Young adults; Black women in the US; Northern Europeans.
Th1 (CD4) response to an unknown antigen → noncaseating granulomas in many organs
Diagnosis of exclusion
Bilateral hilar lymphadenopathy, often found incidentally; interstitial disease (restrictive) with dry cough, dyspnea.
Granulomas along lymphatics
Can progress to upper-lobe fibrosis
Erythema nodosum, lupus pernio (violaceous nose/cheeks), skin nodules; anterior uveitis.
Granulomas / panniculitis
Löfgren: BHL + erythema nodosum + ankle arthritis + fever → good prognosis
Heart (heart block, restrictive CM, sudden death), CNS (facial palsy, diabetes insipidus), parotids, liver, marrow, kidney.
Granulomatous infiltration
Heerfordt: uveitis + parotitis + facial palsy + fever
↑ ACE, hypercalcemia/hypercalciuria, ↑ CD4:CD8 in BAL, hypergammaglobulinemia, cutaneous anergy.
Granuloma macrophages make ACE and calcitriol (1α-hydroxylase)
Kidney stones; PPD may be negative
Tight noncaseating granulomas with Schaumann bodies (laminated calcium) and asteroid bodies (stellate inclusions in giant cells).
Often remits on its own
Steroids for organ-threatening disease
Hypersensitivity pneumonitis
Inhaled organic antigens: farmer's lung (thermophilic actinomycetes in moldy hay), bird fancier's lung, hot-tub lung (MAC), humidifiers.
Type III (acute) + type IV (chronic); loose peribronchiolar granulomas; ↑ CD8 lymphocytes in BAL
Fever, cough, dyspnea hours after exposure · chronic → upper-lobe fibrosis · avoid antigen, steroids
Eosinophilic lung disease
Löffler syndrome (migrating parasite larvae — Ascaris, Strongyloides — or drugs), chronic eosinophilic pneumonia, ABPA, EGPA.
Eosinophil-rich infiltrates
Chronic eosinophilic pneumonia: peripheral "photographic negative" of pulmonary edema