Fatal lupus flare presented as SIADH and hemorrhagic gastroenteritis: a case report

Full Text

Introduction

Systemic lupus erythematous (SLE) has many guises. The syndrome of inappropriate secretion of antidiuretic hormone (SIADH) has been reported rarely in SLE, though sometimes it attributed to other causes such as medications. Lupus enteritis is also a rare but potentially lethal manifestation of SLE as it may evolve to intestinal necrosis and perforation if untreated. We describe the case of a 34-year-old man who had not been diagnosed with SLE before, presented with severe hyponatremia and signs of enteritis, and developed hemorrhagic gastritis soon afterwards. 

Case presentation

A 34-old man was brought to our emergency department by his wife in October, 2019th because he had become disoriented. According to patient’s wife he had up to four loose stools a day for 3-4 days before admission.

He was diagnosed with epilepsy 6 months ago when he started to have generalized seizures. But he had not been having such episodes for the previous five months and had not been taking any anticonvulsants. He was in his usual health until two months earlier when he developed a daily fever up to 38.5°C, fatigue and myalgia in the upper back, shoulder and hip girdles. A month before admission he developed diarrhea and fever up to 39 °C, and was prescribed with antibiotics by primary care physician. Soon afterwards he developed a widespread rash all over the body which was considered an allergic reaction. The patient was treated in other hospital with glucocorticoids and antibiotics for a week. All his symptoms resolved completely after treatment and he did not seek any medical attention until the day of admission to our hospital.

On physical examination, the patient was agitated and disoriented with incoherent speech.   His lungs were clear on auscultation, heart rate was regular, and no murmurs were heard. Vital signs showed  blood pressure of 140/80 mmHg, heart rate of 112 per minute, respiration rate of 20 per minute, oxygen saturation of 98% while breathing on room air, and temperature of 37.3 °C. The abdomen was non-tender and non-distended with active bowel sounds. There was not hepatosplenomegaly or peripheral lymphadenopathy. On rectal examination there was brown stool without blood or mucus. 

The laboratory findings were remarkable for sodium level of 103 mmol/l, potassium level of 2.5 mmol/l, ALT level of 97 IU/L, AST level of 261 IU\L, alfa-amylase level of 117 IU/L, creatine kinase level of 682 IU/L, albumin level of 19 g/l, CRP level is 10 mg/l. BUN, creatinine, bilirubin level were normal. CBC with differential revealed mild normocytic anemia with Hb level of 11.6 g/dl, moderate thrombocytopenia with platelet count of 69, and severe lymphopenia with lymphocyte level of 0,18.

Urine sodium level was as high as 160 mEq/L, and diagnosis of SIADH was made.

CT scan of the head was unremarkable.

СT of the chest (Fog.1) revealed normal lungs, without infiltrates and nodules.

Fig.1. Chest CT. Normal lungs, the heart chambers had normal diameters.

The patient’s hyponatremia were corrected slowly over a few days, and on day three the patient became completely alert and oriented. He continued to have 3 to 4 loose stools a day but otherwise remained stable until day 4 when he was found to have melena, cough and dyspnea. On physical examination new skin changes resembling Janeway lesions were noticed on palms and soles along with bilateral erythema of hypothenars.  Vital signs showed BP of 120/80, HR of 130 per minute, RR of 24, oxygen saturation of 92 % on room air, and a temperature of 37.6 °C.

Blood tests revealed a profound drop in the hemoglobin level and platelet count, and increase in band form count from 18 to 62%. The level of CRP rose up to 134 mg/L.

Upper gastrointestinal endoscopy revealed diffuse bleeding from gastric mucosa.

CT scan of the chest revealed bilateral lower-lobe infiltrates and partial atelectasis, suggestive of pneumonia (Fig.2). Bronchoalveolar lavage fluid was sent for culture.

Fig.2. Second CT of the chest. Bilateral infiltrates and partial atelectasis in the both lower lobes. Left image – axial slice, right one – a frontal reformation.

Abdominal CT found loops of the jejunum with thickened enhancing walls (Fig.3,4), suggestive of enteritis

Fig.3 Abdominal CT, axial slices. Right image, CT without intravenous (IV) contrast enhancement, left one – CT after IV enhancement, arterial phase. Jejunal loops with thickened walls are indicated with arrows.

Fig.4 Abdominal CT, frontal reformation after IV enhancement, arterial phase. Jejunal loops with thickened walls are indicated with arrows.

TTE followed by TEE were performed and revealed vegetation on right coronary and non-coronary aortic valve cusps.

Three blood samples were sent for culture, and empiric therapy with intravenous antibiotics was initiated.

On day 5 IFA  on HEp-2 cell culture showed the presence of antinuclear antibodies in titer of 1:5120, with a homogeneous fluorescence pattern along with low level of C3 and C4 complement components, and positive anti-dsDNA antibodies. As a result, treatment with intravenous methylprednisone 2 mg/kg/day was initiated on day 5.

There was some improvement in patient’s dyspnea with increase in oxygen saturation and decrease of HR and RR. However, the patient continued to have intermittent gastric bleeding, requiring daily transfusions of packed red blood cells, fresh frozen plasma and platelets.

On day 7 the patient developed rapidly progressive respiratory failure requiring invasive lung ventilation. Antibacterial treatment was adjusted for the result of BAL fluid culture, and the dose of methylprednisolone was increased to 1000 mg/day. Despite treatment the patient’s respiratory function continued to deteriorate. He developed severe hypoxia, hypercapnia and respiratory acidosis and died 30 hours after initiation of mechanical ventilation.

The autopsy revealed aseptic verrucous endocarditis of aortic valve, local myocardial scarring without any evidence of coronary artery disease, and non-occlusive thrombosis of common ileac artery, markedly edematous intestinal wall with atrophic mucosa, and pneumonia with subtotal involvement of both lungs and signs of destruction.  Periarteriolar fibrosis ("onion skinning") in the spleen was the only found feature of vasculitis.

Discussion

SLE is a chronic autoimmune disease of unknown cause that can affect virtually any organ of the body. Although it can follow a relatively benign clinical course, in minority of patients it characterized by rapid progression with fulminant involvement of many organs. For such patients, delay in diagnosis even for a few days can have catastrophic consequences. At the same time, symptoms of the disease can vary greatly, requiring a high level of clinical suspicion from physicians.

The patient did not have established diagnosis on admission, and presentation of the disease was not quite typical. However, retrospectively we can propose our patient had been suffering from SLE for at least six month before presentation. We hypothesize that seizures could be the first sign of the disease reflecting repeated microembolic cerebral events. First of all, the patient suffered from secondary antiphospholipid antibodies syndrome and Libman-Sacks endocarditis, and had a potential source of emboli. Secondary, there were evidence of previous embolic event on the autopsy such as iliac artery thrombosis and myocardial scarring.

Repeated gastric hemorrhage without obvious eliminable source was a big challenge in this case, since bleeding recurred despite adequate gastroprotection and even after initiation of treatment with methylprednisone. 

It is difficult to tell whether or not an acute lupus pneumonitis preceded patient’s bacterial pneumonia, even with available autopsy result. However we see this scenario as quite possible, considering extreme disease activity and multiple organ involvement in our patient. On the other hand severe lymphopenia presented a big risk factor for major nosocomial infection in our patient. 

Conclusion

Moreover, for the best of our knowledge this is the first case to be reported of lupus flare presented as combination of SIADH and hemorrhagic gastroenteritis. Both SIADH and enteritis are rare but potentially dangerous manifestation of lupus. Physician alertness of this possibility will prompt diagnosis and can be crucial for early initiation of potentially life-saving treatment.

Consent

Written informed consent was obtained from the patient’s spouse for publication of this case report and any accompanying images. A copy of the written consent is available by the Editor-in-Chief of this journal.

About the authors

Валентин Евгеньевич Синицын

Author for correspondence.
Email: vsini@mail.ru
ORCID iD: 0000-0002-5649-2193

MD, Professor, Doctor of Medical Sciences, Head of Radiology Department in the University Hospital

Kira Dalgatova

Email: kira_1975@mail.ru
ORCID iD: 0009-0007-3327-009X

References

Supplementary files