Lung disease at RA 4.25/5 (4)

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A 57 year-old male with obliterative bronchiolitis secondary to RA; (a) and (b) HRCT axial sections demonstrate market mosaic pattern attenuation.

Rheumatoid arthritis (RA) with pulmonary seals (Interstitial lung disease): Obliterative Broncholitis (BO)t. Reference: Sidhu HS, 2011. CC BY NC SA 3.0

RA associated lung disease, rheumatoid lung disease

Definition

Lung manifestations are common in rheumatoid arthritis (RA, arthritis) and can occur in the form of several different types. Interstitial lung disease (ILD), pulmonary inflammation (pleuritis), rubbing nodules in the lungs and drug damage is elaborated on below. In general, the lung manifestation is unfortunately associated with increased morbidity and mortality. However, early treatment can have a good effect. It is therefore important to pay attention to the symptoms.

  • Rheumatoid lung disease is related to rheumatoid arthritis (RA, arthritis)
  • Symptoms and findings by investigations vary and may in some cases lead to pulmonary failure and death
  • There are several different types
  • CCP antibody (ACPA) are present in almost every case
  • Also, some medications used for RA can damage the lungs
  • The investigation and follow-up of rheumatoid arthritis is best done when rheumatologists and lung physicians cooperate.

Occurrence

Overall, lung changes can be detected in 30-50% with RA, but few are serious (references: Gabbay E, 1997, Habib HM, 2011).

  • Men are attacked more often than women (as opposed to RA in general)
  • Smokers are most exposed
  • In most cases lung disease occurs after joint disease has been detected

Symptoms

Most people are thus without symptoms. About 5-10% have lung symptoms (references: Bongartz T, 2010; Olson AL, 2011: 

  • Cough without mucus (Dry cough)
  • Breathing difficulties with physical strain
  • symptoms from lungs occur before joint disease in 10-20% of cases (reference Hyldgaard C, 2017)

Diagnosis

  • Lung function tested (Lung function tests)
  • CT examination of the lungs
    • Regular radiographs are not sufficient
  • Other causes of lung disease are excluded

Interstitial lung disease (ILD)

Common lung disease at RA

Treatment of ILD in RA

Generally, classic, immunosuppressive anti-rheumatic drugs are used. These have good effect in contrast to what is the case Idiopathic Pulmonary Fibrosis (IPF) who are not expected to respond to immunosuppressive drugs

Prognosis at ILD and RA

  • ILD is expected to improve and stop during treatment, but after cardiovascular disease it is the second most common cause of death in RA

Pleura manifestation at RA

Pulmonary (Pleura) is affected in most people with RA (up to 70% in autopsy studies), but only 3-5% have symptoms (Pleurisy) (references: Balbir-Gurman A, XNUM,  Corcoran JP, 2014,)

Treatment of pleuritis at RA

  • Intensify the anti-rheumatic treatment
  • Tapping of pleural fluid

Other pulmonary manifestations

Asthmatic symptoms of bronchial hyperreactivity

Bronchiolitis

Bronchiectasis

  • May be caused by chronic infection
    • Increased infection tendencies at RA

Follicular bronchiolitis

  • HRCT can display centrolobular preri-bronchial nodules that are less than 3 mm and have branches
  • Not "honeycombing"

Obliterative Broncholitis (BO)

  • Acute start
  • Quickly progressive dyspnea
  • Reap
  • Serious prognosis
  • Most often among women
  • Positive CCP (ACPA)
  • Many years of illness
  • Sulfasalazine (Salazopyrin) may be trigger
  • Detection by HRCT (not specific)
  • Lung function tests (pulmonary disease)
    • Obstruction
    • Normal DLCO
  • Treatment of BO
    • Remove triggering cause (if possible)
  • The prognosis is serious

Revma nodules in the lungs

  • Most often with long-term RA
  • CCP (ACPA) antibody
  • At the same time, rheumatoid nodules under the skin
  • Typical location
    • Interlobulic septa (lung patch slits)
    • Subpleural areas (along the lungs)
  • Size from few mm to several cm
  • In biopsy, vasculitis with central fibrinoid necrosis and palisade-forming mononuclear cells is detected
  • Usually without symptoms, but in some cases:
    • May burst and cause pleural fluid, broncho-pleural fistula and infection
    • Increased incidence of rheumatoid nodules during methotrexate treatment
  • Must be separated from cancer and metastasis
    • Biopsy
    • PET / CT usually shows less 18FDG (glucose) uptake than in cancer and metastasis

Caplan syndrome (rheumatoid pneumoconiosis)

  • May be present at occupational load with coal, rock dust or asbestos

Pulmonary hypertension

  • Mostly secondary to pulmonary disease (as opposed to systemic sclerosis)

Increased incidence of venous thromboembolism at RA

methotrexate (Methotrexate-lung)

  • In 0,86-6,9% of treated patients (reference: Roubille C, 2014)
  • Most often at relatively high doses of methotrexate
  • Most commonly within the first year of treatment
  • Dyspnea (heavy breathing)
  • Dry cough
  • X-rays can be normal at the beginning
  • HRCT
    • Diffuse opacities or
    • Patched changes
  • Bronchial lavage (bronchial rinsing, BAL), by lung physician
    • Exclude other cause (including infection)
  • Histology
    • Non-necrotizing granulomas
    • Scattered eosinophilic leukocytes
  • Treatment of MTX lung

    • Stop methotrexate
      • Most likely clinical effect within a few days
      • Radiological changes may last for several weeks
      • Prednisolone can be used if slow decline

Leflunoid (Arava) lung

  • May trigger ILD in exposed patients
  • Most often among Asians

TNF inhibitors and pulmonary manifestation

  • TNF inhibitors are suspected of triggering ILD, but debated. The drugs are also used in the treatment of lungs
  • Sarcoidosis-like changes in lung tissue
  • Organized pneumonia
  • Exacerbation of existing lung disease
  • Unknown disease mechanism
    • May be conditional on the basic disease
    • TNF inhibitors increase the infection's incidence
    • TNF-inhibitor induced ILD has been reported in some patients

Literature


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