Sarcoidosis

Question 1

Defintion

Answer 1

Inflammatory condition defined by the presence of non-caseating granulomas (small patches of red and swollen tissue).
Multi-systemic disease which any organ can be affected

Question 2

Epidemiology

Answer 2

Afro-Caribbean and Scandinavian ethnicity
Young adults: commonly presents at 20-40 years of age
Female gender
Family history

Question 3

Aetiology

Answer 3

Unknown
Maybe due to a type IV hypersensitivity reaction against an unknown antigen. A T-cell-mediated immune response to an antigenic stimulus causes the formation of granulomas, in which macrophages begin to release local mediators that result in inflammation

Question 4

Acute sarcoidosis features

Answer 4

Swinging fever,
Polyarthralgia
Erythema nodosum

Question 5

Insidious sarcoidosis features

Answer 5

Non-productive cough,
Dyspnoea,
Fatigue

Question 6

Signs

Answer 6

Cervical and submandibular lymphadenopathy
Lupus pernio: a lupus-type rash
Erythema nodosum: dusky coloured nodules on the shins

Question 7

Symptoms

Answer 7

Cough: non-productive
Dyspnoea: gradual onset
Polyarthralgia
Uveitis:
- Red-eye
- Photophobia
Constitutional symptoms: swinging fever, fatigue, weight loss

Question 8

Associated syndromes

Answer 8

Lofgrens syndrome
Heerfordt’s syndrome
Mikulicz’s disease

Question 9

Diagnosis

Answer 9

Largely clinical
Routine bloods: inflammatory markers may be raised and can screen for other organ involvement, e.g. renal function.
Serum calcium: hypercalcaemia, non-caseating granulomas consist of activated macrophages which have 1-alpha-hydroxylase activity and activate vitamin D
Angiotensin-converting enzyme (ACE): elevated, but with poor sensitivity and specificity, ACE is typically used to monitor disease
CXR: first-line imaging; may show hilar lymphadenopathy or bilateral infiltrates

Question 10

CXR findings in sarcoidosis

Answer 10

Stage 0 = normal
Stage 1 = bilateral hilar lymphadenopathy
Stage 2 = bilateral hilar lymphadenopathy and interstitial infiltrates
Stage 3 = diffuse pulmonary infiltrates without hilar lymphadenopathy
Stage 4 = diffuse fibrosis

Question 11

Management

Answer 11

FIRST LINE = Corticosteroids
- INHALED BUDESONIDE or ORAL PREDNISOLONE
SECOND LINE = Immunosuppressants
- METHOTREXATE or AZATHIOPRINE
END STAGE = lung transplantation
Acute respiratory failure = oral or IV corticosteroids + ventilatory support
Indications for steroids:
- symptomatic and stage 2/3 on CXR
- extra-pulmonary involvement e.g. ocular, neurological or cardiac disease
- hypercalcaemia

Question 12

Management of extrapulmonary disease

Answer 12

FIRST LINE = corticosteroids
- consider topical for cutaneous or ocular disease
SECOND LINE = immunosuppressants
- Methotrexate or Azathioprine

Question 13

Complications

Answer 13

Respiratory: pulmonary hypertension, respiratory failure, fibrosis (upper zone)
Cardiovascular: cor pulmonale, heart block
Central nervous system: cranial nerve palsies e.g. facial nerve, meningeal disease
Ocular: keratoconjunctivitis sicca, uveitis