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A CHALLENGING DIAGNOSIS OF CELLULAR NONSPECIFIC INTERSTITIAL PNEUMONIA (NSIP) WITH ORGANIZING PNEUMONIA IN A PATIENT WITH ANTI-JO 1 NEGATIVE DERMATOMYOSITIS
Chest ; 162(4):A1287, 2022.
Article in English | EMBASE | ID: covidwho-2060795
ABSTRACT
SESSION TITLE Autoimmune Diffuse Lung Disease Case Posters SESSION TYPE Case Report Posters PRESENTED ON 10/19/2022 1245 pm - 0145 pm

INTRODUCTION:

Nonspecific interstitial pneumonia (NSIP) is an interstitial lung disease (ILD) that can be idiopathic or associated with connective tissue disorders (CTDs). The two subtypes of NSIP are cellular interstitial pneumonitis (CIP) and fibrotic, with CIP being less common. Subtypes can be distinguished through pathology and imaging. NSIP constitutes 14-36% of cases of idiopathic interstitial pneumonias. ILD-associated DM has a higher mortality, making diagnosis crucial. In specific, fibrotic NSIP has a high 10-year mortality rate, making differentiation relevant. CASE PRESENTATION A 36-year-old female presented with fatigue and erythematous papular lesions on her face, palms, shoulder, and neck for one month. She also developed a fever and dry cough a week before. She denied recent travel or sick contacts. COVID-19 was negative. On exam, she was tachypneic and tachycardic with a maculopapular rash. A pulmonary exam revealed bilateral fine crackles. CXR showed dense left and mild right-sided patchy consolidations. Labs revealed elevated inflammatory markers (ESR-63, CRP-1.9, LDH-982). CPK was high - 517. CBC and procalcitonin were normal. CT showed extensive patchy and confluent areas of opacification of the left lower lobe, including a mass-like area measuring 3.3cm. Infectious workup was negative. Autoimmune testing ( Anti-Jo 1 Ab, ANA, etc) was negative. Bronchoscopic left lower lobe biopsy showed cellular interstitial inflammation composed of lymphocytes, plasma cells, rare eosinophils, and foci of intra-alveolar fibrinous exudates, suggestive of CIP and OP. She was treated successfully with corticosteroids and was discharged on prednisone. Repeat autoimmune antibody workup was negative. Skin biopsy showed a lichenoid lymphocytic infiltrate and necrotic keratinocytes consistent with dermatomyositis. Mycophenolate and rituximab were initiated;prednisone was tapered off. Follow-up chest CT showed cleared infiltrates with symptomatic improvement.

DISCUSSION:

CIP is an uncommon form of NSIP. On CT, bilateral ground-glass opacities are the most common feature. CIP is characterized histologically by interstitial thickening due to the presence of inflammatory cells and type-II pneumocyte hyperplasia with preserved lung architecture. Treatment is corticosteroids. The prognosis is excellent. ILD associated with DM is strongly associated with a positive Anti-Jo Ab, which was negative here making diagnosis challenging. She was diagnosed with dermatomyositis using histological findings from a skin biopsy. She responded to steroids at acute presentation and treatment was tailored once DM was diagnosed leading to complete recovery.

CONCLUSIONS:

ILD is not uncommon in CTD, however it is usually associated with a positive Anti-Jo 1 antibody. Our case is unique as the patient had negative Anti- Jo 1 Ab, however was found to have cellular NSIP with DM responding well to treatment following diagnosis. Reference #1 https//ard.bmj.com/content/63/3/297 Reference #2 https//www.ncbi.nlm.nih.gov/books/NBK518974/ Reference #3 https//pubmed.ncbi.nlm.nih.gov/33916864/ DISCLOSURES No relevant relationships by Nawal Ahmed No relevant relationships by TAIKCHAN LILDAR No relevant relationships by Namratha Shripad No relevant relationships by David Wisa
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Full text: Available Collection: Databases of international organizations Database: EMBASE Language: English Journal: Chest Year: 2022 Document Type: Article

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Full text: Available Collection: Databases of international organizations Database: EMBASE Language: English Journal: Chest Year: 2022 Document Type: Article