Close relationship between autoimmune pancreatitis and multifocal fibrosclerosis

T Kamisawa; N Funata; Y Hayashi; K Tsuruta; A Okamoto; K Amemiya; N Egawa; H Nakajima

doi:10.1136/gut.52.5.683

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Pancreas

Close relationship between autoimmune pancreatitis and multifocal fibrosclerosis

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Author's reply
Terumi Kamisawa
Published on: 18 April 2016
Primary antiphospholipid syndrome as a new cause of autoimmune pancreatitis
Hal L Spencer
Published on: 18 April 2016
Response to steroid therapy of sclerosing cholangitis after duodenopancreatectomy due to autoimmune
David Padilla
Published on: 18 April 2016
Author's reply
Terumi Kamisawa
Published on: 18 April 2016
Interstitial pneumonia associated with autoimmune pancreatitis
Takao Taniguchi
Published on: 18 April 2016

Published on: 18 April 2016
Author's reply
- Terumi Kamisawa, Department of Internal Medicine
- Other Contributors:
  Nobuaki Funata and Atsutake Okamoto
Dear Author
Autoimmune pancreatitis is a recently described clinical entity, in which autoimmune mechanisms are involved in pathogenesis. As Etemand et al.[1] described that autoimmunity was one of six risk factors of chronic pancreatitis, autoimmune pancreatitis is not acute but chronic pancreatitis. Patients with autoimmune pancreatitis rarely showed acute attacks of pancreatitis or marked elevation of serum...
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Dear Author
Autoimmune pancreatitis is a recently described clinical entity, in which autoimmune mechanisms are involved in pathogenesis. As Etemand et al.[1] described that autoimmunity was one of six risk factors of chronic pancreatitis, autoimmune pancreatitis is not acute but chronic pancreatitis. Patients with autoimmune pancreatitis rarely showed acute attacks of pancreatitis or marked elevation of serum amylase .[2] Although the pancreas of autoimmune pancreatitis is swollen similar to acute pancreatitis On US and CT, it is induced by dense lymphoplasmacytic infiltration with fibrosis.
Obliterated phlebitis throughout the pancreas is the one of the characteristic pathological findings of autoimmune pancreatitis. The lumen of the vein was filled with prominent cellular infiltrates and fibrosis. Venous occlusion was due to not thromboembolism but phlebitis. Although the role of obliterated phlebitis is unknown in the pathogenesis of autoimmune pancreatitis, many IgG4-positive plasma cells, which might be closely related to pathogenesis, were observed in the obliterated veins. Signs of thrombosis were not observed in any organs of our patients with autoimmune pancreatitis. We think that autoimmune pancreatitis is quite different from pancreatitis reported by Spencer.
References
(1) Etemad B, Whitcomb DC. Chronic pancreatitis: diagnosis,classification, and new genetic developments. Gastroenterology 2001;120:682-707.
(2) Yoshida K, Toki F, Takeuchi T, et al. Chronic pancreatitis caused by an autoimmune abnormality. Proposal of the concept of autoimmune pancreatitis. Dig Dis Sci 1995;40:1561-1568.
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Conflict of Interest:
None declared.
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Published on: 18 April 2016
Primary antiphospholipid syndrome as a new cause of autoimmune pancreatitis
- Hal L Spencer, Gastroenterology Registrar
Dear Editor
I read with interest the recent article by Kamisawa et al. regarding the aetiology of autoimmune pancreatitis.[1] The cause of a significant proportion of cases of acute pancreatitis remains uncertain. I would like to describe a case of acute pancreatitis associated with antiphospholipid syndrome to highlight another potentially important cause of autoimmune pancreatitis which I believe has not...
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Dear Editor
I read with interest the recent article by Kamisawa et al. regarding the aetiology of autoimmune pancreatitis.[1] The cause of a significant proportion of cases of acute pancreatitis remains uncertain. I would like to describe a case of acute pancreatitis associated with antiphospholipid syndrome to highlight another potentially important cause of autoimmune pancreatitis which I believe has not been previously described.
Case report
A 30-year old woman was admitted twice in the space of three months with acute pancreatitis. She had a past medical history of anxiety and occasional migraines, for which she took alprazolam and propranolol respectively. She had had two miscarriages and one healthy child. She drank 3 units of alcohol per day. She was otherwise well and had no history of musculoskeletal problems.
On both occasions her amylase was significantly elevated (787 and 364 respectively, normal range 30-154). Ultrasound and CT of her abdomen were carried out each time and demonstrated a diffusely swollen pancreas consistent with acute pancreatitis, but with no evidence of gallstones or biliary duct dilatation.
Liver function tests were all normal with the exception of a slightly elevated gGT. Glucose, lipids, TSH, calcium and clotting (INR and APTT) were all normal. FBC was normal except for a neutrophilia during her acute illness. Her ESR was raised at 78. Urine microscopy and urinary protein excretion were both normal.
On her second admission to hospital she had 4 generalised seizures and MRI scanning showed cortical vein thrombosis with associated venous infarction. Subsequent investigation revealed a strongly positive ANA (1 in 640) but her ENA and dsDNA were negative as was her ANCA and her anti- mitochondrial and anti-smooth muscle antibodies. Her thrombophilia screen was negative but her IgG anti-cardiolipin antibodies were positive at 22.3 (0-5.5).
A diagnosis of antiphospholipid syndrome was made. She was treated with heparin and warfarin and subsequently made a good recovery.
Discussion
Primary antiphospholipid syndrome is defined as the presence of antiphospholipid antibodies (lupus anticoagulant or anticardiolipin antibodies) in association with thrombosis or recurrent miscarriage, but in the absence of an associated connective tissue disorder such as SLE.[2]
Systemic lupus erythematosis (SLE) has been previously described as an unusual cause of acute autoimmune pancreatitis. In the literature there are a small number of cases of pancreatitis in association with SLE and antiphospholipid syndrome.[3,4] A single case of pancreatitis associated with lupus anticoagulant but without anticardiolipin antibodies is also described.[5] However, to my knowledge this is the first reported case of primary antiphospholipid syndrome associated with anticardiolipin antibodies causing acute pancreatitis.
In the antiphospholipid syndrome vascular occlusion is due to thromboembolism, whereas in SLE the primary abnormality is a vasculitis. In a single post-mortem case of pancreatitis due to APS associated with SLE, the pathological abnormality was vascular occlusion due to thromboembolism.[2] Oral anticoagulation rather than steroids is therefore the treatment of choice for antiphospholipid syndrome.[6]
The patient described fulfils the criteria for a diagnosis of primary antiphospholipid syndrome. Whilst there is no histological proof that her pancreatitis was due to vaso-occlusive thromboembolism several facts make this the likely explanation. The recurrent episodes in the absence of another cause, the proven cerebral thromboembolism at the time of her second attack of pancreatitis and her positive anticardiolipin antibodies are highly suggestive that thromboembolism of her pancreatic blood vessels was indeed the cause of her pancreatitis.
I suggest that the investigation of patients with idiopathic pancreatitis should include checking their antiphospholipid antibodies.
References
(1) Kamisawa T, Funata N, Hayashi et al. Close relationship between autoimmune pancreatitis and multifocal fibrosclerosis. Gut 2003;52:683-687
(2) Wilson WA, Ghavari AE, Koike T et al. International consensus statement on preliminary classification criteria for definite antiphospholipid syndrome. Arthritis Rheum 1999;42:1309-11.
(3) Wang CR, Hseih HC, Lee GL et al. Pancreatitis associated to antiphospholipid antibody syndrome in patient with systemic lupus erythematosis. J Rheumatol 1992;19(7):1123-5.
(4) Yeh TS, Wang CR, Lee YT et al. Acute pancreatitis related to anticardiolipin antibodies in lupus patients visiting an emergency department. Am J Emerg Med 1993;11(3):230-2.
(5) Gaspari JC, Sande JR, Thomas CF Jr et al. Am J Gastroenterol 1995;90(5):825-6.
(6) Khamashta MA, Cuadrado MJ, Mujic F et al. The management of thrombosis in the antiphospholipid- antibody syndrome. N Engl J Med 1995;332:993-7.
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Conflict of Interest:
None declared.
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Published on: 18 April 2016
Response to steroid therapy of sclerosing cholangitis after duodenopancreatectomy due to autoimmune
- David Padilla, Digestive Surgeon of Department of Surgery,Complejo Hospitalario, Ciudad Real, Spain
- Other Contributors:
  Teófilo Cubo,Pedro Villarejo, Ricardo Pardo, Alberto Jara, Roberto de la Plaza, José Hernández
Dear Editor,
Autoimmune pancreatitis is a chronic inflammation of the pancreas due to etiopathogenic mechanisms of autoimmunity. There are no established definitive diagnostic criteria, although histological, analytical and radiological characteristics do enable us to identify this entity in the differential diagnosis with chronic alcoholic pancreatitis and cancer of the pancreas [1,2,3]. Nevertheless, this is no...
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Dear Editor,
Autoimmune pancreatitis is a chronic inflammation of the pancreas due to etiopathogenic mechanisms of autoimmunity. There are no established definitive diagnostic criteria, although histological, analytical and radiological characteristics do enable us to identify this entity in the differential diagnosis with chronic alcoholic pancreatitis and cancer of the pancreas [1,2,3]. Nevertheless, this is not always possible, and the patient undergoes surgery with suspected cancer of the pancreas. Lymphoplasmacytic infiltration and the autoimmune response do not only affect the pancreas, but can occasionally involve the retropancreatic and extrapancreatic biliary system. The relationship between the appearance of sclerosing cholangitis in patients with pancreatic pseudotumour due to autoimmune pancreatitis has even been considered the result of a systemic fibroinflammatory response [1,4].
We present the exceptional case of a patient who, after a cephalic duodenopancreatectomy due to pancreatic pseudotumour, in lymphoplasmocytic pancreatitis, presented with a clinical-radiological picture of post-surgical sclerosing cholangitis, which resolved after therapy with steroids. We read with interest the article by Kamisawa et al. [4] and as with them, we consider autoimmune pancreatitis a lesion more as part of a condition with multifocal fibrosclerosis and we believe that this sclerosing cholangitis is an additional manifestation of an autoimmune systemic condition, possibly stimulated by surgery.
A 78-year-old male patient was admitted to our service for obstructive jaundice of a few days history, not accompanied by constitutional syndrome. The patient had undergone surgery 75 days previously, with a pre-operative radiological diagnosis of suspected cancer of the head of the pancreas. A radical, pylorus-preserving cephalic duodenopancreatectomy was performed. The patient was discharged 12 days after the operation. The histopathology report of the resected sample revealed the presence of intense fibrosis and inflammatory, lymphoplasmacytic infiltration of the biliary wall with no evidence of malignity. Similarly, the pancreatic gland presented intense inflammatory, lymphoplasmacytic, glandular atrophy and no signs of malignity. Biochemical work-up on admission: BbT: 16.2 mg/dl. BbD:12.2 mg/dl. GGT:1264 IU/L. ALP:831 IU/L. CEA:2.81 ng/ml. CA 19.9> 500 IU/ml. Anti-IgM (HAV) (-); HbsAg (-); HbcAc (-); Anti-HCV (-); IgG: 1520 mg/dl; IgA: 445mg/dl; IgG4: 28mg/dl; IgM: 206 mg/dl. Abdominal echography: dilation of the intrahepatic biliary tract. Magnetic cholangioresonance: moderate dilation of the complete intrahepatic tract, with no visualization of the principal biliary tract and hilar plate, no anastomotic complications. Transparietohepatic angiography: dilation of the right intraheptic biliary tract. Diffuse stenosis affecting the common hepatic duct, hepatic hilum and segmented biliary branches. External-internal percutaneous drainage of the biliary tract was performed using radiology.
At two days, there was no obvious sign of improvement and the biochemical work-up was as follows: BbT:19.6 mg/dl; BbD:16.5 mg/dl; GGT:673 IU/l; ALP:648 IU/L; GOT:104 IU/L; GPT:111 IU/L. Exploratory laparotomy was performed with no pathological findings which justified cholestasis. Intra-operative echography only showed enlargement of the biliary wall and no intraluminal obstructive findings.Immediately after surgery, due to the suspicion of a basic inflammatory condition, treatment was begun with methylprednisolone, 1mg/kg/24 hrs, i.v. Once intake was tolerated, this treatment was maintained for a month p.o. before being reduced to 10 mg p.o./24 hrs., during the second month. The analytical follow-up was excellent, with BbT reduced to 1.8 mg/dl, and the remaining biological parameters were normal. Similarly, the episode of bicipital tenosynovitis of the left shoulder evolved satisfactorily. The patient maintained treatment with methylprednisolone, 10 mg p.o., for a further two months, with clinical-radiological and analytical resolution of the cholestatic process.
What is exceptional about this patient is the triggering of a severe autoimmune inflammatory response in the biliary system based on the presence of lymphoplasmacytic infiltration, coexistence with other autoimmune processes (episode of tenosynovitis in the shoulder of our patient), and good response to steroids that would reveal an autoimmune etiopathogenesis. Our group would include the possibility of exclusive biliary tract involvement, as was the case with our patient, after the stress of surgery. Taniguchi et al. [5] report the relapse of autoimmune pancreatitis after cephalic duodenopancreatectomy although they do not refer to alterations in the biliary tract. Toosi et al. [6] report the appearance in two of their patients of post-surgical sclerosing cholangitis, although only after biopsy of the pancreatic head.
The appearance of sclerosing cholangitis after duodenopancreatectomy has not been previously reported. The short period of biliary involvement and the progression maintained in the biliary involvement, led us to suspect an inflammatory process similar to that of autoimmune pancreatitis. Neither therapy nor its duration have been well defined and this can be seen in the different regimens used both for autoimmune pancreatitis and in autoimmune pancreato-cholangitis. Erkelens et al. [7] used prednisone at 0.5-1 mg/kg/day, followed by maintenance doses for six months. Some patients also received, albeit exceptionally, azathioprine at 50 mg/day, and this was used temporarily until resolution of the biliary endoprosthesis process. The results were satisfactory, although no therapeutic protocol has been defined. This disparity in criteria is manifested in other authors, such as Toosi et al. [6], who used ursodeoxycholic acid at 750 mg/24 hrs, with an almost complete return to a normal clinical and analytical picture. Other authors, such as Kojima et al. [8], maintain treatment according to the clinical-radiological changes, using a loading dose of 40 mg/24 hrs, with maintenance doses of 5 mg/24 hrs. Tamiguchi et al. [5] used prednisolone at 30 mg/24hrs for one month, followed by 5mg/24 hrs for nine months with satisfactory evolution. Kamisawa [9] et al., on the other hand, used a loading dose of prednisolone of 30-40 mg/24 hrs and maintenance doses of 5 mg/24 hrs until clinical check-up.
Based on the hypothesis of an excessive fibrosclerotic inflammatory response in our patient, we started therapy with prednisolone at 1 mg/kg for four weeks, with progressive reduction to 10 mg/24 hrs for the following four weeks. The maintenance dose was continued for a further two months, with analytical, radiological and clinical resolution of the process.
References
1- Laitt RD, Hubscher SG, Buckels JA, et al. Sclerosing cholangitis associated with multifocal fibrosis: a case report. Gut 1992;33:1430-1432.
2- Ectors N, Maillet B, Aerts R, et al. Non-alcoholic duct destructive chronic pancreatitis. Gut 1997;41:263-268.
3- Okazaki K, Chiba T. Autoimmune related pancreatitis. Gut 2002;51:1-4.
4- Kamisawa T, Funata N, Hayashi Y, et al. Close relationship between autoimmune pancreatitis and multifocal fibrosclerosis.Gut 2003;52:683-687.
6- Toosi MN, Heathcote J. Pancreatic pseudotumor with sclerosing pancreato -cholangitis: Is this a systemic disease? Am J Gastroenterol2004;99:377- 382.
5- Taniguchi T, Hikaru T, Seko S,et al. Autoimmune pancreatitis detected as a mass in the head of the pancreas without hypergammaglobulinemia, which relapsed after surgery. Dig Dis Sci 2003;48:1465-1471.
7- Erkelens GW, Vleggaar FP, Lesterhuis W, et al. Sclerosing pancreato- cholangitis responsive to steroid therapy. The Lancet 1999;354:43-44.
8- Kojima E, Kimura K, Noda Y,et al. Autoimmune pancreatitis and multiple bile duct strictures treated effectively with steroid. J Gastroenterol 2003;38:603-607.
9- Kamisawa T, Egawa N, Nakajima H, et al. Clinical difficulties in the differentiation of autoimmune pancreatitis and pancreatic carcinoma. Am J gastroenterol 2003;98:2694-2699.
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Conflict of Interest:
None declared.
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Published on: 18 April 2016
Author's reply
- Terumi Kamisawa, physician
- Other Contributors:
  Nobuaki Funata and Atsutake Okamoto
Dear Editor
We thank Dr Taniguchi et al. for his interesting presentation of interstitial pneumonia associated with autoimmune pancreatitis (AIP).
We have experienced 24 cases of AIP, however, no cases showed interstitial pneumonia clinically. Recently we immunohistochemically examined the organs of eight patients with AIP using anti-IgG4 antibody. Many IgG4-positive plasmacytic infiltration was dete...
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Dear Editor
We thank Dr Taniguchi et al. for his interesting presentation of interstitial pneumonia associated with autoimmune pancreatitis (AIP).
We have experienced 24 cases of AIP, however, no cases showed interstitial pneumonia clinically. Recently we immunohistochemically examined the organs of eight patients with AIP using anti-IgG4 antibody. Many IgG4-positive plasmacytic infiltration was detected in the portal area of the liver, gastric mucosa, colonic mucosa, and bone marrow as well as the pancreas, peripancreatic tissue, extrahepatic bile duct, gallbladder, salivary gland, and lymph nodes of patients with AIP. However, few IgG4-positive plasma cells were observed in identical control specimens. From these findings, we proposed a new clinicopathological entity of IgG4-related autoimmune disease, and stressed that AIP is not simply pancreatitis but that it is a pancreatic lesion involved in this systemic autoimmune disease.[2] As IgG4-positive plasmacytic infiltration was observed in the transbronchially biopsied pulmonary specimens of the patient with AIP (2), it is likely that interstitial pneumonia occurs in association with AIP.
References
(1) Taniguchi T et al. Interstitial pneumonia associated with autoimmune pancreatitis [electronic response to Kamisawa et al. Close relationship between autoimmune pancreatitis and multifocal fibrosclerosis] gutjnl.com 2003http://gut.bmjjournals.com/cgi/eletters/52/5/683#127
(2) Kamisawa T, Funata N, Hayashi Y et al. A new clinicopathological entity of IgG4-related autoimmune disease. J Gastroenterol 2003;38(10):982-984.
(3)Kamisawa T, Egawa N, Nakajima H. Autoimmune pancreatitis is a systemic autoimmune disease. Am J Gastroenterol 2003;98 (12):in print.
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Conflict of Interest:
None declared.
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Published on: 18 April 2016
Interstitial pneumonia associated with autoimmune pancreatitis
- Takao Taniguchi, MD, PhD
- Other Contributors:
  Mari Ko, Shuji Seko, Osamu Nishida , Fumihiko Inoue, Hisato Kobayashi, Tatsuo Fukuse , Tatsuoki Saiga, and Motozumi Okamoto
Dear Editor
We read with interest the article by Kamisawa and colleagues reporting IgG4-positive plasma cells in the peripancreatic tissue, extraphepatic bile duct, gall bladder, and salivary gland.[1] The association of retroperitoneal fibrosis and sclerosing pancreatitis with IgG4 -bearing plasma cells in the tissues of both lesions has been also reported.[2]
We would like to report the first case of...
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Dear Editor
We read with interest the article by Kamisawa and colleagues reporting IgG4-positive plasma cells in the peripancreatic tissue, extraphepatic bile duct, gall bladder, and salivary gland.[1] The association of retroperitoneal fibrosis and sclerosing pancreatitis with IgG4 -bearing plasma cells in the tissues of both lesions has been also reported.[2]
We would like to report the first case of interstitial pneumonia associated with autoimmune pancreatitis with IgG4-positive plasma cells in the intersitium.
Hyperamylasemia was detected in a routine blood examination in a 63 year-old man who had been treated for duodenal ulcer at a clinic. He was admitted to our hospital for further examination. He did not complain of epigastralgia or back pain. Serum amylase was 323 (39-130 IU/L), IgG was elevated to 2350 (800~1600mg/dl), and IgG4 was 1690 (<_80 mg="mg" dl.="dl." antinuclear="antinuclear" antibody="antibody" anti-ss-a="anti-ss-a" anti-ss-b="anti-ss-b" rheumatoid="rheumatoid" factor="factor" and="and" anti-smooth="anti-smooth" muscle="muscle" were="were" all="all" negative.="negative." abdominal="abdominal" ultrasonography="ultrasonography" computed="computed" tomography="tomography" ct="ct" showed="showed" swelling="swelling" of="of" the="the" head="head" tail="tail" pancreas.="pancreas." endoscopic="endoscopic" retrograde="retrograde" pancreatography="pancreatography" irregular="irregular" narrowing="narrowing" main="main" pancreatic="pancreatic" duct="duct" in="in" tail.="tail." magnetic="magnetic" resonance="resonance" cholangiography="cholangiography" extrinsic="extrinsic" stenosis="stenosis" lower="lower" common="common" bile="bile" duct.="duct." patient="patient" was="was" diagnosed="diagnosed" with="with" autoimmune="autoimmune" pancreatitis="pancreatitis" but="but" he="he" refused="refused" steroid="steroid" therapy="therapy" followed="followed" as="as" an="an" outpatient.="outpatient." p="p"/> Three months later, honeycombing of the bilateral lower lung field was detected in a follow-up abdominal CT. Chest CT revealed ground glass attenuation in the middle and lower lobe, and honeycombing predominantly at the back of the lower lobe, bilaterally (Fig 1A).

Figure 1A chest CT showing ground glass attenuation in the middle and lower lobe, and honeycombing predominantly at the back of the lower lobe bilaterally
Retrospectively, a slight reticular shadow in the lower lung field was detected in the chest roentogenogram taken at the first admission, but the lesion had progressed over 3 months. He was readmitted for further examination. He had a history of smoking 30 to 40 cigaretts a day for approximately 40 years. IgG was 3934 mg/dl, IgG4 was 2690 mg/dl, KL-6 was 1440 ( With Gallium scintigraphy, uptake was observed bilaterally at the back of the lower lobe, suggesting active pneumonia. Histology obtained by transbronchial lung biopsy from segment 8a of the right lobe showed marked thickening of the alveolar septum with marked infiltration of plasma cells and lymphocytes (Fig 1B).

Figure 1B histology of the biopsy specimen from the lung (H.E)
Immunostaining with IgG4 was performed using the imunoperoxidase method. (Mouse Anti-Human IgG4, ICN Biomedicals, Inc, Ohio, Canada). Infiltration of IgG4-positive plasma cells was detected in the alveolar septum (Fig 1C).

Figure 1C IgG4 staining of the biopsy specimen from the lung
Macrophages in the alveoli are considered due to smoking, which often co-exists with interstitial pneumonia in smokers.[3]
Because interstitial pneumonia associated with autoimmune pancreatitis was strongly suggested, prednisolone (40 mg/day) was administered for 2 weeks and then the dose was tapered. Chest CT taken 2 weeks ater treatment showed that the ground glass attenuation in the middle and lower lobe had disapeared, whereas the honeycombing remained (Fig 1D).

Figure 1D chest CT ater treatment
Abdominal ultrasonography performed 2 weeks after treatment showed a marked decrease in the swelling the pancreas.
In the present case, infiltration of IgG4-positive plasma cells in the interstitium strongly suggests that the interstitial lung disease was associated with autoimmune pancreatitis. Interstitial pneumonia asoociated with Sjogren's syndrome is unlikely in this case, although there was decreased lacrimal secretion. Sicca syndrome observed in autoimmune pancreatitis is distinctive from classical Sjogren's syndrome in that it is negative for anti-SS-A or anti-SS-B antibodies, serum IgG4 is elevated and infiltration of IgG4-positive plasma cells in the salivary glands is observed.[1,4]
Autoimmune pancreatitis, in some cases, might be part of a systemic disease associated with IgG4.
References
(1) Kamisawa T, Funata N, Hayashi Y et al. Close relationship between autoimmune pancreatitis and multifocal fibrosclerosis. Gut 2003;52:683-7.
(2) Hamano H, Kawa S, Ochi Y et al. Hydronephrosis associated with retroperitoneal fibrosis and sclerosing pancreatitis. Lancet 2002; 359:1403-04.
(3) American Thoracic Society: American Thoracic Society/European Respiratory Society International Multidisciplinary Concensus Classification of the Idiopathic Interstitial Pneumonias. Am J Respir Crit Care Med 2002;165:277-304.
(4) Hamano H, Kawa S, Horiuchi A et al. High serum concentration in patients with sclerosing pancreatitis. N Engl J Med 2001;344:732-738.
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Conflict of Interest:
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