patients with connective tissue diseases usually develop pulmonary complications which typically presented with dyspnea , chest pain or fever.

1. Pleuro pulmonary manifestations in
connective tissue diseases
By
Dr. Mohamed Abdellatif

2. Patients with connective tissue diseases often develop pulmonary
complications.
Patients typically presents with symptoms of: dyspnea, cough, fever or chest
pain .
They may often already be on immunosuppressive drugs.
Connective tissue diseases such as: (Rheumatoid arthritis, Systemic lupus
erythromatosus, Polymyositis and Dermatomyositis, Systemic sclerosis,
Sjogren’s syndrome, Ankylosing spondylitis, Behcet’s syndrome).
Pleuro pulmonary manifestations in connective
tissue diseases

3. Rheumatoid Arthritis (RA)
It is chronic, systemic inflammatory disease
characterized by articular and extra articular
manifestations.
Most probably autoimmune
Male: female ratio 1:3
Rheumatoid lung dse affect 2:55% of patients
with rheumatoid arthritis

4. 1. Interstitial pulmonary fibrosis
2. Pulmonary nodules
3. Organized pneumonia
4. Bronchiectasis
5. Obliterative bronchiolitis.
Pulmonary manifestations of RA

5. 1. Pleuritis
2. Pleural effusion
3. Pleural fibrosis
4. pneumothorax
Pleural manifestations of RA:

6. Vasculature manifestation of RA
Cricoarytenoid arthritis

7. Pulmonary manifestations
of RA

8.  Interstitial pulmonary fibrosis
Minor pulmonary fibrosis found up to 60% of patients in lung biopsy studies
but CXR changes only seen in 1:5%
Presents with progressive dyspnea
Examination: clubbing and bilateral basal crepitation’s
PFTs: restrictive pattern
Radiologically:
UIP: most common pattern, show: sub pleural basal reticular abnormalities
with honeycombing and ground glass opacities.
NSIP: show bilateral basal ground glass opacities, absence of
honeycombing.
Treatment: steroid and immunosuppressive agents.

10.  Pulomary nodules:
Occur in < 5% of patients with RA.
Usually found incidentally on CXR
Only occur in seropositive disease and patients may have other nodules
elsewhere e.g. elbow, fingers
Characterized by:
Single or multiple
Non calcified, thick wall with smooth out lines
Asymptomatic unless they cavitate or rupture causing infection. pleural
effusion, or fistula.
Caplan’s syndrome: RA, single or multiple chest nodules and coal worker’s
pneumoconiosis

12.  Organizing pneumonia:
Presents with fever, cough, weight loss and multifocal consolidation which
do not respond to antibiotics.
Can be disease or drug induced “gold” or have no obious cause i.e.
cryptogenic
Confirmed by trans bronchial or open lung biopsy showing: acini filled with
loose connective tissue and variable inflammatory infiltrate
Often dramatic response to steroids, may need long term
immunosuppressive agents.

14.  Bronchiectasis:
Affects about 30% of RA patients
Irreversible dilatation of bronchial tree mostly in lower lobes
Presented with chronic cough with expectoration of thick sputum

15.  Obliterative bronchiolitis:
Is a progressive bronchiolar inflammation with
submucosal fibrosis associated with luminal stenosis
and occlusion
Presented with acute rapid progressive dyspnea,
cough, basal crepitation
PFT: irreversible obstructive pattern
CXR: hyperinflation
HRCT: mosaic attenuation pattern, bronchial wall
thickening, ground glass opacities
Treatment: oral steroids and immunosuppressive
agents show little response, some improvement in
few cases with anti TNF therapy, consider lung
transplantation.

16. Pleural manifestations of
RA

17. Pleuritis:
Frequent, occurring in > 30% of patients with RA
Usually presented with mild pleuritic chest pain
Pleural effusion:
The most common pleural disease with RA.
Usually asymptomatic
Fluid is typically exudative with: (high protein content >4 g /d, low PH <7.3,
elevated LDH, low glucose < 30mgdl, increased WBCs: lymphocytic mainly or
neutrophilic and RF is often present in pleural fluid and higher than serum level)
Need to exclude other causes of effusion such as empyema or malignancy
Pleural fibrosis:
Long standing pleural effusion leading to formation of a fibrous bands causing
pleural fibrosis
Pneumothorax:
Can occur secondary to rupture of rheumatoid pulmonary nodule or end stage
fibrotic lung disease

18. Vasculature manifestation
of RA
Pulmonary HTN, pulmonary vasculitis and venous
thromboembolism are the most common
manifestation related to RA.

19. Cricoarytenoid arthritis
seen in studies in up to 75% of patients with RA by
fiber optic laryngoscopy and HRCT
commoner in women
rarely symptomatic
can cause sore throat, hoarse of voice, upper airway
obstruction, stridor or OSA
may need tracheostomy and steroids orally and joint
injection

20. SLE is multi-organ autoimmune disease in which the immune system
attacks its own tissues causing widespread inflammation and tissue
damage in the affected organ
It is a systemic connective tissue disease characterized by articular and
extra articular manifestations, affects the joint in 90% of the patients, lung
50:70%, skin in 80%, kidney in 60%
Pulmonary disease (lung, vasculature, diaphragm, pleura) often seen and
may be a presenting feature of the disease
Female: male ratio 9:1
systemic lupus erythematosus “SLE”

21. Acute lupus pneumonitis:
Presents with fever, cough, dyspnea, pleuritic pain and hypoxia
CXR shows infiltrates which may be widespread
HRCT: poorly defined areas of ground glass opacities or consolidation
peripherally at lung bases
Treatment: steroids and cytotoxic drugs, may have good response,
can progress to chronic interstitial pneumonitis
Chronic interstitial pneumonitis:
Usually asymptomatic
Radiologically: interlobular septal thickening, pulmonary fibrosis,
atelectatic bands
Treatment: steroids and immunosuppressive or cytotoxic agents
Pulmonary manifestations of SLE

22. Alveolar hemorrhage:
Presented with dyspnea, hemoptysis, associated GN
May be life threatening
Radiologically: diffuse alveolar infiltrates
Treatment: steroids with high dose, cyclophosphamide, plasmapheresis
Shrinking lung syndrome (diaphragmatic dysfunction):
Presented with dyspnea with or without episodic pleurisy caused by reduced lung volumes and
poor respiratory reserve due to diaphragmatic muscle weakness
CXR: small lung volume
PFT: restrictive pattern
Treatment: steroids, inhaled B2agonist, theophylline
Vasculature manifestation:
Pulmonary HTN; due to pulmonary vasoconstriction
Pulmonary embolism; common, due to circulating anticardiolipin antibodies
Bronchiolitis obliterans: rare with SLE

23. Pleuritis:
Pleuritic pain with or without pleural effusion
May be also due to musculoskeletal cause
Pleural effusion:
Found in 50% of patients with SLE
Usually bilateral and exudative with neutrophilia or lymphocytosis if
the effusion is chronic
May be hemorrhagic
Atelectasis:
Associated with pleurisy or pleural effusion
Pleural manifestations of SLE

24. These are 2 separate idiopathic inflammatory
myopathies:
PM: causes symmetrical proximal muscle weakness
DM: which has characteristic rash
Pulmonary complications are common and frequent
cause of death occurring in both condition.
Polymyositis “PM” and Dermatomyositis “DM”

25. Interstitial lung disease “ILD”:
In 20:30% of patients
Commoner when CK level normal and /or antisynthetase +ve
Less common when DM is associated with malignancy
Presents with: dyspnea, cough, arthralgia, fever and fine basal
crackles
HRCT: patchy consolidation, peripheral reticular pattern
Treatment: steroids and cyclophosphamide
Pulmonary manifestations of PM and DM

26. Chronic organizing pneumonia:
Poor prognosis if associated with features of fibrosis
Pulmonary HTN
Aspiration pneumonia:
In 20 % of the patients
Associated with marked increase in mortality rate
Caused by dysphagia and pharyngeal muscle weakness
Spontaneous pneumo mediastinum:
Rare
Presented with acute retro sternal pain, neck, and face subcutaneous
emphysema
Usually in association with ILD in DM > PM

27. This disease affects women > men 3:1, often presents in 5th decade
Has HLA association
It is largely a clinical diagnosis and there are several types:
 Limited cutaneous “CREST syndrome”:
60% of systemic sclerosis cases
 Diffuse cutaneous:
Abrupt onset disease with spread symmetrical itchy, painful
swellings of finger, arms, feet, leg and face
 Overlap syndrome or mixed connective tissue diseases
 Systemic sclerosis sine scleroderma:
Vascular or fibrotic visceral features without skin scleroderma
Pulmonary complications are the most common cause of death
Systemic sclerosis or scleroderma

29. Systemic sclerosis associated ILD:
The most common pulmonary manifestations of systemic sclerosis, affecting
about 40% of patients
Presented with: dyspnea, cough, fatigue
HRCT: ground glass changes, honeycombing, lung volume loss, interlobular
septal thickening
Treatment: low dose steroids and immunosuppressive agents
Lung transplantation is considered as an option
Pulmonary manifestations of systemic sclerosis

30. Systemic sclerosis related pulmonary arterial HTN:
The most common cause of death
May be due to vascular endothelial injury with subsequent apoptosis,
inflammation leading to arterial obliteration and narrowing
Aspiration pneumonia:
Uncommon, may be due to esophageal dysmotility
Chest wall limitation:
By skin scleroderma over chest (very rare)

31. Inflammation , lymphocytic infiltration and destruction of salivary and lacrimal
glands
Classical sicca syndrome includes dry eye, dry mouth with parotid or salivary
gland enlargement
Pulmonary involvement occurs in about 25% which includes;
Pleuritic chest pain
Pleural thickening, pleural effusion
Dry cough: due to atrophy of mucus gland in trachea and bronchi with higher
incidence of chest infection
Diffuse lung disease: may develop later, often asymptomatic but may presented
with cough, dyspnea and crackles
Sjogren’s syndrome

32. 1. It is chronic inflammatory disease causing spinal ankylosing with
sacroiliac joint involvement
2. 90% of patients are HLAB27+ve
Ankylosing spondylitis

33. Pulmonary fibrosis:
Occur in 5:15% of patients especially those with advanced disease
Typically, bilateral in upper lobes
May develop cysts or cavities and become colonized with Aspergillus
Pleuritis and pleural thickening
Restrictive defect:
Due to costovertebral rigidity
Pulmonary manifestations of ankylosing
spondylitis

34. It is systemic vessel vasculitis involving arteries and veins of all sizes
with recurrent painful oral and/ or genital ulceration, skin lesion,
arthritis and chronic relapsing uveitis which can cause blindness.
Pulmonary arterial aneurysms, arterial and venous thrombosis and
pulmonary infarcts in 5% .
Recurrent attacks of hemoptysis: which may be massive and fatal .
Pleural effusion, eosinophilic pneumonia: rare.
Behcet’s syndrome

35. THANKS!