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Affida > Sarcoidosis > Flashcards

Sarcoidosis Flashcards

1
Q

What is sarcoidosis?

A
  • Multisystem granulomatous disorder of unknown aetiology
  • Characterised by pathological non-caseating granulomas in involved organs.
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2
Q

What is the epi of sarcoidosis?

A

~10-20/100000
3-4x more in Black Americans
Age of onset: 20-60y

Rare in Asia:
Spore – 0.56/100000, predominantly Indians (49.2%), bimodal age distribution

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3
Q

What is the mortality risk and complications of sarcoidosis?

A
  • 5-year survival is 93–95%
  • Mortality higher in individuals with more severe disease at diagnosis and if more organs are involved
  • Higher risk of infection, CCF, CVA, VTE, autoimmune disease, malignancy
    Complications: Aspergilloma, Pulmonary HTN
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4
Q

What are the Sx of sarcoidosis?

A

Pulmonary: coughs, dyspnoea, wheeze (if with endobronchial involvement or traction bronchiectasis)

Others: chest pain, fatigue, malaise, fever, LOW, skin lesions, visual changes, dry eyes or mouth, parotid swelling, palpitations, syncope, joint pain, muscle weakness

Children: skin rash, erythema nodosum, arthritis, uveitis, lymph nodes, hyperglobulinemia, hyperCa

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5
Q

What are the investigations for sarcoidosis?

A

Requires:
- Compatible clinical & radiographic manifestation
- Exclusion of other similar disease

Laboratory testing:
FBC: anaemia, leukopenia, eosinophilia, thrombocytopenia
Ca (noted Hypercalciuria >common than hyperCa)
Cr, ALP
Serum Vit D
Hypergammaglobulinemia
Mantoux test
Elevated ACE level but poor sensitivity and specificity
Histopathology detection of noncaseating granulomas, except in:
Lӧfgren syndrome (Erythema nodosum + bilat hilar adenopathy + fever + arthritis)
Heerfordt syndrome (uveitis + parotiditis + fever)

Imaging:
CXR
HRCT
MRI scan – extrapulmonary
FDG-PET scan -
Gallium-67 scan showing uptake in parotid and lacrimal glands (Panda sign) with right paratracheal and bilateral hilar uptake (Lambda sign)
TTE

Others:
PFT
Opthalmic exam
Cardiac Ax (ECG)

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6
Q

What are the CXR stages of pulm sarcoidosis?

A

Stage I: bilateral hilar adenopathy
Stage II: bilateral hilar adenopathy and reticular opacities
Stage III: reticular opacities in the upper zones with shrinking hilar nodes
Stage IV: reticular opacities in the upper zones with volume loss

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7
Q

What are the CT changes of sarcoidosis

A

String of beads
Galaxy appearance

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8
Q

What are the DDx of sarcoidosis?

A

Mycobacterial infection – MTB, NTM
Fungal infection – histoplasmosis, blastomycosis, PJP
Hypersensitivity pneumonitis
Pneumoconiosis
Drug-induced hypersensitivity
Pulmonary histiocytic disorders
Foreign body granulomatosis

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9
Q

What are the Rx for sarcoidosis?

A

Depending on the organ involved
Most cases do not require Rx
Initial Rx: oral glucocorticoid 4-6w, then taper the dose if improving, inhaled glucocorticoid for coughs
Refractory to steroid: MTX, Aza, leflunomide, MMF
- Consider trial of combination of 2 immunosuppression, or adding TNF-a antagonist (infliximab, adalimumab)

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10
Q

What are the monitoring required in sarcoidosis?

A

Symptomatic: 1-2 monthly
Asx: 3-4 monthly for 1st year, then yearly

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11
Q

In sarcoidosis showing progression and fibrosis, what should be done?

A

Add on antifibrotic nintedanib, and stop other meds which are not effective

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12
Q

Dosage of meds used in sarcoidosis

A
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13
Q

ERS 2022 Clinical practice guideline for Mx of sarcoidosis

A
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14
Q

What is Lofgren syndrome?

A

Erythema nodosum + bilat hilar adenopathy + fever + arthritis

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15
Q

What is Heerfordt syndrome?

A

uveitis + parotiditis + fever

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16
Q

What are the complications from sarcoidosis?

A

VTE
Aspergilloma
Pulmonary HTN

16
Q

What are the adverse prognostic factors in sarcoidosis?

A
17
Q

DDx of granuloma on lung Bx

Reference: Oxford Resp Med Handbook

A
  • Sarcoidosis
  • tuberculosis
  • hypersensitivity pneumonitis
  • Granulomatosis with polyangiitis (Wegener’s)
  • Drug reactions
  • non-tuberculous mycobacteria infection
  • Fungal infections:
  • Cryptococcosis
  • aspergillosis
  • Coccidioidomycosis * Blastomycosis
  • aspiration of foreign material
  • primary biliary cirrhosis
  • Sarcoid-like reaction to malignancy.
18
Q

How does sarcoidosis cause hyperCa?

A

Hypercalcemia in sarcoidosis is due to the uncontrolled synthesis of 1,25-dihydroxyvitamin D3 by macrophages. 1,25-dihydroxyvitamin D3 leads to an increased absorption of calcium in the intestine and to an increased resorption of calcium in the bone.

19
Q

Indications for immunosuppressive Rx in sarcoidosis

A
  • Increasing symptoms, deteriorating pFts, and worsening CXr infiltrates
  • Cardiac sarcoidosis
  • neurosarcoidosis
  • Sight-threatening ocular sarcoidosis
  • hypercalcaemia
  • Lupuspernio
  • Splenic, hepatic, or renal sarcoidosis.
20
Q

BTS guidelines for the management of sarcoidosis (2008)

A
  • Rx not indicated in asymptomatic or mildly abnormal stage I, II & III disease because of high rates of remission.
  • 1st line: OCS in patients with progressive disease determined by radiology or on lung function, significant symptoms, or extrapulmonary disease requiring treatment
  • prednisolone (or equivalent) 0.5 mg/kg/day for 4 weeks, then reduced to a maintenance dose which will control symptoms and disease progression, for 6–24 months
  • Give high doses, such as 30 mg prednisolone/day, to control active disease. rarely need >40 mg/day. Usually give this high dose for 2–3 weeks, and then reduce if there has been a response
  • Maintenance dose of around 5–20 mg to control symptoms and prevent progression of disease. Leave on this dose for a few months, and then slowly reduce steroid dose further.
  • Maintain on low dose of prednisolone (5–7.5 mg/day or alternate days) for prolonged period of up to 12 months to consolidate resolution, before considering complete withdrawal.
  • remember bone protection with a bisphosphonate (avoid routine calcium and vitamin D supplementation)
  • Bisphosphonates should be used to minimize steroid-induced
    osteoporosis
  • Inhaled corticosteroids may be considered for
    symptom control (cough) in a subgroup of patients
  • other immunosuppressive or anti-inflammatory treatments only
    have a limited role but should be considered in patients when corticosteroids are not controlling the disease or side effects are intolerable. at present, methotrexate is the treatment of choice
  • Lung transplantation should be considered in end-stage pulmonary sarcoidosis.

If steroid treatment fails or sarcoidosis life-threatening, other immunosuppressive regimes may be indicated e.g. pulsed high-dose intravenous (IV) methylprednisolone, esp for neurosarcoidosis.
If steroid side effects cannot be tolerated, other immunosuppressive drugs should be considered. possibilities include azathioprine and methotrexate.

Affida (53 decks)

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