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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
Q
A

Massive lymphadenopathy of CGD

26
Q

Symptoms and signs of pulmonary sarcoidosis

A
  • Dyspnea
  • Dry cough
  • Normal physical exam, possibly crackles on lung auscultation
  • Chest X-ray reveals hilar and mediastinal lymphadenopathy
27
Q

Symptoms and signs of cutaneous sarcoidosis

A
  • 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
Q
A

Cutaneous sarcoidosis of the eye

29
Q

What do the arrows show?

A

Beading in cutaneous sarcoidosis

30
Q
A

Sarcoidosis in knee scar

31
Q

Diascopy

A
  • Press glass slide against a lesion
  • Reveals characteristic apple jelly color in granulomatous inflammation
32
Q

Joint involvement in sarcoidosis

A
  • 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
Q

Eye involvement in sarcoidosis

A
  • 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
Q

Differential for sarcoidosis

A

Major differentials are infection and cancer

35
Q

Treatment of sarcoidosis

A
  • Oral steroids
  • Other immunosuppressants
36
Q

Clinical variants of Sarcoidosis

A
  • 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
Q

Erythema nodosum

A
  • Hypersensitivity reaction which may result from drugs, infections, or induction of autoimmune disease
  • Development of multiple erythematous patches
38
Q
A

Cutaneous sarcoidosis (lupus pernio)

39
Q
A

Cutaneous sarcoidosis

Lupus pernio variant

40
Q
A

Cutaneous sarcoidosis, subcutaneous variant

Lupus pernio

41
Q

Crohn’s disease

A
  • 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
Q

Normal IG vs Crohn’s GI vs UC GI

A
43
Q

Extracutaneous manifestations of Crohn’s

A
44
Q

Eosinophilic Granulomatosis with Polyangiitis

A
  • 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
Q

Suppurative granuloma

A
  • 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
Q

Naked granulomas are associated with. . .

A

. . . sarcoidosis

47
Q

When you diagnose a patient with sarcoidosis, always immediately order ___.

A

When you diagnose a patient with sarcoidosis, always immediately order an EKG.

48
Q

If a sarcoidosis patient is found to have heart involvement, what steps should be taken?

A

Implantation of a pacemaker and debibrillator.

49
Q

In a non-naked granuloma, the T cells will be . . .

A

. . . 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
Q

The apple jelly sign for granulomas is. . .

A

. . . very specific, but not very sensitive

51
Q

Findings: Reynaud’s and telangiectasias of the finger nail beds and palm.

What is your likely diagnosis?

A

Scleroderma

52
Q

Findings: Raynaud’s, malar rash, and alopecia

What is the likely diagnosis?

A

Lupus

53
Q

Findings: Raynaud’s, palpable purpura, and fingertip necrosis

What is your likely diagnosis?

A

Medium vessel vasculitis

54
Q

Findings: Raynaud’s, Gottron’s papules, and GERD

What is the likely diagnosis?

A

Dermatomyositis

55
Q

Gastric antral vascular ectasia

A

aka GAVE aka watermelon stomach

Associated with anti-RNA Pol III

HMS IDD (28 decks)

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