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Familial interstitial lung disease in a family with familial juvenile idiopathic arthritis.


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Publication Information

Publication Type
Publication Sub Type
Journal Article, Original
Philippine Heart Center Journal
Publication Date
January-December 2014


Background. Interstitial lung disease (ILD) includes a large, heterogeneous group of mostly rare pulmonary conditions that cause derangements of the alveolar walls and loss of functional alveolar capillaries. It is a complex disease. FamiliallLD is a rare type of hereditary disease and a thorough evaluation of the root cause is needed. It becomes explicitly rare and multifaceted when the main cause is another familial type of disease, familial juvenile idiopathic arthritis.

Case. We present a case of an 8 year old girl who presented with difficulty of breathing, swelling of fingers, ankles and knees and respiratory distress when she was 2 years old. She was diagnosed initially to have miliary tuberculosis. Workups done included Chest CT scan and open lung biopsy. RF factor was extremely high, and anti-dsDNA was equivocal. Her eldest sister died of respiratory distress when she was 1 year and 4 months old. Her third sibling was diagnosed with systemic lupus erythematosus (SLE), juvenile idiopathic arthritis (JIA) at 3 years old. There is a familial history of ILD among the sisters, with one unaffected younger sister. She is presently maintained on corticosteroids, and sildenafil for the pulmonary hypertension.

Conclusion. The diagnosis of interstitial lung disease is challenging. In this report, we describe a rare type familial ILD in Filipino children. Searching for the known etiology of such disease requires a thorough history and a complete physical examination. Since cases are under-reported and often misdiagnosed, vigilance is imperative to decrease morbidity and mortality among these children.


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Philippine Heart Center Medical Library Fulltext Print Format

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