS TRMs

A

Microglia

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4
Q

Lung TRMs

A

Alveolar macrophages

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5
Q

Five main functions of macrophages

A
  • Phagocytosis
  • Fibrosis
  • Antigen presentation
  • Tissue destruction/remodeling
  • Angiogenesis
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6
Q

Th1-derived ___ activates ___ to secrete ___ which is repsonsible for inducing and maintaining granuolmas.

A

Th1-derived IFN-g activates macrophages to secrete TNF-a which is repsonsible for inducing and maintaining granuolmas.

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7
Q

“Naked granuloma”

A

No or minimal lymphocytes surrounding granuloma

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8
Q

Granulomas

A
  • Assembly of macrophages in tissue, which may appear as “epithelioid histiocytes”
  • May or may not contain giant cells
  • May or may have lymphocytic involvement
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9
Q

Blood cell lineage diagram

A
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10
Q

Monocytes circulate for ___ before entering the tissue where they live for ___ as histiocytes

A

Monocytes circulate for only a few hours to days before entering the tissue where they live for months to years as histiocytes

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11
Q

Histiocyte

A

A term for monocytes that have entered a tissue

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12
Q

Bone TRMs

A

Osteoclasts

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13
Q

Kidney TRMs

A

Intraglomerular mesangial cells

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14
Q

While ___ have a phenotype similar to that of a dendritic cell, they are in fact derived from ___.

A

While Langerhans cells have a phenotype similar to that of a dendritic cell, they are in fact derived from blood monocytes.

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15
Q

Monocyte-derived lineages

A

Dendritic cells AND macrophages

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16
Q
A

Foreign body granuloma

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17
Q
A

Foreign body granuloma

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18
Q

Clinically, foreign body granulomas may appear as . . .

A

. . . erythematous papules, plaques or dermal or subcutaneous nodules. They can sometimes become scaly, weepy, or ulcerate.

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19
Q

Fish Tank Granuloma

A
  • Caused by Mycobacterium marinum
  • Usually seen in individuals who clean aquariums or sell fish and have a small cut or are exposed to contaminated water
  • Often the lesion heals fine, but then ~3 weeks later a verrucous or wart-like papule or nodule will emerge at the site of injury, then begins to spread
  • Lesions may be asymptomatic or may be painful if tissue containing nerves is involved
  • Progression typically very slow, over the course of years
  • Treatment involves months of antibiotics
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20
Q
A

Verrucous cutaneous tuberculosis

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21
Q

Tuberculosis marinum infection spreads in the body via the ___.

A

Tuberculosis marinum infection spreads in the body via the lymphatics.

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22
Q
A

Multiple erythematous nodules spreading linearly via the lymphatics. Initial spread of M. marinum

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23
Q

Chronic granulomatous disease

A
  • Characterized by recurrent, life-threatening bacterial and fungal infections and granuloma formation
  • Caused by genetic deficiency in Phox
24
Q
A

Massive lymphadenopathy in CGD

25
Massive lymphadenopathy of CGD
26
Symptoms and signs of pulmonary sarcoidosis
* Dyspnea * Dry cough * Normal physical exam, possibly crackles on lung auscultation * Chest X-ray reveals hilar and mediastinal lymphadenopathy
27
Symptoms and signs of cutaneous sarcoidosis
* Systemic involvement * Sometimes referred to the "great immitator" for its difficulty to pin down * Violaceous papules (eyelids & nostrils) * **When nostril rim involved, respiratory track involved** * Violaceous plaques & nodules (face, neck, & extremities)
28
Cutaneous sarcoidosis of the eye
29
What do the arrows show?
Beading in cutaneous sarcoidosis
30
Sarcoidosis in knee scar
31
Diascopy
* Press glass slide against a lesion * Reveals characteristic apple jelly color in granulomatous inflammation
32
Joint involvement in sarcoidosis
* May appear as acute polyarthritis involving **periarthritic** symptoms and with symmetric involvement, often of ankles * In chronic cases, periosteal bone resorption creates cysts which may be detected on X-ray * May cause substantial deviation at DIPs
33
Eye involvement in sarcoidosis
* 25% of patients * Presents as uveitis with substantial redness and vasodilation * Highly correlated with systemic involvement * Can involve many parts of the eye (orbit, lacrimal glands, muscle, conjunctiva, sclerae)
34
Differential for sarcoidosis
Major differentials are infection and cancer
35
Treatment of sarcoidosis
* Oral steroids * Other immunosuppressants
36
Clinical variants of Sarcoidosis
* **Löfgren’s Syndrome:** More mild form. Tetrad of hilar lymphadenopathy, acute periarthritis of ankles, fever and/or uveitis, erythema nodosum * **Lupus Pernio:** Severe form of sarcoidosis. Lymphadenopathy, organomegaly, uveitis, bone cysts. Very difficult to treat.
37
Erythema nodosum
* Hypersensitivity reaction which may result from drugs, infections, or induction of autoimmune disease * Development of multiple erythematous patches
38
Cutaneous sarcoidosis (lupus pernio)
39
Cutaneous sarcoidosis Lupus pernio variant
40
Cutaneous sarcoidosis, subcutaneous variant Lupus pernio
41
Crohn's disease
* IBD characterized by granulomatous inflammation along the entire GI tract * Can also manifest in the skin, joints, eyes and kidneys * Mucosal T cells secrete large amounts of TNF-a and IFN-g which produces ongoing acute and chronic inflammation of GI tract * abdominal pain, bloody diarrhea, peritoneal signs * Increases risk of gut colonization by microbes
42
Normal IG vs Crohn's GI vs UC GI
43
Extracutaneous manifestations of Crohn's
44
Eosinophilic Granulomatosis with Polyangiitis
* Granulomatous vasculitis of small- and medium-sized vessels of unclear etiology * chronic allergic rhinitis, sinusitis, asthma, peripheral eosinophilia, and lung and skin disease * vasculitic lung disease can cause lung nodules and alveolar hemorrhage * Cutaneous vasculitis can cause subcutaneous nodules or palpable purpura * Granulomatous nodules can be seen on the elbows and knees
45
Suppurative granuloma
* Granuloma with a central abscess * Defined by **epithelioid histiocytes and multinucleate giant cells** with a **central collection of neutrophils** * May occur with necrotizing or non-necrotizing granulomatous inflammation * Associated with coccidiomycosis and *Bartonella henselae* (cat scratch disease)
46
Naked granulomas are associated with. . .
. . . sarcoidosis
47
When you diagnose a patient with sarcoidosis, always immediately order \_\_\_.
When you diagnose a patient with sarcoidosis, always immediately order **an EKG**.
48
If a sarcoidosis patient is found to have heart involvement, what steps should be taken?
Implantation of a pacemaker and debibrillator.
49
In a non-naked granuloma, the T cells will be . . .
. . . **intermixed with the macrophages**, not just surrounding it. Even if there are a ton of T cells surrounding the granuloma, it is a naked granuloma.
50
The apple jelly sign for granulomas is. . .
. . . **very specific, but not very sensitive**
51
Findings: Reynaud's and telangiectasias of the finger nail beds and palm. What is your likely diagnosis?
Scleroderma
52
Findings: Raynaud's, malar rash, and alopecia What is the likely diagnosis?
Lupus
53
Findings: Raynaud's, palpable purpura, and fingertip necrosis What is your likely diagnosis?
Medium vessel vasculitis
54
Findings: Raynaud's, Gottron's papules, and GERD What is the likely diagnosis?
Dermatomyositis
55
Gastric antral vascular ectasia
aka GAVE aka watermelon stomach Associated with anti-RNA Pol III

HMS IDD (28 decks)

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