Categorizing interstitial pneumonia can be a challenge for physicians. We present a 52 year old female with scleroderma and associated lung disease who manifested recurrent acute illness resembling acute interstitial pneumonitis.
A 52 year old female with a two year history of scleroderma including esophageal dysmotility, morphea, Raynaud’s, telangiectasias, sclerodactyly, and pulmonary fibrosis without pulmonary hypertension and with normal LV function presented with repeated hospitalizations over a period of a year with acute respiratory distress. At the initial hospitalization, the patient presented with increasing shortness of breath and dyspnea on exertion. Vital signs, including oxygen saturation, were normal. A chest CT revealed ongoing lower lobe pulmonary fibrosis. The patient, who was on prednisone 60mg, was treated with intravenous steroids and quickly improved. One month later, while on low dose steroids, the patient developed acute respiratory distress. Chest radiograph revealed “diffuse bilateral opacities”. Her respiratory rate was 30, heart rate was 147, BP was 140/70. The patient was afebrile with a normal WBC count. An ABG revealed 7.17/36/43/13/64%. The patient was intubated and transferred to the ICU. IV steroids and broad-spectrum antibiotics were started. On the same day, a bronchoscopy with BAL was negative for legionella, AFB, fungal, PCP, CMV, adenovirus, VZV, and HSV. Blood cultures were negative. The patient quickly improved and was extubated. Again a month later, the patient developed hypoxic respiratory failure requiring intubation. A chest radiograph revealed “extensive air space disease”, a chest CT with contrast showed, “extensive interstitial lung disease at both bases” but also revealed new “ground-glass opacities in the upper lobes”. Again a BAL found no infectious etiology. IV steroids and broad-spectrum antibiotics were restarted. An open-lung biopsy was obtained. Right middle lobe tissue revealed “end-stage lung with extensive fibrosis and honeycombing …also mild focal chronic interstitial inflammation”. Special stains were negative for organisms. The patient improved, and was eventually discharged from the hospital. Finally seven months later, the patient again presented with hypoxic respiratory failure requiring intubation. Chest radiograph revealed “diffuse airspace consolidation”. The patient was afebrile with a normal WBC. IV steroids and broad-spectrum antibiotics were given. A BAL was negative for infection. The patient’s oxygenation and chest radiograph rapidly improved. The patient was soon extubated. 6 days later the patient’s shortness of breath increased. Unlike previously, the patient began to have fevers and increased WBC. A repeat BAL revealed multiple organisms. A chest CT again revealed the previously new upper lobe interstitial changes, but also showed air bronchograms in the lower lobes. The patient continued to deteriorate, became progressively hypoxic, and later expired. The autopsy revealed “extensive interstitial fibrosis, diffuse alveolar damage, multifocal areas of organizing pneumonia” as the cause of death.
Over 70% of patients with scleroderma develop pulmonary disease. There are two usual manifestations: pulmonary fibrosis and pulmonary hypertension. The pulmonary fibrosis is slowly progressive, lower lung zone predominant, and similar in character to UIP. This patient is unique in that she had two separate but coexisting lung processes: scleroderma related disease, and a fulminant diffuse process. This process clinically presented as idiopathic recurrent ARDS without systemic involvement, and pathologically showed diffuse alveolar damage. Although, rarely RA and polymyositis have had a Hamman-Rich type syndrome associated with them, scleroderma has not. We, therefore, propose this as a rare case of scleroderma associated with an AIP-type syndrome.
With reference to the ATS consensus statement, this case exemplifies an “area of uncertainty” surrounding the interstitial pneumonias. In this statement, a connective tissue disease such as scleroderma excludes a diagnosis of AIP. Uncertainty presents itself in such issues of overlap challenging proper diagnosis, perhaps affecting treatment, and leaving questions as yet unanswered.
M.Y. Kline, None.