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Thanking you
With sincere regards
Dr. Rajendra Kumar Ghritlaharey, M.S., M. Ch., FAIS
Associate Professor,
Department of Paediatric Surgery, Gandhi Medical College & Associated
Kamla Nehru & Hamidia Hospitals Bhopal, Madhya Pradesh 462 001 (India)
E-mail: drrajendrak1@rediffmail.com
On May 11,2011




Dr. Shankar P.R.

"On looking back through my Gmail archives after being requested by the journal to write a short editorial about my experiences of publishing with the Journal of Clinical and Diagnostic Research (JCDR), I came across an e-mail from Dr. Hemant Jain, Editor, in March 2007, which introduced the new electronic journal. The main features of the journal which were outlined in the e-mail were extensive author support, cash rewards, the peer review process, and other salient features of the journal.
Over a span of over four years, we (I and my colleagues) have published around 25 articles in the journal. In this editorial, I plan to briefly discuss my experiences of publishing with JCDR and the strengths of the journal and to finally address the areas for improvement.
My experiences of publishing with JCDR: Overall, my experiences of publishing withJCDR have been positive. The best point about the journal is that it responds to queries from the author. This may seem to be simple and not too much to ask for, but unfortunately, many journals in the subcontinent and from many developing countries do not respond or they respond with a long delay to the queries from the authors 1. The reasons could be many, including lack of optimal secretarial and other support. Another problem with many journals is the slowness of the review process. Editorial processing and peer review can take anywhere between a year to two years with some journals. Also, some journals do not keep the contributors informed about the progress of the review process. Due to the long review process, the articles can lose their relevance and topicality. A major benefit with JCDR is the timeliness and promptness of its response. In Dr Jain's e-mail which was sent to me in 2007, before the introduction of the Pre-publishing system, he had stated that he had received my submission and that he would get back to me within seven days and he did!
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KIST Medical College, P.O. Box 14142, Kathmandu, Nepal.
E-mail: ravi.dr.shankar@gmail.com
On April 2011
Anuradha

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Dr. Anuradha
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On Jan 2020

Important Notice

Case report
Year : 2021 | Month : July | Volume : 15 | Issue : 7 | Page : OD13 - OD15 Full Version

Sarcoidosis and Primary Biliary Cholangitis- An Overlap


Published: July 1, 2021 | DOI: https://doi.org/10.7860/JCDR/2021/49016.15101
Akshatha Manohar, CA Jayashankar, Nithin Kumar, Prakash Bhanu, Prathima Shivaji Rao

1. Junior Resident, Department of General Medicine, Vydehi Institute of Medical Sciences and Research Centre, Bengaluru, Karnataka, India. 2. Professor and Head, Department of General Medicine, Vydehi Institute of Medical Sciences and Research Centre, Bengaluru, Karnataka, India. 3. Senior Resident, Department of Gastroenterology, Vydehi Institute of Medical Sciences and Research Centre, Bengaluru, Karnataka, India. 4. Professor, Department of Dermatology, Vydehi Institute of Medical Sciences and Research Centre, Bengaluru, Karnataka, India. 5. Professor and Head, Department of Pathology, Vydehi Institute of Medical Sciences and Research Centre, Bengaluru, Karnataka, India.

Correspondence Address :
Dr. Akshatha Manohar,
501, Shankari Apartment, Ittamadu Road, Banashankari 3rd Stage,
Bengaluru, Karnataka, India.
E-mail: akshu1992@hotmail.com

Abstract

Primary Biliary Cholangitis (PBC) is a chronic progressive autoimmune cholestatic liver disease. Sarcoidosis is a multisystem chronic granulomatous disease. Both diseases are known to affect the liver causing granulomas. Sarcoidosis commonly involves the skin while PBC is associated with autoimmune skin disorders. Diagnosis of PBC requires biochemical, serological and histological confirmation. Steroids are used in the treatment of sarcoidosis. The role of steroids in the treatment of PBC is not completely established. In this case report, authors present the case of a 31-years-old female diagnosed as sarcoidosis based on granulomatous lesions in skin biopsy with concurrent PBC diagnosed on basis of serology and liver biopsy.

Keywords

Autoimmune liver disease, Chronic granulomatous disease, Liver granuloma, Skin plaque

Case Report

A 31-years-old female presented to the General Medicine Outpatient Department with a history of generalised non itchy hyperpigmentation of the skin with skin lesions over the nose and forehead since six months. She had intermittent low-grade fever, weight loss and breathlessness on exertion which was not associated with palpitations, chest pain or syncope since six months. She had hair loss since past six months. Patient also had a prior three years history of frontal headache associated with nausea and vomiting. There was no history of athralgia, skin tightening, Raynaud’s phenomenon, pain abdomen, jaundice, oliguria, haematuria or goitre. She had a past history of hypothyroidism and was on supplementation which was discontinued for unknown reasons one month before presentation. There was no other significant illness in the past in the patient or the family. There was no history of chronic drug intake or exposure to heavy metals or history of promiscuous behaviour.

On examination, the patient was poorly nourished. She had multiple well defined, erythematous infiltrative plaques over the right ala of nose and forehead, 2 cm above left supra orbital ridge and 6 cm above left supra orbital ridge. There were hyperpigmented patches over the extremities and trunk (Table/Fig 1). There was no peripheral nerve thickening.

Her pulse was 96 beats per minute in the right radial artery with a weak pulse in the left radial artery. Blood pressure measured in the right upper limb was 170/100 mmHg and the left upper limb was 160/100 mmHg and systolic blood pressure in both lower limbs was equal. She was afebrile and had grade 2 hypertrophied tonsils bilaterally. Fundus showed flame shaped haemorrhages with grade 1 hypertensive retinopathy. Anterior segment of the eye was normal. Cardiovascular system examination revealed a loud Pulmonic Valve Closure (P2) in the pulmonary area. Nervous system examination was normal.

On blood investigation, there was mild anaemia, elevated Erythrocyte Sedimentation Rate (ESR), mildly elevated serum creatinine, and albuminuria. Total protein was elevated with hyperglobulinemia. Liver function tests showed elevated alkaline phosphatase and gamma glutyamyl transferase (Table/Fig 2). Electrocardiography (ECG) and Echocardiogram (ECHO) showed left ventricular hypertrophy.

Antinuclear Antibody (ANA) titres were positive in 1:100 dilution in a perinuclear pattern and Anti Mitochondrial M2 Antibody (AMA-M2) was positive. Chest X-ray showed diffuse nodular opacities in bilateral lung fields. Ultrasound of abdomen showed normal sized liver with heterogenous and mildly course echotexture with normal portal venous architecture. Doppler of left upper limb showed reduced peak systolic flow in left radial artery. Montoux test was negative. Skin biopsy from the lesion over the forehead showed dense non caseating granulomatous infiltrates in the dermis extending into the subcutaneous fat. Granulomas contained epitheloid histiocytes with oval nuclei with variable Langhans giant cells. These findings were suggestive of sarcoidosis (Table/Fig 3).

Liver biopsy showed predominantly portal based inflammation with involving an occasional bile duct with ductular proliferation. There was moderate fibrosis (Ishak score 3-4/6, i.e., fibrous expansion of portal areas with some areas having occasional and some areas having marked portal to portal bridging as well as portal to central bridging) These features were suggestive of early stages of PBC (Table/Fig 4), (Table/Fig 5). Other conditions to be suspected with this histopathological picture are autoimmune cholangitis, autoimmune hepatitis, secondary biliary cholangitis, drug induced liver injury.

Patient was started on Tab.Ursodeoxycholic (UDCA) acid 600 mg once daily and prednisolone 40 mg/day and other supportive treatment such as Angiotensin-Converting Enzyme (ACE) inhibitors, anti hypertensives and steroid, glycolic acid and tretinoin ointment for local application. She was telephonically followed-up after three weeks and there was improvement in the lesions along with no further fever or weight loss. Follow-up was lost after that.

Discussion

Primary Biliary Cholangitis (PBC) is a chronic progressive autoimmune cholestatic liver disease leading to end stage liver disease. It presents more commonly in females in the fourth and fifth decade of life. It has a prevalence of around 12.9 per 100,000 populations (1). Fatigue and pruritus are said to be the most common dermatologic presentation of the disease (2). The first line of therapy includes ursodeoxycholic acid at 13-15 mg/kg. Licensed second line therapy includes obeticholic acid 5-10 mg/day while fibrates and budesonide are used off label (2).

Sarcoidosis is a chronic granulomatous disease affecting multiple organ systems. The prevalence of sarcoidosis in India is 61.2 per 100,000 new cases at a tertiary care center in New Delhi (3). Pulmonary involvement is most common followed by cutaneous manifestations such as erythema nodosum, lupus pernio and plaques (3). There is no specific diagnostic criteria for sarcoidosis and is considered as a diagnosis of exclusion. As ACE levels are non specific, histopathological examination of the site showing non caseating epitheloid cell granuloma is confirmatory (3). Corticosteroids are the first line of therapy while azathioprine and methotrexate act as second line agents for the treatment of sarcoidosis (4). Derangement in the cell mediated immunity plays a role in the pathogenesis of both the diseases hence both diseases may overlap in their presentation.

PBC can be diagnosed based on three criteria-biochemical abnormalities, serology and histology. It can be diagnosed when two of three criteria are abnormal (5). Elevation of Alkaline Phosphatase test (ALP) and GGT is the hallmark of biochemical abnormalities whereas Aspartate Transaminase/Alanine Aminotransferase (AST/ALT) ratio of more than one can be used a marker for liver fibrosis (2). Elevation of serum bilirubin levels occurs as the disease progresses. The hallmark immunological marker is AMA-M2 (E2 subunit) (1:40) positivity which is seen in more than 95% of patients with PBC. It has a sensitivity of about 90% and specificity of 98% (5). The most common immunofluorescence patterns for observed in PBC are ‘nuclear rim pattern’ and the ‘multiple nuclear dots’ pattern (6). On histology, the hallmark features are chronic, non suppurative cholangitis affecting interlobular and septal bile ducts causing ductopenia with infiltration of plasma cells, macrophages, mononuclear inflammatory cells and presence of epitheloid granulomas (7). PBC can be seen in association with extrahepatic conditions such Sjogren’s syndrome, Raynaud’s syndrome and autoimmune thyroid disease but association with sarcoidosis is rare (8). The skin diseases commonly associated are lichen planus, vitiligo and psoriasis (9).

There have been data of overlap of PBC with sarcoidosis following liver biopsy such as PBC with neurosarcoidosis and hepatic sarcoidosis where hepatic granulomas typical for sarcoidosis and PBC (10). A population based study showed statistically insignificant association between cases of PBC with hepatic sarcoidosis (11). This relationship may be attributed to a similar pathogenesis of the two diseases. In sarcoidosis, there is exaggerated immune cell activation by an antigenic stimulus causing macrophages to secrete Interleukin (IL)-1, IL-6 and IL-12 which stimulate Th1 cells. The accumulation of mononuclear cells is mediated by imbalances in the T cell response with an increase in Th1 and Th17 response and decrease in Treg response. The triggering antigens may be infectious or non infectious agents (12). Pathogenesis of PBC involves autoreactivity of B-cells and T-cells against the mitochondrial antigens namely the E2 component of the Pyruvate Dehydrogenase Complex (PDC) (13). On the contrary, there is published data regarding cholangitis mimicking primary billiary cirrhosis in a 52-year-old man with no actual morphological evidence of PBC (14).

Both the diseases are known to have hepatic granulomas. Hepatic granulomas occur in 11-65% of cases of sarcoidosis. They can be differentiated from the granulomas in PBC by being well defined and greater in number. The granulomas of sarcoidosis tend to coalesce in the portal and periportal area without loss of bile ducts. The Cluster of Differentiation 4 (CD4) cells accumulate in the centre of the granulomas in both the conditions whereas the CD8 cells tend to accumulate in the periphery of the granuloma in case of sarcoidosis and around the bile ducts in cases of PBC (15).

Present case report demonstrates that both sarcoidosis and PBC involves similar organ systems and can present in similar ways due to the similar pathogenesis. In a similar case study, a 69-year-old female who presented with pain abdomen, exhaustion and headache was found to have deranged liver function tests, positive AMA-M2, liver biopsy showing granulomatous lesions, elevated ACE levels and was started on treatment with UDCA and prednisolone (10). Hence, we must investigate for simultaneous presentations of these diseases when presented with hepatic and skin involvement. The treatment modalities also vary in both the diseases. The response of PBC to steroid therapy is not completely established (16).

Conclusion

Primary Biliary Cholangitis (PBC) and sarcoidosis are diseases with varied aetiology, similar pathogenesis and affects multiple organ systems. These two diseases can present simultaneously and requires to be managed individually. The distinction between the two diseases based on liver histology is challenging and needs further elucidation with a case series.

Declaration: This case report was presented in the Association of Physicians of India Conference in January 2020. https://pubmed.ncbi.nlm.nih.gov/31979772/.

References

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Primary biliary cirrhosis | India| PDF | PPT| Case Reports | Symptoms | Treatment [Internet]. [cited 2020 Dec 27]. Available from: https://www.omicsonline.org/india/primary-biliary-cirrhosis-peer-reviewed-pdf-ppt-articles/.
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European Association for the Study of the Liver. EASL Clinical Practice Guidelines: The diagnosis and managament of patients with primary biliary cholangitis. J Hepatol. 2017;67:145-72. [crossref] [PubMed]
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Sharma SK, Mohan A. Sarcoidosis in India: Not so rare. Journal, Indian Academy of Clinical Medicine. 2004;5(1):12-21.
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Rigopoulou EI, Davies ET, Pares A, Zachou K, Liaskos C, Bogdanos DP, et al. Prevalence and clinical significance of isotype specific antinuclear antibodies in primary biliary cirrhosis. Gut. 2005;54(4):528-32. [crossref] [PubMed]
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Lindor KD, Gershwin ME, Poupon R, Kaplan M, Bergasa NV, Heathcote EJ. Primary biliary cirrhosis. Hepatology. 2009;50(1):291-308. [crossref] [PubMed]
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Floreani A, Franceschet I, Cazzagon N, Spinazzè A, Buja A, Furlan P, et al. Extrahepatic Autoimmune Conditions Associated with Primary Biliary Cirrhosis. Clinic Rev Allerg Immunol. 2015;48(2):192-97. [crossref] [PubMed]
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Terziroli Beretta-Piccoli B, Guillod C, Marsteller I, Blum R, Mazzucchelli L, Mondino C, et al. Primary Biliary Cholangitis Associated with Skin Disorders: A Case Report and Review of the Literature. Arch Immunol Ther Exp (Warsz). 2017;65(4):299-309. [crossref] [PubMed]
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Alempijević T, Sokić-Milutinović A, Toncev L, Pavlović-Marković A, Djuranović S, Tomanović N, et al. Primary biliary cirrhosis and hepatic sarcoidosis-a case report. Vojnosanit Pregl. 2014;71(1):83-86. [crossref] [PubMed]
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Rajoriya N, Wotton CJ, Yeates DGR, Travis SPL, Goldacre MJ. Immune-mediated and chronic inflammatory disease in people with sarcoidosis: Disease associations in a large UK database. Postgrad Med J. 2009;85(1003):233-37. [crossref] [PubMed]
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Müller-Quernheim J, Prasse A, Zissel G. Pathogenesis of sarcoidosis. Presse Med. 2012;41(6 Pt 2):e275-87. [crossref] [PubMed]
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Kouroumalis E, Notas G. Pathogenesis of primary biliary cirrhosis: A unifying model. World J Gastroenterol. 2006;12(15):2320-27. [crossref] [PubMed]
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Selvan O, Vij M, Narasiman G, Venkatkrishnan L, Bharathan A, Rela M. Sarcoidosis mimicking primary billiary cirrhosis- A clinicopathological description. Tropical Gastroenterology. 2015;36(3):207-09. [crossref] [PubMed]
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Sherman S, Nieland NS, Van Thiel DH. Sarcoidosis and primary biliary cirrhosis. Coexistence in a single patient. Dig Dis Sci. 1988;33(3):368-74. [crossref] [PubMed]
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Dhanda A, Lee R, Collins PL. PMO-112 Is primary biliary cirrhosis a steroid sensitive autoimmune disease? Gut. 2012;61(Suppl 2):A118-18. [crossref]

DOI and Others

10.7860/JCDR/2021/49016:15101

Date of Submission: Feb 14, 2021
Date of Peer Review: Apr 29, 2021
Date of Acceptance: May 25, 2021
Date of Publishing: Jul 01, 2021

AUTHOR DECLARATION:
• Financial or Other Competing Interests: None
• Was informed consent obtained from the subjects involved in the study? Yes
• For any images presented appropriate consent has been obtained from the subjects. Yes

PLAGIARISM CHECKING METHODS:
• Plagiarism X-checker: Feb 15, 2021
• Manual Googling: Apr 29, 2021
• iThenticate Software: June 04, 2021 (4%)

ETYMOLOGY: Author Origin

JCDR is now Monthly and more widely Indexed .
  • Emerging Sources Citation Index (Web of Science, thomsonreuters)
  • Index Copernicus ICV 2017: 134.54
  • Academic Search Complete Database
  • Directory of Open Access Journals (DOAJ)
  • Embase
  • EBSCOhost
  • Google Scholar
  • HINARI Access to Research in Health Programme
  • Indian Science Abstracts (ISA)
  • Journal seek Database
  • Google
  • Popline (reproductive health literature)
  • www.omnimedicalsearch.com