PassFinals
Respiratory

Sarcoidosis

Sarcoidosis is a multisystem inflammatory disease characterised by non-necrotising granulomas, usually involving the lungs and intrathoracic lymph nodes; management balances spontaneous remission against progressive, organ-threatening or quality-of-life-limiting disease after infection and malignancy have been excluded.

In a nutshell

Sarcoidosis is a multisystem non-necrotising granulomatous disease, most often involving the lungs and hilar nodes. Diagnosis requires a compatible clinical-radiological picture with infection and malignancy excluded; typical Lofgren syndrome may be monitored without biopsy. Treat only progressive, organ-threatening or quality-of-life-limiting disease, usually with specialist-directed corticosteroids and specialist second-line immunosuppression when needed.

Classic presentation

A person has cough or breathlessness with bilateral hilar lymphadenopathy, or acute erythema nodosum, ankle arthralgia and fever in Lofgren syndrome; ECG, calcium, eye and symptom screening look for dangerous extrapulmonary disease.

Key points

  • Non-necrotising granulomas support sarcoidosis only after tuberculosis, fungal infection, berylliosis, silicosis and malignancy have been considered.
  • Bilateral hilar or mediastinal lymphadenopathy is typical, but a normal chest radiograph does not exclude disease and isolated node enlargement is not a progression marker.
  • Baseline assessment includes blood count, renal and liver tests, calcium and ECG; serum ACE is neither diagnostic nor a reliable activity marker.
  • Lofgren syndrome often remits spontaneously; observation with planned follow-up is appropriate when no organ is threatened.
  • Treat when there is danger of death or permanent disability, progressive organ dysfunction or unacceptable quality-of-life loss, balancing steroid toxicity against benefit.
  • Cardiac, neurological, sight-threatening ocular, severe calcium-related and progressive fibrotic disease require urgent specialist involvement.

First-line investigation

Chest radiograph plus baseline calcium, blood count, renal/liver tests and ECG, followed by multidisciplinary CT and biopsy decisions when the presentation is atypical or diagnosis remains uncertain.

Management

Screen for organ threat and mimics

  • Urgently assess cardiac, neurological, ocular, calcium-related and severe respiratory red flags, and exclude important infection or malignancy before immunosuppression when clinically safe.1,2

Confirm the pattern and choose observe versus treat

  • Use chest imaging, selective tissue sampling and baseline organ screening; observe typical mild or Lofgren disease with an explicit follow-up plan when no organ is threatened.1

Treat progressive or organ-threatening disease

  • Use specialist-directed systemic corticosteroids when treatment is indicated, with BNF-checked safety monitoring and bone, metabolic, infection and adrenal safeguards.1,2

Escalate steroid-sparing and organ-specific care

  • Refer for methotrexate or another steroid-sparing agent when disease progresses, steroids cannot be tapered or toxicity is unacceptable; use organ-specific multidisciplinary teams for cardiac, neurological, ocular, renal, pulmonary-hypertension and transplant decisions.1,2

Monitor and taper safely

  • Trend symptoms, quality of life, lung function, calcium and selected imaging; consider a slow steroid-withdrawal trial after controlled disease has been stable, while monitoring for relapse and adrenal insufficiency.1,2

Exam traps

  • A biopsy showing non-necrotising granulomas does not exclude tuberculosis or malignancy; histology must be interpreted with microbiology, imaging and clinical context.
  • Serum ACE is neither sensitive nor specific and is a poor standalone measure of activity or treatment response.
  • Typical Lofgren syndrome often needs observation rather than immunosuppression, but severe symptoms or organ threat change the decision.
  • Do not assume mild chest imaging means mild disease: ask about syncope, palpitations, visual symptoms, neurological symptoms and calcium-related symptoms.
  • Do not use a single FVC, DLco, CT or ACE value in isolation to define progression; integrate symptoms, physiology and imaging in a specialist review.

Illustrations

Chest X-ray in sarcoidosis with prominent hilaPA chest radiograph showing bilaterally prominent, lobulated hila in sarcoidosis, the classic site of bilateral hilar lymphadenopathy.Mikael Häggström, Wikimedia Commons · CC0
Non-caseating granulomaA histology image of a non-caseating (non-necrotising) granuloma with epithelioid macrophages and surrounding lymphocytes, contrasted with the caseating granuloma of tuberculosis.Mutleysmith, Wikimedia Commons · Public domain
Lupus pernio: cutaneous extrapulmonary sarcoidosisAn illustration of extrapulmonary features: erythema nodosum, lupus pernio, uveitis and the multisystem distribution of granulomas.M. Sand, D. Sand, C. Thrandorf, V. Paech, P. Altmeyer, F. G. Bechara, Wikimedia Commons · CC-BY-2.0

Key sources

  1. British Thoracic Society, Clinical Statement on pulmonary sarcoidosis (Current BTS clinical statement covering diagnosis, biopsy decisions, extrapulmonary screening, treatment, second-line immunosuppression and follow-up; published 2 December 2020 and listed as valid by BTS)Published 2 Dec 2020
  2. BNF, corticosteroids and immunosuppressants (Current UK prescribing reference for corticosteroid safety, interactions, monitoring and immunosuppressant prescribing; access may require an NHS or institutional subscription)

This page is exam revision material, not medical advice, and must not be used for patient care. Always check drug doses against the BNF and current guidance. Full disclaimer.