Table 1. Summary of previous studies.

Observational studies

Derk [11]

Retrospective; 2947 hospitalizations due to SLE between 1982 and 2002

5 cases of pancreatitis (0.85%)

Pascual-Ramos [7]

Retrospective case-control design - patients with SLE and pancreatitis between 1984 -2001

35 identified patients; In 26 acute pancreatitis was an isolated event, recurrent in 9 patients

Vergara-Fernandez [19]

Prospective; 11 years of follow-up - 73 SLE patients with abdominal pain

21 patients had pancreatitis: 6 caused by gallstones, 5 by drugs, 4 associated to SLE, 4 unknown, 2 alcohol

Campos [20]

Retrospective; 263 patients with juvenile SLE

11 patients with acute pancreatitis, 1 with recurrent episodes. Association of pancreatitis with disease activity and hemophagocytic syndrome

Chang [21]

Retrospective cohort of pancreatitis in patients under 18 years between1993-2008

7 patients with SLE, 4 died

Makol [16]

Prospective cohort of 1811 SLE patients

76 patients with pancreatitis, in which this was attributable to SLE in 63. Association with hypertriglyceridemia, psychosis, pleurisy, anemia. No association with antiphospholipid syndrome

Xu [22]

Retrospective. Cohort of 177 patients with SLE

3 patients with pancreatitis

Dhir [10]

Retrospective; 550 patients with SLE

10 patients with pancreatitis, the majority in the beginning of disease (less than 1.5ys of SLE)

Proca [23]

Retrospective

Cohort of patients submitted to pancreatic resection due to chronic pancreatitis

One of 44 patients had diagnosis of SLE

Wang [12]

Retrospective. 2976 SLE patients. Between 1991-2005

48 episodes of pancreatitis in 40 patients (13 children and 27 adults). Children more frequent than adults; predominantly females; 2 patients as initial manifestation of SLE; adults with bigger survival than children

Tu [24]

Retrospective, SLE patients with abdominal pain

5 pancreatitis cases in 23 children; 14 pancreatitis cases in 88 adults

Dhaou [25]

Retrospective, monocentric

 

Pancreatitis present in 6 of 110 patients. In 4, it was the initial manifestation of SLE

Goel [6]

Retrospective. Cohort of 551 SLE patients

11 patients with acute pancreatitis, all in the first 12 months of disease

Yang [8]

Retrospective; Follow-up of 4053 SLE patients between 2000-2012

27 patients had acute pancreatitis. Pancreatitis was associated to high SLEDAI, multi-organ involvement, high mortality. 12 patients got recovery with GC

Yuan [2]

Retrospective, cohort of 3823 SLE patients between 2002-11

23 patients with acute pancreatitis (same cohort of Yang 2012, patients not included in counting)

Case reports and case series

Wolman [26]

1 patient (25ys, female)

Pancreatitis as early manifestation of SLE

Yeh [27]

4 patients

All patients had anticardiolipin antibodies. At autopsy, all had thrombi in the pancreas

Hortas [28]

2 patients, one of them with diagnosed systemic sclerosis

Female, 61ys - 3 episodes of pancreatitis, with overture of clinical SLE just before the third

Lam [29]

1 patient (65ys, female)

Pancreatitis after 1 year of SLE, with death

Marum [30]

1 patient

Pancreatitis as early manifestation of SLE

Cutlan [31]

1 patient (21ys, female)

Pancreatitis and panniculitis as initial manifestations of SLE

Ramanan [32]

1 patient (14ys, female)

Positive anticardiolipin antibodies

Duncan [33]

1 patient

Initial manifestation of SLE

Fan [34]

1 patient (12ys, female)

Pancreatitis as early manifestation of SLE, good response to GC

Fantini [35]

1 patient (23ys, female)

Pancreatitis and hemolytic anemia during SLE flare. Invasive aspergillosis as a complication. Death after 17 days.

Penalva [36]

1 patient (14ys, female)

Calcifying chronic pancreatitis

Singh [37]

1 patient (24ys, female)

SLE and TTP onset after an acute pancreatitis episode. Treatment with GC and plasmapheresis, with full recovery

Swol-Ben [38]

1 patient (59ys, female)

Pancreatic pseudotumor with pancreatic fibrosis; antiphospholipid syndrome. Treatment with cyclosporine, immunoglobulin, GC

Izzedine [39]

1 patient (59ys, female)

One episode of acute pancreatitis preceding relapse of lupus nephritis. She presented pancreatic calcifications and pseudocysts

Wang [3]

1 patient (46ys, female)

Pancreatitis as initial manifestation of SLE; Successful treatment with somatostatin

Nesher [5]

3 patients

High mortality

Agoumi [40]

1 patient

Pancreatitis as initial manifestation of SLE

Ergas [41]

1 patient (male)

Relapsing pancreatitis responding to GC treatment

Kobayashi [42]

1 patient (37ys, female)

Pancreatitis and elevation of IgG4. Treatment with GC

Carducci [43]

2 patients (24 and 34ys, females)

Pancreatitis as initial manifestation of SLE. Treatment with GC and somatostatin

Gutierrez [44]

1 patient (26ys, female)

Calcifying chronic pancreatitis in 2 years of SLE onset

Tominaga [45]

1 patient (12ys, female)

Pancreatitis as initial manifestation of SLE. Treatment with plasmapheresis, methylprednisolone and cyclophosphamide, with full recovery

Myung [46]

1 patient (33ys, female)

Pancreatitis, pseudocyst and central nervous system vasculitis; Improved with GC, but had just after infeccion of pseudocyst, improved with local surgery

Rose [47]

1 patient (14ys, female)

Pancreatitis as initial manifestation of SLE. Treatment with methylprednisolone and oral GC

Vyas [48]

1 patient; Previous diagnosis of SLE and secondary antiphospholipd syndrome

Relapsing ischemic pancreatitis, resistant to CS and anticoagulation. Better results with plasmapheresis, GC, cyclophosphamide and anticoagulation

Cairoli [49]

1 patient (39ys, female)

Acute pancreatitis during a severe lupus flare; pseudocysts; death despite GC use

Campos [50]

2 patients

Female, 27ys: pancreatitis one year after diagnosis of SLE. Good response to GC. Female, 20ys: just after diagnosis of SLE, death by spontaneous rupture of pancreatic pseudocyst

Geraldino [51]

1 patient (29ys, female)

Pancreatitis as early manifestation of SLE (2 months), accompanied of parotiditis. Resolution with GC in high dosage

Ko [52]

1 patient

In the post-partum period, manifestations of SLE started, followed by acute pancreatitis

Al-Musawi [17]

1 patient with recurrent attacks of acute pancreatitis

Patient was treated with rituximab (antiCD20 monoclonal antibody), maintaining remission for 2 years

Hoorn [53]

1 patient (25ys, female)

Pancreatitis with pseudocyst. Treatment with GC, azathioprine, immunoglobulin, with recovery

Malavya [54]

1 patient (55ys, female), history of 6 ys of SLE

Treatment with cyclophosphamide and methylprednisolone

Linwattana [14]

3 patients (11ys, 7ys and 18ys, female)

Successful treatment with GC

Domingues-Pinilla [55]

1 patient (12ys, female)

Pancreatitis as early manifestation of SLE. Treatment with methylprednisolone and hydroxychloroquine

Nguyen [56]

1 patient (38ys, female)

Antiphospholipid syndrome, initial manifestation. Thrombotic microangyopathy in the pancreas and kidneys

Studies without full text available or text in languages other than English, French, Portuguese, Spanish or Italian

György [57] - 1 patient (52ys, female)- histology: nucleophagocytosis, leukophagocytosis, erytrhophagocytosis in the pancreas

Hamed [58] - 3 patients with SLE - 2 died of hemorrhagic pancreatitis and 1 had pseudocysts

Reynolds [59] - Retrospective - 20 patients out of 63 SLE patients had pancreatitis, 4 attributable to SLE, with recovering with GC

Ossi [60] - 1 patient - SLE with pancreatitis during flare, recovery with plasmapheresis and GC

Simons-Ling [61] - 1 patient (12ys, female) - pancreatitis,panniculitis and calcynosis

Zanen [62] - 1 patient (33ys, female) - pancreatitis during severe flare. Recovery with plasmapheresis and GC

Brujin [63] - 1 patient (16ys, female) - pancreatitis without histologic findings of autoimmune activation in pancreas

Giordano [64] - Pancreatitis as initial manifestation of SLE, treatment with methylprednisolone

Rupprecht [65] - 1 patient (17ys, female) - chronic pancreatits since her 11 years, then onset of SLE. Use of azathioprine, GC, plasmapheresis and cyclophosphamide

Eaker [66]- Pancreatitis as initial manifestation of SLE

Pereira [67] - Pancreatitis during flare of SLE, recovery with GC

Levy [68] - cohort of pancreatic patients with unfavorable outcomes of SLE- 1 patient with pancreatitis

Serrano-López [69] - autopsy of a patient with SLE - pancreatitis with vascular damage and intimal proliferation

Garcia-Consuegra [70] - 1 patient (15ys, female) - acute pancreatitis with psudocyst formation

Wang [71] - 1 patient (39ys, female) - elevated anticardiolipin. Postmortem examen with generalized thrombi formation. In the pancreas, chronic inflammation with thrombi in pancreatic arteries, without vasculitic changes

Borum [72] - 2 patients with chronic pancreatitis

Braun [73] - 1 patient (15ys, female) - mixed disease of the connective tissue - widespread vasculopathy with intima proliferation and minimal fibrosis

Huang [74] - 1 patient (13ys, female) - Pancreatitis as early manifestation of SLE. Treatment with GC

Huong [75] - 5 patients, 3 died

Kolk [76] - 1 patient (16ys, female) - autoimmune vasculitis

Leong [77] - 1 patient - good response to GC high doses

Saab [78] - Retrospective cohort - 8 patients with lupus pancreatitis in 10 years, with good response to GC

Tahara [79] - 1 patient (22ys, female) - Pancreatitis as early manifestation of SLE. Good response to GC

Hani [80] - 2 patients, both died

Saito [82] - 1 patient - Concomitant onset of SLE and pancreatitis. Treatment with GC

Abdallah [82] - 1 patient, SLE for 7 years - pancreatitis and hemofagocytic syndrome.

Ozenc [83] - 1 patient; pseudocyst

Perrin [84] - 1 patient (13ys, female) - Pancreatitis as initial manifestation of SLE. Treatment with mycophenolate mophetil and GC

Suzuki [85] - 1 patient with overlap of systemic sclerosis and SLE. Treatment with GC and plasmapheresis

Duval [86] - 1patient, 27ys, female. Pancreatitis as early manifestation of SLE      

Wang [87] - Retrospective, 28 autoimmune disease related pancreatitis - 20 SLE, 6 Sjögrens’s syndrome

Soyibo [88] - 1 patient (50ys, female)

Ghamarchehreh [18] - 1 patient (24ys, female), 2 episodes in the onset of SLE. Use of GC and rituximabe, evolution to death

Koga [89] - 1 patient (55ys, female) - pseudocysts with rupture

Medeiros [90] - retrospective; 136 patients, 3 with clinical pancreatitis, 7 with subclinical pancreatitis

Elgatni [91] - Pancreatitis and hemophagocytosis as initial manifestation of SLE

 

GC = glucocorticoids