Extrapulmonary findings in the interstitial lung diseases

Physical findings	Associated conditions
Systemic arterial hypertension
	Systemic rheumatic disease, neurofibromatosis, anti-GBM antibody syndrome, systemic vasculitis
Skin
Discoid lupus	IPF, SLE, drug-induced SLE
Maculopapular rash	Drug-induced, amyloidosis, systemic rheumatic disease, Gaucher's disease
Heliotrope rash; Gottron papules; poikiloderma	Dermatomyositis
Mechanic's hands	Antisynthetase syndrome
Palmar papules, ulcerating lesions	Amyopathic dermatomyositis
Telangiectasia, sclerodactyly, hyperpigmentation/hypopigmentation, digital ulcers or pitting	Systemic sclerosis (scleroderma)
Raynaud phenomenon	Systemic rheumatic disease (scleroderma, SLE, mixed systemic rheumatic disease)
Cafe-au-lait spots, neurofibromas	Neurofibromatosis
Cutaneous vasculitis (eg, palpable purpura)	Systemic vasculitides; systemic rheumatic disease
Reticulated or mottled skin hyperpigmentation, nail dystropy, mucosal leukoplakia	Dyskeratosis congenita
Albinism	Hermansky-Pudlak syndrome
Calcinosis	Dermatomyositis-polymyositis; scleroderma
Subcutaneous nodules	Rheumatoid arthritis, neurofibromatosis, ANCA-associated vasculitis
Erythema nodosum (deep, painful nodules predominantly on anterior surfaces lower extremities)	Sarcoidosis; systemic rheumatic disease; Behçet syndrome; inflammatory bowel disease; histoplasmosis, coccidioidomycosis
Eyes
Uveitis	Sarcoidosis (eg, mutton-fat keratic precipitates), Behçet syndrome (eg, panuveitis, hypopyon), ankylosing spondylitis (eg, acute anterior uveitis)
Scleritis	ANCA-associated vasculitis, SLE, systemic sclerosis (scleroderma), sarcoidosis
Keratoconjunctivitis sicca	Lymphocytic interstitial pneumonia (in Sjögren syndrome)
Cherry red macula, macular halo	Neiman-Pick disease
Hematologic and reticuloendothelial system
Peripheral lymphadenopathy	Sarcoidosis, lymphangitic carcinomatosis, lymphocytic interstitial pneumonia, lymphoma, LAM-TSC
Hepatosplenomegaly	Sarcoidosis, pulmonary Langerhans cell histiocytosis, systemic rheumatic disease, amyloidosis, lymphocytic interstitial pneumonia
Anemia, thrombocytopenia, hypocellular marrow	Dyskeratosis congenita
Heart
Pericarditis, pericardial effusion	Radiation pneumonitis, systemic rheumatic disease, LAM-TSC
Cardiomyopathy	ANCA-associated vasculitis, sarcoidosis, SLE
Musculoskeletal/neurologic
Muscle weakness	Systemic rheumatic disease, drugs (eg, statins), sarcoidosis
Nervous system abnormalities	Pulmonary Langerhans cell histiocytosis, Neiman-Pick disease, neurofibromatosis, sarcoidosis, SLE, systemic vasculitis, TSC
Arthritis	ANCA-associated vasculitis, Rheumatoid arthritis, SLE, sarcoidosis (predominantly periarthritis of ankles and knees)
Gastrointestinal/renal
Glomerulonephritis	Anti-GBM antibody syndrome, ANCA-associated vasculitis, sarcoidosis
Nephrotic syndrome	Amyloid, drug-induced, sarcoidosis, SLE
Renal mass (eg, angiomyolipoma)	TSC, LAM
Gastrointestinal symptoms (eg, diarrhea, abdominal pain, hematochezia)	Pneumonitis related to inflammatory bowel disease
Other
Salivary or lacrimal gland enlargement	Sarcoidosis, lymphocytic interstitial pneumonia (in Sjögren syndrome), IgG4-related disease
Pleural abnormalities	Systemic rheumatic disease, LAM-TSC (chylous effusion), abestosis

GBM: glomerular basement membrane; IPF: idiopathic pulmonary fibrosis; SLE: systemic lupus erythematosus; ANCA: antineutrophil cytoplasmic antibodies; LAM: lymphangioleiomyomatosis; TSC: tuberous sclerosis complex; IgG4: immunoglobulin G4.

Adapted from:

Schwarz MI, King TE Jr, Cherniack RM. General principles and diagnostic approach to the interstitial lung diseases. In: Murray JF, Nadel JA, (Eds), Textbook of Respiratory Medicine, 2nd ed, Philadelphia, WB Saunders Co, 1994, pp. 1803-1826.
Cosgrove GP, Schwarz MI. Approach to the evaluation and diagnosis of interstitial lung diseases. In: Interstitial Lung Disease, 5th ed, Shelton, CT, Peoples Medical Publishing House, 2011, pp. 3-33.

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Extrapulmonary findings in the interstitial lung diseases