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Chapter 61: Sarcoidosis Melissa > Sarcoidosis > Flashcards

Sarcoidosis Flashcards

1
Q

Immune cells which play a role

A

Increased production of Th1 cytokines, including interleukin (IL)-2 and interferon (IFN)-γ, as well as release of tumor necrosis factor (TNF)-α by macrophages, leads to B-cell stimulation and hypergammaglobulinemia. IL-12 is also thought to play a role in sarcoidosis as are CD4+ T cells of the Th17 subtype3a.

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2
Q

What % of ppl with sarcoidosis have skin lesions?

A

30%

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3
Q

Favoured sites

A

Sarcoidal lesions favor the face, lips, neck, upper trunk and extremities

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4
Q

Clinical features of lupus pernio

A

Lupus pernio is characterized by papulonodules and plaques, primarily involving areas most affected by cold (i.e. pernio), including the nose, ears and cheeks; there is often a beaded appearance along the nasal rim. More common in women. Seldom resolve.

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5
Q

Associations of lupus pernio

A

chronic sarcoidosis of the lungs (approximately 75% of patients), upper respiratory tract (approximately 50% of patients), cystic lesions within the bones and phalanges.

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6
Q

DDx of lupus pernio

A

Wegeners, malignant pleomrophic lymphoma, lymphoma cutis, BCC, SCC, leishmaniasis, cutaneous lupus.

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7
Q

Tx of lupus pernio

A

Establish Diagnosis. Evaluate for systemic sarcoidosis. High potency topical and IL steroids, HCQ, MTX, MMF, Infliximab (3-7mg/kg) at 0,2,6 weeks, thalidomide.

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8
Q

Lofgren syndrome

A 
IF NELA
Acute iritis
Fever
EN
lymphadenopathy
Migratory arthritis 
95% HLADRB1*03 +ve will resolve in 2 years.
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9
Q

Heerfordt syndrome

A

PUFF
(uveoparotid fever) includes parotid gland enlargement, uveitis, fever, and cranial nerve palsies, usually of the facial nerve.

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10
Q

% with hypercalcaemia and why?

A

10%, increase in calcitriol synthesis by sarcoidal histiocytes

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11
Q

What occurs/does not occur in childhood sarcoidosis

A

Lung involvement rare

triad of arthritis, uveitis, cutaneous lesions + constitutional Sx.

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12
Q

Investigations & expected results

A 
FBC - leukopenia, lymphopenia
ESR - elevated
eLFTs - check Ca, liver involvement, kidney involvement 
ACE: may be elevated in 60%
VitD - may be high 
urinary calcium: may be high - risk of nephrolithiasis
CXRay: bilateral hilar adenopathy
gadolinium enhancement on MRI/PET, Spirometry DLco, KLco
Ophthal: slit lamp
ECG, Echo
TB skin test - may be depressed 
sputum MCS exclude infection
Kviem test - not done anymore 
Bx: see histo card
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13
Q

Histo

A

naked granulomas. Epithelioid cells with pale staining nuclei.
No caseation.
Inclusion bodies eg Schaumann (cytoplasmic inclusion body), Asteroid (stellate bodies): central core with radiating spicules.

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14
Q

Diagnosis

A

is a dx of exclusion, requires proof in 2 separate organs, but histological confirmation is not required in second organ

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15
Q

Types of sarcoidosis

A 
localised vs generalised
popular/micropapular
plaque
lupus pernio
Subcutaneous
Annular
Angiolupoid
Scar
Verrucous
Photodistributed
Ichthyosiform 
Mucosal/nail/alopecia 
Atrophic
Ulcerative 
Erythrodermic
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16
Q

Other name for subcutaneous sarcoidosis

A

Darier-Roussey

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17
Q

Causes of drug induced sarcoidosis

A

IFNalpha, TNFalpha

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18
Q

What to do if histo comes back with granuloma

A

Incisional biopsy for atypical mycobacteria, TB (eg lupus vulgaris) and deep fungal culture. Chest xray, QF gold

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19
Q

Histo DDx of sarcoidal granuloma

A

Deep fungal, mycobacterial

FB eg silica, tattoo, beryllium (should be polarised), granulomatous MF, crohns, granulomatous rosacea, Hodgkin

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20
Q

oral pred dose for systemic disease

A

1mg/kg for 4-6 weeks then slow taper over months-years

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21
Q

Treatment options for cutaneous sarcoidosis

A 
Low dose pred 
Potent topical CS, topical calcineurin, IL steroids, PUVA
HCQ
minocycline 200mg/day
MTX 10-25mg/week
thalidomide 50-300mg/day
isotretinoin 1mg/kg/day for 3-8 months 
allopurinol 100-300mg/day 
Infliximab -but may also trigger it (etanercept trial terminated early due to lymphoproliferative)
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22
Q

General prognosis

A

• 60% these will resolve with no problems in 2-5 years but is chronic or progressive in 10-30%

23
Q

Organs affected

A

o May affect any organ (maybe not adrenal)

 Most frequently affected: lymph nodes, lungs, liver, spleen, skin, eyes, small bones of hands, feet, salivary glands

24
Q

What is the Kveim test?

A

o Kveim rection is positive in most active cases (spleen of +ve patient injected into suspected patient – if granuloma present in 4-6 weeks, +ve)

25
Q

scar sarcoidosis

A

Scar - prefers traumatised tissue, around embedded FB. Inflammation of old scars may parallel or precede systemic disease activity. May be tender or pruritic.
1) acute eruptive phase, 2) later stage – may be warning sign of other organ involvement, 3) inoculation sites
Can be a warning sign of systemic disease
Most common on knees

26
Q

Angiolupoid

A

Angiolupoid: pink papules and plaques with prominent telangiectasis on face and may be a variant of lupus pernio. Affects females, almost always at the side of the bridge of the nose, corner of the eye, below the inner edge of the eyebrow or adjacent cheek.

27
Q

What is mikulicz syndrome

A

Mikulicz syndrome (bilateral enlargement of lacrimal, parotid, sublingual and submandibular glands)

28
Q

Examination

A 
Full skin exam - ?EN
lymph nodes 
HSM
lungs
neuro
red eyes
29
Q

History

A 
Onset of condition
Systems review eg
lungs, eyes, CNS, abdo pain, kidney pain, arthritis 
cough, sob
exposures during occupation, TB exposure
30
Q

Good prognostic

A

females
EN + fevers and arthralgias (Lofgren) – 80% spontaneous remission in 4-6 weeks
Stage I CXR vs II,III
+ve tuberculin test and normal globulin levels
HLA-B28 – more likely to spontaneously resolve
60% f ppl with stage 1 pulmonary disease will have recovered within 2 years
Papular type (small nodular)

31
Q

Poor prognosis

A 
African descent
Extrathoracic disease
Stage II-III
Age >40
Splenic involvement
Lupus pernio (URT involvement, pulmonary fibrosis and bony cyst associations) 
Disease duration >A 2 years
FVC
32
Q

Indications for tx

A 
Symptomatic pulmonary disease
Progressive or persistent parenchymal lung disease after 2 years
Posterior ocular disease or anterior disease 
Fever or weight loss
Liver disease with significant dysfunction or hepatosplenomegaly (synthetic dysfunction)
Skin disease or lymphdenompathy
Hypercalcaemia
Nervous system disae
Myocardial
Myopathy or myositis
Thrombocytopenia
Other significant organ involvement
33
Q

Monitoring

At each visit:

A

RV 6 monthly if treated
If no therapy rv 12 monthly
Follow for at least 3 years after therapy discontinued
Med Hx: SOB, fatigue, cough, weight loss, ocular problems, neuro problems
Exam: lung, cardiac, abdo, skin, lymph nodes, neuro exam
Annual tests: Eye, ECG, Chest xray, RFTs, FBC, serum chemistries

34
Q

Associated diseases

A
  • Infections – particularly wart virus
  • Immunologically mediated conditions eg thyroid disease, connective tissue
  • Effects of infiltration: granulomas in pituitary or thyroid
  • Vasculitis with sarcoidosis
  • Malignancy: may precede development of lymphoma by 18 months to 28 years. Increased risk of thyroid cancer and leukaemia
  • NLD and GA
35
Q

Other sarcoidal like reactions

A

FB, crohns, infections, lymphoma, granulomatous cheiltiis

36
Q

Exposures that are triggers

A

Hep c, interferon alpha, mycobacterium, p acnes, ebv, hsv

Dust, metals, mould, combustible wood products

37
Q

Eyes involved how often

A

25-75%

38
Q

Eye signs

A

Uveitis, cataracts, glaucoma, iris nodules, retinochoroiditis, conjunctivitis, lacrimal gland involvement, optic nerves, orbital involvement, heerfordt syndrome (anterior uveitis)

39
Q

Enlarged lymph nodes

A

50%

40
Q

Spleen enlarged in %

A

15%

41
Q

% Cardiac involvement

A

5%

42
Q

Mikulicz syndrome

A

Bilateral enlargement of lacrimal, parotid, sublingual, submandibular glands

43
Q

Raised lfts in what %

A

1/3

44
Q

Ecg abnormal in %

A

14%

45
Q

Cns involvement

A

Cranial nerve palsies, diabetes insipidus, hypopituitarism, endocrine abnormalities

46
Q

Associations with sarcoidosis

A 
Vain me 
Vasculitis, AI eg thyroid, chronic urticaria, ctd
Infection: hpv, aspergillus, mycetoma
Nld and ga
MalignancY: solid tumours, lymphoid 
Endocrine: infiltration eg cushings, DI
47
Q

% hypercalciuria

A

40%, 11% hypercalcaemia

48
Q

Bone and joints

A

Polyarthralgia, bone cysts, small hands and feet classically

49
Q

Cardiac

A

20-50% often young, sudden death, myocarditis, conduction, pericarditis

50
Q

% stage 1 on chest X-ray

A

35-45%

51
Q

If abn chest X-ray - % with abnormal lung function tests

A

50-70%

52
Q

3 ocular syndromes

A

Lofgrens
Heerfordt
Mikulicz
Kc sicca and parotid, lacrimal enlargement
Lupus pernio, chronic iridocyclitis, bone cysts, pulmonary fibrosis

53
Q

% ocular involvement

A

25-75%

54
Q

Ddx

A 
Ga, nld
Tb, leprosy, lupoid leishmaniasis
Rosacea
Cutaneous crohns
Syphilis

Chapter 61: Sarcoidosis Melissa (1 decks)

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