SESSION TITLE: Interstitial Lung Disease Student/Resident Case Report Posters II
SESSION TYPE: Medical Student/Resident Case Report
PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM
INTRODUCTION: The association of NSIP with connective tissue disorders, HIV, and drug exposure is known. We hereby present the first reported successful treatment of NSIP in a patient with psoriasis.
CASE PRESENTATION: A 43-year old, non-smoking, African-American woman with past medical history of hypertension and anemia, presented with worsening exertional dyspnea. Patient denied any exposure to exotic pets, molds, dusts, chemicals, drugs, or TB. Three months previously, she developed erythematous scaling plaques over the extensor surfaces of her elbows and knees, associated with joint stiffness and mild swelling of the eyelids, hands, wrists, and ankles but no nail changes. Punch biopsy from right antecubital fossa revealed focal parakeratosis admixed with neutrophils over a moderate epidermal hyperplasia with edema of papillary dermis consistent with psoriasis. Meanwhile, CT chest showed bilateral interstitial opacities and traction bronchiolectasis with no honeycombing and lymphadenopathy. After a nondiagnostic bronchoscopy, surgical lung biopsy revealed diffuse fibro-inflammatory changes consistent with nonspecific interstitial pneumonitis (NSIP). HIV and tuberculin skin tests were negative. Initial PFT showed FEV1 of 59%, FVC of 60%, FEV1/FVC of 86%, TLC of 45% and a corrected diffusion capacity of 51%, consistent with moderately severe restrictive lung disease. Autoimmune panel showed weakly positive ANA, RF, and SS-A antibodies. She was treated with topical steroids followed by narrow band ultraviolet light with improvement of plaques. Unresponsive to oral prednisone for NSIP, she was started on cyclophosphamide, which caused hemorrhagic cystitis. Azathioprine was substituted and was associated with remarkable improvement of symptoms and resolution of lung function, oxygenation, and radiographic abnormalities.
DISCUSSION: NSIP is subtype of idiopathic interstitial pneumonia that has been associated with autoimmune diseases, HIV infection, and drugs like amiodarone, methotrexate and nitrofurantoin. Psoriasis is an autoimmune, chronic inflammatory skin disorder which is often associated with arthritis and renal diseases, but rarely interstitial pneumonia. Pathogenesis is still unexplained but psoriasis and NSIP-related activation of T helper cells producing inflammatory cytokines has been postulated. Kawakami et al reported the only case of NSIP associated with psoriasis and polymyalgia rheumatica, which was unresponsive to corticosteroid therapy. Our patient developed hemorrhagic cystitis with cyclophosphamide but showed remarkable clinical, physiological, and radiographic improvement with azathioprine.
CONCLUSIONS: Azathioprine was associated with clinical, physiological, and radiographic resolution of a rare occurrence of NSIP in a patient with psoriasis.
Reference #1: Kawakami K, et al. A case of non-specific interstitial pneumonia associated with psoriasis vulgaris and polymyalgia rheumatica. Nihon Kyobu Shikkan Gakkai Zasshi 1997; 35:1395-9.
DISCLOSURE: The following authors have nothing to disclose: Raghav Gupta, Joseph Espiritu
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