CTDs and lung disease Flashcards
what 4 things are important to consider with ILD and CTDs
- is ILD part of the underlying disease or unrelated
- what investigations are needed
- is it linked to the treatment
- how is it going to affect the prognosis
what is UIP
Usual interstitial pneumonia (UIP) - a form of lung disease characterized by progressive scarring of both lungs ( fibrosis of interstitium)
characteristics of IPF/UIP pattern disease (4)
> 50yro affects M>F; dry cough refactory to treatment; gradual deterioration; median survival 2-.5-3.5yrs
characteristics of NSIP pattern of disease (4)
median age of onset is 40-50yr; presents w SOB, cough and fatigue; cellular and fibrotic subtypes; prognosis better than IPF
characteristics of COP pattern of disease (4)
mean age is 55yr; x2 as common in smokers; productive cough; good response to oral steroids (treatment needed for >6 months)
characteristics of AIP pattern of disease (3)
meam age is 50yro; rapid onset of symptoms with dramatic detioration; hypoxemia and resp failure occur/ARDS
what is another name for AIP
Hamman Rich syndrome
characteristics of LIP (lymphatic interstitial pneumonia) pattern of disease
poorly defined clinical characteristics; mean age 50yro; cough and SOB; strongly associated with Sjogren’s; variable response to steroids
6 CTD conditions that are associated with ILD
RA (majority); sytemic sclerosis; SLE; dermatomyositis; Sjogren’s; mixed connective tissue disease
what RA pts are more likley to get ILDs (2)
men; those with a high-titer RhF
what patterns are seen in RA-ILD (2)
UIP; NSIP
is ILD prognosis better or worse if CTD associated
better
what is a strong predictor for poor prognosis
progressive SOB
what are 3 good measures for ILD progression
shrinking lung volumes on CXR; extent of fibrosis on HRCT; serial FVC on lung funciton
when can ILD occur during the course of CTDs (timeline)
can occur up to 5 years prior to typical CTD symptoms (joint disease etc.) - must check for CTD if pt presents with ILD (esp RA and dermatomyositis)
which subtype of systemic sclerosis is more strongly associated with ILD
dcSSc (diffuse)
what does ILD commonly coexist with in systemic sclerosis
pulmonary hypertension
what disease pattern is associated with SSc-ILD
NSIP; UIP
what antibody is strongly associated with SSc-ILD development
Scl-70
what is the management for SSc-ILD
Mgx directed at detecting ILD early; various immunosuppressive agents used (steroids, colchicine, D-pencillamine, chlorambucil) but the most effective is CYCLOPHOPHAMIDE (infusion or oral)
in someone with lung involvement and SLE what must be rules out first
infection - immunosuppression leads to increased risk of infection and chronic ILD is not very commonly seen
what lung involvement is most seen in SLE (and its histology)
acute lupus pnuemonitis with alveolar hemorrhage;
histology - features of interstitial pneumonitis
management of lupus pnuemonitis
heavy immunosuppression and plasmapherisis (1-2mg/kg corticosteroids per day + azothioprine/cyclophophamide)
what does dermatomyositis-ILD present as (3)
rapidly progressing AIP; slowly progressing UIP; COP
