Table 2.
Characteristics of sarcoidosis and some primary rheumatic diseases.
| Features | Sarcoidosis | Rheumatoid arthritis | Sjögren syndrome | Systemic lupus erythematosus | Systemic sclerosis | Dermatomyositis and polymyositis | Spondyloarthropathy |
|---|---|---|---|---|---|---|---|
| Age (year) | 20–40 | 30–50 | 30–50 | 15–35 | 35–50 | 45–55 | 25–45 |
| Gender (W/M) | 2/1 | 3/1 | 20/1 | 10/1 | 3–7/1 | 3/1 | 1/2 |
| Type | Insidious acute | Insidious | Insidious | Insidious | Insidious | Insidious | Insidious |
| Pathogenesis | Th1/Th17 CD4/CD8⇧ |
Th1/Th17 B cells |
Th1/Th17 B cells |
Th2 B cells |
Th1 B cells |
Th1/Th2 B cells |
Th1/Th17 |
| Genetic |
HLA-DRB1
HLA-DQB1 TNF-308A Butyrophilin-like 2 gene Annexin A11 |
HLA-DR4 HLA-DR1 PTPN22 PADI4 TRAF1/C5 STAT4 |
HLA-DRB1
HLA-DQA1 IRF5 STAT4 |
HLA-DR2 HLA-DR3 C1q insufficiency FCGR2A IRF5 PTPN22 |
HLA-DRB1
HLA-DQA1 HLA-DQB1 PTPN22 NLRP1 IRF5 |
HLA-DRB1
HLA-DQA1, B8, HLA-DR3 HLA-DR52 |
HLA-B27 Endoplasmic reticulum amino peptidase 1 Interleukin 23 receptor Interleukin I receptor type II Anthrax toxin receptor |
| Prevalence | 4–65/100,000 | 0.5–1% | 0.1–4.6% | 10–50/ 100,000 |
7–24/ 100,000 |
2.1–10/1,000,000 /year |
0.75–2% |
| Locomotor system involvement | Arthritis Myositis Dactylitis Sacroiliitis Enthesitis |
Arthritis Myositis |
Arthritis Myositis |
Arthritis Tenosynovitis Myositis Avascular necrosis |
Arthritis Tendonitis Myositis |
Myositis Arthritis |
Sacroiliitis Spondilitis Asymmetric oligoarthritis enthesitis |
| Pulmonary involvement | Hilar lymphadenopathy ILD PAH |
ILD Pleuritis Rheumatoid nodule PAH SAD |
SAD Nonspecific interstitial pneumonia ILD PAH |
Pleuritis ILD Lupus pneumonia Alveolar hemorrhagia PAH |
ILD PAH |
ILD PAH |
Apical lobe fibrosis |
| Neurologic involvement | Meningitis MNM İntracranial Tumor Multiple sclerosis-like features Peripheral neuropathy Multiple cranial neuritis |
CTS PNP MNM |
CNS Vasculitis PNS ANS MNM |
Cognitive Lupus headache Vasculitis Hemorrhagia Transverse myelitis MNM |
Neuropathy ANS |
Neuropathy | Cauda equina syndrome |
| Gastrointestinal system involvement | Rare (1–2%) | Rare | Esophagus AIH Painful bladder syndrome Vasculitis |
AIH Pancreatitis Vasculitis |
Esophagus Stomach Intestine |
Esophagus Stomach Intestine Vasculitis |
Inflammatory bowel disease |
| Cardiac involvement | Arrhythmias Cardiomyopathy/congestive heart failure Pericarditis Valvulopathy PAH |
Pericarditis Valvulopathy Atrioventricular block Myocarditis Coronary vasculitis |
Valvulopathy Arrhythmias AHD PAH |
Pericarditis Myocarditis Endocarditis AHD |
Pericarditis Myocarditis |
Myocarditis Arrhythmias |
Aortitis Aortic valvulopathy AHD |
| Ocular involvement | Uveitis Scleritis Sicca syndrome Orbital tumor Optic neuritis |
Sicca syndrome Scleritis Episcleritis Scleromalacia perforans |
Sicca syndrome Conjunctivitis Retinal vasculitis |
Retinal vasculitis Orbital myositis Episcleritis Sicca syndrome |
Sicca syndrome | Sicca syndrome | Anterior uveitis Conjunctivitis |
| Renal involvement | Calciuria Chronic renal failure |
AA amyloidosis | Tubulointerstitial nephritis Renal tubular acidosis GN |
GN | Scleroderma renal crisis | Rare | IgA nephropathy AA amyloidosis |
| Lymphadenopathy | Yes | Yes | Yes | Yes | Rare | Rare | No |
|
Skin-mucosa
involvement |
Erythema nodosum Lupus pernio Maculopapular Subcutaneous nodule Psoriasis-like |
Rheumatoid nodule Palmar erythema Rheumatoid vasculitis |
Raynaud Palpable purpura Annular erythema Pernio |
Malar rash Alopecia Discoid lupus Raynaud Livedo reticularis Paniculitis Oral lesion |
Raynaud Localized Diffuse Calcinosis |
Heliotropic rash Gottron papule V-erythema Machinist hand Raynaud Calcinosis |
Oral lesion Psoriatic lesion Keratoderma Blenorragica Balanitis circinata |
| Serology tests | ANA RF Anti-CCP (overlap) |
RF Anti-CCP ANA |
ANA Ro La Aquaporin-4 |
ANA dsDNA Sm Ro La Anti-cardiolipin antibodies Ribosomal P |
ANA Scl-70 Sentromer |
ANA Signal recognition particle Mi-2 tRNA synthetase |
None |
| Pathology | Noncaseating granuloma | Pannus | Autoimmune epithelitis |
Complement Ig |
Fibrosis Vasculopathy |
Muscle inflammation/ necrosis |
None |
| Treatment | CS, MTX, AZA, LEF, anti-TNF-alpha |
CS, MTX, LEF, SSZ, anti-TNF-alpha RTX, abatacept, tocilizumab, tofacitinib |
CS, HQ, MTX, RTX | CS, HQ, MTX, CyP, belimumab, RTX |
HQ, MTX, CyP, AZA, CS, Ca blocker, apremilast endothelin receptor antagonist |
CS, MTX, AZA, RTX, Iv İg, plasmapheresis |
Nonsteroidal anti-inflammatory drug, SSZ, anti-TNF-alpha, anti-interleukin-17 |
AHD, atherosclerotic heart disease; AIH, autoimmune hepatitis; ANA, antinuclear antibody; ANS, autonomic nervous system; anti-CCP: anti-cyclic citrullinated peptide; anti-TNF-alpha, anti-tumor necrosis factor-alpha; AZA, azathioprine; CHF, congestive heart failure; CMP, cardiomyopathy; CNS, central nervous system; CS, corticosteroid; CTS, carpal tunnel syndrome; CyP, cyclophosphamide; FCGR2A, Fc gamma receptor IIa; GN, glomerulonephritis; HQ, hydroxychloroquine; Ig, immunoglobulin; ILD, interstitial lung disease; IRF5, interferon regulatory factor 5; LEF, leflunomide; MNM, mononeuritis multiplex; MTX, methotrexate; PADI4, peptidyl arginine deiminase; PAH, pulmonary arterial hypertension; PNP, polyneuropathy; PNS, peripheral nervous system; PTPN22, protein tyrosine phosphatase-22; RF, rheumatoid factor; RTX, rituximab; SAD, small airway disease; SRC, scleroderma renal crisis; STAT4, signal transducer and activator of transcription4; Th, T helper cell; TRAF1/C5, tumor necrosis factor-receptor associated factor 1/complement component 5.