Tuesday, February 07, 2006

42 year old with cystic lesions

42 year old woman with lupus diagnosed in 2003, with rash on the arms that left hyperpigmented scars, alopecia, joint pain with stiffness, hands going numb with cold, weight loss from nausea, and photosensitivity. Skin biopsy positive for both discoid and tumid lupus and she has a positive ANA 1:320 speckled pattern; and lymphopenia, mild, 1400. Her criteria are photosensitivity, joint pain, and stiffness, positive ANA,
lymphopenia, and some alopecia. She also has leukopenia.

She has no respiratory symnptoms. A routine CXR followed by a high res CT are shown below.

A bronchoscopy was done. Tbbx of the upper lobe revealed (path report):

very mild chronic inflammation and intra-alveolar hemosiderin laden macrophages. These findings are non-specific. Also present is a fragment of epithelium and scant fibrous stroma with a papillary configuration.

Any thoughts?

5 comments - CLICK HERE to read & add your own!:

Jeff H said...

So, it looks like these cystic lesions are present in both the upper and lower lobes bilaterally. Setting aside the Lupus for a moment, these thin wall cysts raise suspicion for Langerhan's cell histiocytosis/eosinophilic granulama, LAM, or an atypical appearence of sarcoid. A did a quick search and did not find any known association between these diseases and Lupus (and the diagnosis of Lupus here seems pretty well established).

I did find one report of Lymphocytic interstitial pneumonitis (LIP) and diffuse think wall cysts in a patient with SLE.

Finally, is this patient being treated with immunosupression for the Lupus. We need to consider atypical infections, and in light of the multiple diffuse cysts, I would have to consider PCP, although I would have expected this to be found on a BAL.

Bottom line: in the absence of a definitive diagnosis here, I would probably proceed with a surgical biopsy.

Jennings said...

good comments; BAL: micro all negative (including PCP). The cell count was 117 WBC's with 97% macrophages. We sent off a CD1a but didn't see it in the computer. Iguess the biopsy is too small to rule out an LIP, as you suggest. I agree with open lung as next step now that infection is pretty much ruled out.

I did a search and seem to remember lupus can have cysts in lung but can't remember where I saw that so who knows.

As for LAM, the HMB-45 stain was negative.

Baleeiro said...

Lupus and Sjogren's can also present with bronchiectasis but i haven't found an association with cysts like those either. Is she a smoker?

Baleeiro said...

What do her PFTs look like?

Jennings said...

The patient quit smoking 5 years ago. She used to smoke 15 cigarettes a day for 12 years. Her PFT's are normal (120% predicted) but her DLCO is slightly decreased at 71% predicted.