Rheumatol Int (2008) 29:211–216 DOI 10.1007/s00296-008-0649-x
C A S E RE P O RT
A case of catastrophic antiphospholipid syndrome presenting with acute respiratory distress syndrome as the initial manifestation Kazushi Kinjo · Hiroya Terabe · Megumi Ogawa · Hideki Takeda · Doki Cho · Shinichi Hoshino · Takeshi Miyahira · Mariko Oshiro
Received: 1 March 2008 / Accepted: 9 July 2008 / Published online: 24 July 2008 © Springer-Verlag 2008
Abstract Antiphospholipid syndrome (APS) is a systemic autoimmune disorder characterized by a combination of arterial or venous thrombosis and recurrent fetal loss, accompanied by elevated titers of antiphospholipid antibodies (aPL). Catastrophic antiphospholipid syndrome (CAPS) is a small subset of APS characterized by widespread systemic thrombotic disease with multiorgan failure. We herein describe an autopsy case of CAPS who developed severe respiratory failure due to acute respiratory distress syndrome (ARDS) as the initial manifestation. Patients with APS may exhibit a broad spectrum of pulmonary diseases. ARDS is the common pulmonary complication in CAPS, although it rarely occurs in APS. Some mechanisms of ARDS in CAPS have been postulated but the precise mechanism is still not clearly understood. It is important to understand that APS or CAPS could be a cause of ARDS since ARDS might develop as the initial manifestation of APS or CAPS as seen in our case. Our case is interesting in that severe respiratory failure due to ARDS was the initial presentation of CAPS.
Keywords Acute respiratory distress syndrome · Catastrophic antiphospholipid syndrome · Multiorgan failure · Pulmonary complication
Introduction Antiphospholipid syndrome (APS) is characterized by vascular thrombosis (arterial or venous) in association with antibodies to certain plasma proteins that are often bound to anionic phospholipids. These antibodies are known as antiphospholipid antibodies (aPL) [1]. Catastrophic antiphospholipid syndrome (CAPS) is a small subset of APS characterized by widespread systemic thrombotic disease with multiorgan failure. ARDS is one of the pulmonary complications of CAPS [2, 3]. We herein describe an autopsy case of CAPS who developed severe respiratory failure due to ARDS as the initial manifestation, followed by full-blown CAPS which was characterized by widespread thrombotic microangiopathy.
Case report This work was performed at Okinawa Prefectural Hokubu Hospital. K. Kinjo (&) · H. Terabe · M. Ogawa · H. Takeda · D. Cho · S. Hoshino · T. Miyahira Department of Internal Medicine, Okinawa Prefectural Hokubu Hospital, 2-12-3 Onaka, Nago, Okinawa 905-8512, Japan e-mail:
[email protected] M. Oshiro Department of Pathology, Okinawa Prefectural Hokubu Hospital, 2-12-3 Onaka, Nago, Okinawa 905-8512, Japan
A 48-year-old female, who had a previous history of two episodes of spontaneous abortion, developed pain in her shoulders and swelling of her hands, followed by jaw pain since August 2005. In early September 2005, she suVered from morning stiVness in her hands, pain in her knees, and leg edema. In addition, mild dyspnea appeared. As a result, she visited a private clinic. Rheumatoid arthritis was diagnosed and the patient was treated with oral predonisolone 10 mg daily. However, the patient’s respiratory symptoms thereafter deteriorated and they were accompanied by fever and a worsening of the leg edema, and Wnally she
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was brought to our emergency room in late September 2005. She did not have either dry eyes or dry mouth at presentation. Her vital signs were as follows: blood pressure, 140/80 mmHg; pulse rate, 124/min; respiratory rate, 50/min; body temperature, 38.9°C. She was very dyspneic and slightly agitated. On physical examination, the pulmonic component of the second heart sound demonstrated an increased intensity. In both lower lung Welds, late inspiratory crackles were heard and in the left lower lung Weld a pleural friction rub was noted. Engorged jugular veins were observed. There was mild pitting edema in both legs. The patient’s Wngers were swollen, thus showing a sausage-like appearance. The laboratory data were as follows: white blood cell 15,800/l; hemoglobin 11.4 g/dl; platelet 28.0 £ 104/l; prothrombin time 13.5 s (normal 9.5–11.5 s); activated partial thromboplastin time, 33.4 s (normal 29.5–31.5 s); BUN 22 mg/dl (normal 8–20 mg/dl); Cr 0.7 mg/dl (normal 0.6– 1.3 mg/dl); Na 140 mEq/l; K 5.0 mEq/l; Cl 103 mEq/l; AST 187 IU/l (normal 8–38 IU/l); ALT 135 IU/l (normal 4–44 IU/l); LDH 948 IU/l (normal 106–220 IU/l); ALP 290 IU/l (normal 104–338 IU/l); CPK 3,732 IU/l (normal 43–165 IU/l); T-Bil 0.6 mg/dl (normal 0.2–1.2); Glucose 100 mg/dl (normal 65–105 mg/dl);CRP 11.96 mg/dl (normal 0–0.18 mg/dl). Urinalysis: protein +1, red blood cell 5–9/HPF, white blood cell 5–9/HPF. The urine pregnancy test was positive. The analysis of arterial blood gas demonstrated a PaO2 of 43.7 Torr and a PaCO2 of 28.3 Torr, while providing O2 at 15 l/min with a facial mask with a reservoir. The chest radiograph revealed diVuse inWltrates in the bilateral lung Welds with mild cardiomegaly. The chest CT scan showed air space consolidations predominantly in the bilateral lung bases and ground glass opacity (GGO) in the both lung Welds. Mild to moderate pleural eVusion was observed in both lungs (Fig. 1). These imaging studies suggested the presence of either ARDS, cardiogenic pulmonary edema, or interstitial pneumonitis. The electrocardiogram showed right axis deviation and a delayed precordial transition zone, thus suggesting right heart pressure overload. Echocardiography showed normal left ventricular contractility with an ejection fraction of 65%. A mild enlargement of the right atrium and the right ventricle were observed and the interventricular septum showed paradoxical bulging of the septum into the left ventricle during systole, suggesting pulmonary hypertension. Mild pericardial eVusion, mild tricuspid regurgitation, and mitral regurgitation were noted. The initial problems in this patient were acute respiratory failure with diVuse bilateral pulmonary inWltrates, pulmonary hypertension, arthritis, positive pregnancy test and
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Fig. 1 Chest CT scan showing air space consolidations predominantly in the bilateral lung bases and ground glass opacity in both the lung Welds
elevated CPK and liver enzymes. A pregnancy test was positive but no gestational sac was identiWed, thus suggesting either that the pregnancy was too early for detection or that a spontaneous abortion had already occurred. These Wndings prompted us to evaluate the patient for collagen vascular diseases and treat her with steroids. Initially, based upon the patient’s history which seemed to indicate some type of collagen vascular disease, the lung lesion in this patient was thought to be interstitial pneumonitis rather than ARDS or heart failure. Accordingly, steroid pulse therapy was performed with methylpredonisolone 1 g daily for 3 days and followed by oral predonisolone 40 mg daily. The patient was intubated on the second hospital day, but after a 3-day course of pulse therapy a dramatic improvement was observed in the patient’s symptoms and radiographic Wndings. However, on the 8th hospital day the patient developed dyspnea and hypoxemia again. Bronchoscopy was done, thus revealing diVuse alveolar hemorrhaging. Bronchoalveolar lavage (BAL) Xuid showed a cell count of 40 £ 105/ml with neutrophil predominance (neutrophils 82%, lymphocytes 13%, eosinophils 2%, and histiocytes 3%). A culture of the BAL Xuid for bacterial organisms was negative. Although BAL could not conWrm the etiology of the lung lesions, the Wndings of the BAL Xuid were not inconsistent with ARDS. At this time conditions other than the pulmonary manifestations were found in the patient, such as microangiopathic hemolytic anemia and acute renal failure. Microangiopathic hemolytic anemia was evidenced by a decreased hemoglobin level of 6.8 g/dl, an increased LDH level of 2,980 IU/l (normal 106–220 IU/l) and outstanding schistocytes on the peripheral blood smear. Thrombocytopenia with a platelet count of 2.1 £ 104/l (normal 12–38 £ 104/l) and increased Wbrinolytic activity which was demonstrated by an increased FDP level of 155.4 g/ml (normal 30.0 g/ml (normal
