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pulm.sarcoidosis.v1PRODUCTION
pulm.sarcoidosis.v1

Sarcoidosis (pulmonary + extrapulmonary)

pulmonologychronicsubacuteacuteadult
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12/12 authored

Canonical 12-phase frame with authored status for this dossier.

Current phase

Frame

Detailed

Confirm sarcoidosis = compatible clinico-radiological picture + non-caseating granuloma on tissue + EXCLUSION of alternatives. Diagnosis is "never fully secure" (ATS 2020 PMID 32293205); pretest favored by bilateral hilar adenopathy + young adult + multisystem (BHL+EN+arthritis = Löfgren, LR for sarcoid very high, biopsy often unnecessary)

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Advance when

Granuloma confirmed (or classic Löfgren) AND TB/fungal/lymphoma/CBD excluded

Patient inputs (18)

Peak 20–40 yr; second peak women >50; cardiac more in <60 (ATS 2020)

Beryllium exposure (aerospace/nuclear/ceramic/dental) — chronic beryllium disease is histologically identical; ask in EVERY apparent sarcoid (ATS 2020)

Drug-induced sarcoid-like reaction (immune-checkpoint inhibitors, interferon, TNF-α, HAART-IRIS); immunosuppressant interactions (ATS 2020)

Hypercalcemia (1,25-OH-D driven), renal involvement; ATS 2020 strong rec for baseline serum calcium

Hepatic granulomas; baseline before MTX / AZA / TNF-α (ATS 2020)

Lymphopenia at baseline; cytopenias on MTX / AZA / TNF-α (ATS 2020)

MANDATORY before TNF-α (FDA boxed warning — TNF-α reactivates latent TB RR 4–12×); also rules out TB mimicking sarcoid (ATS 2020; CDC/ACR)

Scadding 0–IV staging — stage maps to spontaneous-remission probability and treat-vs-observe (ATS 2020)

Perilymphatic micronodules, upper-lobe predominance, fibrosis / honeycombing assessment (ATS 2020)

FVC + DLCO — functional severity drives treat-vs-observe and monitors response (ERS 2021)

AV block, fascicular block, low voltage, VT — screening for cardiac sarcoid in ALL patients (HRS 2014 PMID 24819193)

Non-caseating granuloma from least-invasive accessible site (skin / peripheral node / EBUS-TBNA / liver / endomyocardial) — required for diagnosis (ATS 2020)

Late-gadolinium CMR / FDG-PET when ECG/echo/symptoms suggest cardiac sarcoid — drives ICD + immunosuppression (HRS 2014)

African-American + Scandinavian higher prevalence + severity; AA more relapse/multiorgan (ACCESS PMID 11734441)

Serum ACE — supportive only, NOT diagnostic; sens ~57% spec ~90%, not for monitoring (ATS 2020)

1,25-OH elevated with suppressed PTH in sarcoid hypercalcemia (granuloma 1α-hydroxylase) (ATS 2020)

Hypercalciuria (more common than hypercalcemia); nephrocalcinosis; renal sarcoid (ATS 2020)

TPMT (and NUDT15) before azathioprine — deficiency → severe myelosuppression (ERS 2021)

* = hard-required. Engine cannot meaningfully run until these are filled.

Severity triggers (10)

10 need judgement
  • informationallife_threateningcardiac_sarcoidosis
    New high-grade AV block / sustained VT / LVEF reduction / FDG-PET active uptake in sarcoid patient (HRS 2014 PMID 24819193)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateningTB_unmasked_on_TNF_alpha
    TB reactivation on infliximab / adalimumab (fever, cough, weight loss, new infiltrate)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereneurosarcoidosis
    Bilateral facial palsy / aseptic meningitis / cord lesion / hydrocephalus / new seizure / optic neuropathy with sarcoid (ERS 2021 PMID 34140301)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverepre_tnf_alpha_latent_tb_screen_mandatory
    Latent TB screening MANDATORY before infliximab / adalimumab (or any TNF-α) for sarcoid — IGRA (preferred) or TST + CXR + risk review
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseveresarcoid_hypercalcemia_crisis
    Calcium >14 (or symptomatic + AKI/AMS) with PTH suppressed + 1,25-OH-D elevated in sarcoid (granuloma 1α-hydroxylase) (ATS 2020 PMID 32293205)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseveresevere_uveitis_vision_threat
    Posterior/panuveitis with vision loss or cystoid macular edema (ATS 2020)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereprogressive_pulmonary_fibrosis_or_SAPH
    Scadding IV fibrosis with declining FVC despite immunosuppression, OR echo TRV >2.8 m/s / RV dysfunction (sarcoidosis-associated pulmonary hypertension, WHO Group 5) (ERS 2021)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderateheerfordt_syndrome
    Uveoparotid fever: parotid enlargement + anterior uveitis + facial nerve palsy ± fever
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderatepregnancy_with_sarcoid
    Pregnancy / pregnancy-planning in patient on immunosuppression (ERS 2021)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmildlofgren_syndrome
    Acute BHL + erythema nodosum + ankle arthritis ± fever ± uveitis (good-prognosis variant)
    Trigger could not be auto-evaluated — needs clinician judgement.

Workflow calculators

Run this disease's risk and dosing calculators inline.

RISK_STRATIFICATIONoptionalDrives severity classification
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Recommended regimen

ERS 2021 / HRS 2014 sarcoidosis treat-vs-observe + organ-driven stepwise ladder
axis: sarcoidosis_immunosuppression_ladderstep 1 - Step 0/1 — Observation (do not treat)
Selected step "Step 0/1 — Observation (do not treat)" — Asymptomatic Scadding I/II, mild Scadding II without functional impairment, classic Löfgren, isolated extrathoracic disease NOT threatening organ function. Spontaneous remission: Scadding I ~55–90%, II ~40–70%, Löfgren >85% by 2 yr; steroid-induced remission relapses 74% vs 8% spontaneous (Gottlieb Chest 1997 PMID 9118698)
  • structured observation (no immunosuppression) ± NSAID for Löfgren arthralgia
    first line
    watchful_waiting
    FVC/DLCO + symptom review q3–6 mo; NSAID PRN for Löfgren ankle arthritis • n/a • q3–6 month surveillance
    triggers: asymptomatic_scadding_I_or_II, lofgren_syndrome, isolated_non_organ_threatening
    ERS 2021 (PMID 34140301): treatment is to reduce morbidity/mortality or improve QoL — NOT to normalise the CXR. Most Scadding I/II remit; premature steroid commits to taper + relapse. Löfgren good prognosis: NSAID for arthralgia, avoid steroid escalation

outpatient playbook — drug actions (5)

  1. 1. observation (no drug)
    FVC/DLCO + symptom review q3–6 mo; NSAID PRN for Löfgren arthralgia • n/a • q3–6 mo
    trigger: Asymptomatic Scadding I/II, Löfgren, isolated non-organ-threatening
    Most remit spontaneously; steroid-induced remission relapses 74% vs 8% (Gottlieb PMID 9118698)
  2. 2. prednisone
    20–40 mg/day (cardiac/neuro 0.5–1 mg/kg); taper 5–10 mg q4–8 wk, total 12–18 mo • PO • daily then taper
    trigger: Symptomatic/progressive lung OR any organ-threatening
    ERS 2021 first-line (PMID 34140301)
  3. 3. methotrexate (+ folate) or azathioprine
    MTX 10–15 mg/wk + folic acid 5 mg/wk; AZA 1–3 mg/kg/day after TPMT • PO/SC • weekly / daily
    trigger: Steroid toxicity / >10 mg/day need / chronic disease
    MTX steroid-sparing RCT PMID 10746262; Cochrane PMID 16856012
  4. 4. infliximab (screen latent TB first)
    3–5 mg/kg IV 0/2/6 wk then q4–8 wk • IV • q4–8 wk
    trigger: Refractory / CNS-neuro / refractory cardiac / severe ocular
    Pulmonary RCT PMID 16840744 (FVC +2.5% p=0.038); CNS series PMID 29030454
  5. 5. hydroxychloroquine (adjunct)
    200–400 mg/day (≤5 mg/kg/day) • PO • daily
    trigger: Cutaneous sarcoid / hypercalcemia
    ERS 2021 cutaneous + hypercalcemia adjunct

Auto-drafted A&P note

outpatient

Subjective

- Possible entry pathways: Bilateral hilar adenopathy ± pulmonary infiltrates on chest imaging (ATS 2020 dx CPG PMID 32293205); Erythema nodosum, lupus pernio, or other cutaneous sarcoid (ATS 2020); Acute Löfgren syndrome: BHL + erythema nodosum + ankle arthritis ± fever (good-prognosis variant, spontaneous remission >85% at 2 yr).

Objective

- No vitals, labs, or imaging entered for this encounter.

Assessment

**Sarcoidosis (pulmonary + extrapulmonary)** (pulm.sarcoidosis.v1).
Phenotype framing: Bayesian: sarcoid vs TB (caseating granuloma + AFB/NAAT — biopsy/culture is the pivot; route pulm.tuberculosis.v1) vs lymphoma (asymmetric bulky nodes, B-sx — EXCISIONAL node biopsy + flow, NOT FNA) vs chronic beryllium disease (BeLPT positive; identical histology — occupational pivot) vs fungal (histo/coccidio/blasto serology+culture) vs hypersensitivity pneumonitis (exposure + lymphocytic BAL, poorly-formed granuloma) vs IgG4-RD (storiform fibrosis, IgG4+ plasma cells, serum IgG4) vs metastatic malignancy (route onc as applicable). Non-caseating granuloma is necessary NOT sufficient — exclusion is the diagnostic act (ATS 2020 PMID 32293205)
Scope: Confirm sarcoidosis = compatible clinico-radiological picture + non-caseating granuloma on tissue + EXCLUSION of alternatives. Diagnosis is "never fully secure" (ATS 2020 PMID 32293205); pretest favored by bilateral hilar adenopathy + young adult + multisystem (BHL+EN+arthritis = Löfgren, LR for sarcoid very high, biopsy often unnecessary)

No severity triggers fired against current inputs.

Plan

Regimen axis: **ERS 2021 / HRS 2014 sarcoidosis treat-vs-observe + organ-driven stepwise ladder** — step "Step 0/1 — Observation (do not treat)".
1. structured observation (no immunosuppression) ± NSAID for Löfgren arthralgia FVC/DLCO + symptom review q3–6 mo; NSAID PRN for Löfgren ankle arthritis n/a q3–6 month surveillance (watchful_waiting, first line) — ERS 2021 (PMID 34140301): treatment is to reduce morbidity/mortality or improve QoL — NOT to normalise the CXR. Most Scadding I/II remit; premature steroid commits to taper + relapse. Löfgren good prognosis: NSAID for arthralgia, avoid steroid escalation

Setting playbook (outpatient) — Confirm diagnosis + exclude mimics, map organ extent (Scadding + cardiac/neuro/ocular/Ca), make treat-vs-observe decision, run organ-driven immunosuppression ladder, supervise taper + monitoring (ERS 2021 PMID 34140301)
2. observation (no drug) FVC/DLCO + symptom review q3–6 mo; NSAID PRN for Löfgren arthralgia n/a q3–6 mo — Asymptomatic Scadding I/II, Löfgren, isolated non-organ-threatening (Most remit spontaneously; steroid-induced remission relapses 74% vs 8% (Gottlieb PMID 9118698))
3. prednisone 20–40 mg/day (cardiac/neuro 0.5–1 mg/kg); taper 5–10 mg q4–8 wk, total 12–18 mo PO daily then taper — Symptomatic/progressive lung OR any organ-threatening (ERS 2021 first-line (PMID 34140301))
4. methotrexate (+ folate) or azathioprine MTX 10–15 mg/wk + folic acid 5 mg/wk; AZA 1–3 mg/kg/day after TPMT PO/SC weekly / daily — Steroid toxicity / >10 mg/day need / chronic disease (MTX steroid-sparing RCT PMID 10746262; Cochrane PMID 16856012)
5. infliximab (screen latent TB first) 3–5 mg/kg IV 0/2/6 wk then q4–8 wk IV q4–8 wk — Refractory / CNS-neuro / refractory cardiac / severe ocular (Pulmonary RCT PMID 16840744 (FVC +2.5% p=0.038); CNS series PMID 29030454)
6. hydroxychloroquine (adjunct) 200–400 mg/day (≤5 mg/kg/day) PO daily — Cutaneous sarcoid / hypercalcemia (ERS 2021 cutaneous + hypercalcemia adjunct)

Non-pharmacologic actions:
- Non-live vaccinations before/on immunosuppression (ATS 2020)
- Bone protection (Ca/vit-D normally — but NOT if hypercalcemic; bisphosphonate per FRAX) on chronic steroid (ERS 2021)
- PJP prophylaxis if combined immunosuppression (ERS 2021)
- Cardiac MRI/FDG-PET baseline if ECG/echo/symptom abnormal (HRS 2014)
- Sun/UV + vitamin-D/calcium-supplement avoidance if hypercalcemia (ATS 2020)

AVOID / contraindication checks:
- Pre_TNF_alpha_latent_TB_screen_MANDATORY_IGRA_or_TST_plus_CXR_treat_LTBI_first (FDA boxed warning; CDC/ACR — TNF α reactivates latent TB RR 4–12×)
- Pre_TNF_alpha_HBV_HCV_screen (reactivation risk)
- MTX_teratogen_avoid_pregnancy_switch_to_AZA + folate_5mg_weekly + avoid_eGFR_lt_30
- AZA_check_TPMT_and_NUDT15_before_start_avoid_allopurinol_co_administration (ERS 2021)
- MMF_teratogen_REMS_pregnancy_prevention
- HCQ_baseline_then_annual_retinal_screen_max_5mg_per_kg_per_day
- Steroid_taper_minimum_12_to_18mo_no_abrupt_stop (relapse 74% steroid induced vs 8% spontaneous — Gottlieb PMID 9118698; adrenal insufficiency)
- Sarcoid_hypercalcemia_AVOID_vitamin_D_and_calcium_supplements_and_sun_UV (1,25 OH D driven; bisphosphonate ineffective without steroid)
- Cardiac_sarcoid_device_NOT_replaced_by_immunosuppression_ICD_or_PPM_per_HRS_2014
- Non_live_vaccines_only_on_immunosuppression + PJP_prophylaxis_if_combined_IS + bone_protection_on_chronic_steroid

Monitoring

Regimen monitoring:
- FVC DLCO q3 to 6m pulmonary sarcoid (ERS 2021 PMID 34140301)
- corrected Ca plus 24h urine Ca if history hypercalcemia (ATS 2020 PMID 32293205)
- CBC LFT q4 to 8w on MTX or AZA or MMF (ERS 2021)
- baseline then annual retinal exam on HCQ
- annual TB screen on TNF alpha (FDA boxed warning)
- echo and cardiac MRI or FDG PET q6 to 12m cardiac sarcoid (HRS 2014 PMID 24819193)
- MRI brain q6m neurosarcoid (recurs same location ~56% on TNF-α stop — Gelfand PMID 29030454)
- BP glucose DEXA on chronic steroid + supervised taper 5 to 10mg q4 to 8w total 12 to 18mo
- serum ACE NOT used for monitoring disease activity (ATS 2020 — supportive only)

Setting (outpatient) monitoring:
- FVC + DLCO q3–6 mo (ERS 2021)
- CBC/LFT q4–8 wk on MTX/AZA/MMF (ERS 2021)
- Annual retinal exam on HCQ; annual TB screen on TNF-α
- Corrected Ca + 24-h urine Ca if hypercalcemia history (ATS 2020)

Follow-up plan: Pulm + organ-relevant specialty q3–6 mo; non-live vaccinations before/on immunosuppression; PJP prophylaxis if combined IS; bone protection on chronic steroid; fatigue + small-fiber neuropathy + QoL screen (ERS 2021)
- Close-out criterion: Follow-up + prevention bundle booked

Monitoring phase: FVC+DLCO q3–6 mo (pulmonary), CXR, corrected Ca + 24-h urine Ca, organ-specific imaging; on-steroid BP/glucose/DEXA; on-MTX/AZA CBC+LFT q4–8 wk; on-HCQ annual retinal exam; on-TNF-α annual TB screen; cardiac sarcoid echo/CMR q6–12 mo; neuro MRI q6 mo; supervise prednisone taper (5–10 mg q4–8 wk; total 12–18 mo) to avoid relapse + adrenal insufficiency (ERS 2021)

Disposition

Current setting: outpatient — Confirm diagnosis + exclude mimics, map organ extent (Scadding + cardiac/neuro/ocular/Ca), make treat-vs-observe decision, run organ-driven immunosuppression ladder, supervise taper + monitoring (ERS 2021 PMID 34140301)

Disposition criteria:
- Continue ladder + surveillance if stable (ERS 2021)
- Refer transplant clinic if progressive Scadding IV despite therapy (ERS 2021)

Escalation triggers (move to higher acuity):
- New cardiac symptom / arrhythmia → STAT ECG + cardiac MRI/FDG-PET + EP (HRS 2014)
- New neuro deficit → MRI brain/spine + LP (ERS 2021)
- Hypercalcemia + rising creatinine → urgent steroid + saline (ATS 2020)
- FVC decline ≥10% or DLCO ≥15% → step up immunosuppression (ERS 2021)

Earlier-Return Triggers

Return-precaution thresholds (watch for):
- [LIFE_THREATENING] New high-grade AV block / sustained VT / LVEF reduction / FDG-PET active uptake in sarcoid patient (HRS 2014 PMID 24819193)
- [LIFE_THREATENING] TB reactivation on infliximab / adalimumab (fever, cough, weight loss, new infiltrate)
- [SEVERE] Bilateral facial palsy / aseptic meningitis / cord lesion / hydrocephalus / new seizure / optic neuropathy with sarcoid (ERS 2021 PMID 34140301)

Citations

- ATS 2020 Diagnosis & Detection of Sarcoidosis CPG (Crouser AJRCCM 2020; PMID 32293205) + ERS 2021 Treatment of Sarcoidosis CPG (Baughman Eur Respir J 2021; PMID 34140301) + HRS 2014 cardiac sarcoid expert consensus (Birnie Heart Rhythm 2014; PMID 24819193) [PMID:32293205](https://pubmed.ncbi.nlm.nih.gov/32293205/)
- Cited evidence (PMID 34140301) [PMID:34140301](https://pubmed.ncbi.nlm.nih.gov/34140301/)
- Cited evidence (PMID 24819193) [PMID:24819193](https://pubmed.ncbi.nlm.nih.gov/24819193/)
- Cited evidence (PMID 29030454) [PMID:29030454](https://pubmed.ncbi.nlm.nih.gov/29030454/)
- Cited evidence (PMID 16840744) [PMID:16840744](https://pubmed.ncbi.nlm.nih.gov/16840744/)

Last reconciled with current guidelines: 2026-05-16.
References
  • ATS 2020 Diagnosis & Detection of Sarcoidosis CPG (Crouser AJRCCM 2020; PMID 32293205) + ERS 2021 Treatment of Sarcoidosis CPG (Baughman Eur Respir J 2021; PMID 34140301) + HRS 2014 cardiac sarcoid expert consensus (Birnie Heart Rhythm 2014; PMID 24819193) — PMID:32293205
  • Cited evidence (PMID 34140301) — PMID:34140301
  • Cited evidence (PMID 24819193) — PMID:24819193
  • Cited evidence (PMID 29030454) — PMID:29030454
  • Cited evidence (PMID 16840744) — PMID:16840744