Caplan's syndrome

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Caplan's syndrome Classification and external resources
ICD-10	J99.0 M05.1
ICD-9	714.81
DiseasesDB	1961
MedlinePlus	000137
MeSH	D002205

Caplan's syndrome (or Caplan's disease) is a combination of rheumatoid arthritis and pneumoconiosis that manifests as intrapulmonary nodules, which appear homogenous and well-defined on chest X-ray.^[1]

Contents 1 Classification 2 Signs and symptoms 3 Causes 4 Pathophysiology 5 Diagnosis 6 Management 7 Prognosis 8 Epidemiology 9 History 10 References 11 External links

[edit] Classification

[edit] Signs and symptoms

There is cough and shortness of breath. In addition there are the features of RA with painful joints and morning stiffness. Examination should reveal tender, swollen MCP joints and rheumatoid nodules; auscultation of the chest may reveal diffuse râles that do not disappear on coughing or taking a deep breath.

[edit] Causes

The condition occurs in miners (especially those working in anthracite coal-mines), asbestosis, silicosis and other pneumoconioses. There is probably also a genetic predisposition and smoking is thought to be an aggravating factor.

[edit] Pathophysiology

[edit] Diagnosis

• Chest radiology shows multiple, round, well defined nodules, usually 0.5-2.0 cm in diameter, which may cavitate and resemble tuberculosis.
• Lung function tests may reveal a mixed restrictive and obstructive ventilatory defect with a loss of lung volume. There may also be irreversible airflow limitation and a reduced gas transfer factor.
• Rheumatoid factor, antinuclear antibodies, and non-organ specific antibodies may be present in the serum.
• Silicosis and asbestosis must be considered in the differential with TB.

[edit] Management

Once tuberculosis has been excluded, treatment is with steroids. All exposure to coal dust must be stopped, and smoking cessation should be attempted. Rheumatoid arthritis should be treated normally with early use of DMARDs.

[edit] Prognosis

The nodules may pre-date the appearance of rheumatoid arthritis by several years. Otherwise prognosis is as for RA; lung disease may remit spontaneously, but pulmonary fibrosis may also progress.

[edit] Epidemiology

Incidence is currently 1 in 100,000 people but is likely to fall as the coal mining industry declines.

[edit] History

Caplan's syndrome was originally described in coal miners with progressive massive fibrosis.

[edit] References

[edit] External links

• 00057 at CHORUS
• 932839427 at GPnotebook

v • d • e Pathology of respiratory system (J, 460-519), respiratory diseases Upper RT (including URTIs) Acute Common cold - Rhinitis - Sinusitis - Pharyngitis (Strep throat) - Tonsillitis - Laryngitis - Tracheitis - Croup - Epiglottitis Other nose disease (Vasomotor rhinitis, Hay fever, Atrophic rhinitis, Nasal polyp, Deviated septum) - Adenoid hypertrophy - Peritonsillar abscess - Vocal fold nodule - Laryngospasm Lower RT (including LRTIs) Pneumonia Viral - Bacterial - Bronchopneumonia Other acute Bronchitis (Acute, Chronic) - Bronchiolitis Chronic Emphysema - COPD - Asthma (Status asthmaticus) - Diffuse panbronchiolitis - Bronchiectasis Lung diseases due to external agents Pneumoconiosis (Asbestosis, Bauxite fibrosis, Berylliosis, Caplan's syndrome, Chalicosis, Coalworker's pneumoconiosis, Siderosis, Silicosis) - Byssinosis - Hypersensitivity pneumonitis (Bagassosis, Bird fancier's lung, Farmer's lung) Other lung disease, principally affecting the interstitium ARDS - Pulmonary edema - Löffler's syndrome - Eosinophilic pneumonia - Interstitial lung disease (Hamman-Rich syndrome, Idiopathic pulmonary fibrosis) Suppuration Empyema - Lung abscess Other pleural disease/pleural effusion (Pneumothorax, Hemothorax, Hemopneumothorax, Hydrothorax) mediastinal disease (Mediastinal emphysema, Mediastinitis) Mendelson's syndrome - Atelectasis Other/general Respiratory failure - Influenza - SARS see also congenital (Q30-Q34, 748)

v • d • e Diseases of the musculoskeletal system and connective tissue (M, 710-739) Arthropathies Arthritis (Septic arthritis, Reactive arthritis, Rheumatoid arthritis, Psoriatic arthritis, Felty's syndrome, Juvenile idiopathic arthritis, Still's disease) - crystal (Gout, Chondrocalcinosis) - Osteoarthritis (Heberden's node, Bouchard's nodes) acquired deformities of fingers and toes (Boutonniere deformity, Bunion, Hallux rigidus, Hallux varus, Hammer toe, Swan neck deformity) - other acquired deformities of limbs (Valgus deformity, Varus deformity, Wrist drop, Foot drop, Flat feet, Club foot, Unequal leg length, Winged scapula) patella (Luxating patella, Chondromalacia patellae) Protrusio acetabuli - Hemarthrosis - Arthralgia - Osteophyte Systemic CT disorders vasculitis: Churg-Strauss syndrome - Kawasaki disease - Hypersensitivity vasculitis - Wegener's granulomatosis - Arteritis (Polyarteritis nodosa, Takayasu's arteritis, Temporal arteritis) immune/autoimmune: Goodpasture's syndrome - Microscopic polyangiitis - Systemic lupus erythematosus (Drug-induced) - Dermatomyositis (Juvenile dermatomyositis) - Polymyositis - Scleroderma - Sjögren's syndrome - Behçet's disease - Polymyalgia rheumatica - Eosinophilic fasciitis Hypermobility Dorsopathies spinal curvature (Kyphosis, Lordosis, Scoliosis) - Scheuermann's disease - Spondylolysis - Torticollis - Spondylolisthesis Spondylopathies (Ankylosing spondylitis, Spondylosis, Spinal stenosis) - Schmorl's nodes - Degenerative disc disease - Coccydynia - Back pain (Radiculopathy, Neck pain, Sciatica, Low back pain) Soft tissue disorders muscle: Myositis (Pyomyositis) - Myositis ossificans (Fibrodysplasia ossificans progressiva) synovium and tendon: Synovitis/Tenosynovitis (Calcific tendinitis, Stenosing tenosynovitis, Trigger finger, DeQuervain's syndrome) - Irritable hip - Ganglion cyst bursa: bursitis (Olecranon, Prepatellar, Trochanteric) - Baker's cyst fibroblastic disorders: Dupuytren's contracture - Fasciitis (Plantar fasciitis, Nodular fasciitis, Necrotizing fasciitis) - Fibromatosis shoulder lesions: Adhesive capsulitis - Rotator cuff tear - Subacromial bursitis enthesis: enthesopathies (Iliotibial band syndrome, Achilles tendinitis, Patellar tendinitis, Golfer's elbow, Tennis elbow, Metatarsalgia, Bone spur, Tendinitis) other, NEC: Muscle weakness - Rheumatism - Myalgia - Neuralgia - Neuritis - Panniculitis - Fibromyalgia Osteopathies disorders of bone density and structure: Osteoporosis - Osteomalacia - continuity of bone (Pseudarthrosis, Stress fracture) - Monostotic fibrous dysplasia - Skeletal fluorosis - Aneurysmal bone cyst - Hyperostosis - Osteosclerosis Osteomyelitis - Avascular necrosis - Paget's disease of bone - Algoneurodystrophy - Osteolysis - Infantile cortical hyperostosis Chondropathies Juvenile osteochondrosis (Legg-Calvé-Perthes syndrome, Osgood-Schlatter disease, Köhler disease, Sever's disease) - Osteochondritis - Tietze's syndrome - Relapsing polychondritis See also congenital conditions (Q65-Q79, 754-756)