Skip to main content

Main menu

  • Home
  • Current issue
  • ERJ Early View
  • Past issues
  • ERS Guidelines
  • Authors/reviewers
    • Instructions for authors
    • Submit a manuscript
    • Open access
    • Peer reviewer login
    • WoS Reviewer Recognition Service
  • Alerts
  • Subscriptions
  • ERS Publications
    • European Respiratory Journal
    • ERJ Open Research
    • European Respiratory Review
    • Breathe
    • ERS Books
    • ERS publications home

User menu

  • Log in
  • Subscribe
  • Contact Us
  • My Cart

Search

  • Advanced search
  • ERS Publications
    • European Respiratory Journal
    • ERJ Open Research
    • European Respiratory Review
    • Breathe
    • ERS Books
    • ERS publications home

Login

European Respiratory Society

Advanced Search

  • Home
  • Current issue
  • ERJ Early View
  • Past issues
  • ERS Guidelines
  • Authors/reviewers
    • Instructions for authors
    • Submit a manuscript
    • Open access
    • Peer reviewer login
    • WoS Reviewer Recognition Service
  • Alerts
  • Subscriptions

A “respiratory” cause of abdominal pain

D. Gupta, R. Agarwal, A. Singh, K. Joshi
European Respiratory Journal 2006 27: 430-433; DOI: 10.1183/09031936.06.00074105
D. Gupta
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
R. Agarwal
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
A. Singh
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
K. Joshi
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • Article
  • Figures & Data
  • Info & Metrics
  • PDF
Loading

CASE HISTORY

A 52-yr-old female presented with complaints of upper abdominal pain, nausea and vomiting. There was no history of prior abdominal pain, abdominal distension and constipation. In June 2003, the patient had presented to her general practitioner with pain and swelling in multiple joints, both small and large. She was diagnosed to have seronegative rheumatoid arthritis and had received diclofenac, vitamin D, calcium supplements and methotrexate (7.5 mg p.o.·week−1) for the same. She had partial response, and methotrexate was stopped for the last 6 months of treatment up until June 2005; however she was continued on diclofenac and calcium supplements.

On examination the patient was conscious and afebrile with a pulse rate of 96 beats·min−1, blood pressure of 130/84 mmHg and respiratory rate of 18 breaths·min−1. Examination of the abdomen showed epigastric tenderness and normal bowel sounds. Otherwise, the physical examination was unremarkable. There were no joint deformities or joint tenderness.

A summary of investigations is shown in table 1⇓, and these included a chest radiograph (fig. 1⇓) and computed tomography of the chest (fig. 2⇓). Fibreoptic bronchoscopy was carried out, and transbronchial biopsies were performed which showed characteristic histological findings (fig. 3⇓).

Fig. 1—
  • Download figure
  • Open in new tab
  • Download powerpoint
Fig. 1—

Chest radiograph of the patient.

Fig. 2—
  • Download figure
  • Open in new tab
  • Download powerpoint
Fig. 2—

Computed tomography scan of the chest.

Fig. 3—
  • Download figure
  • Open in new tab
  • Download powerpoint
Fig. 3—

Photomicrograph of the transbronchial lung biopsy specimen (haematoxylin and eosin). Scale bar = 50 µm.

View this table:
  • View inline
  • View popup
Table 1—

Summary of investigations

BEFORE TURNING THE PAGE, INTERPRET THE HISTORY, INVESTIGATIONS, CHEST RADIOGRAPH, COMPUTED TOMOGRAPHY SCAN AND BIOPSY AND SUGGEST A DIAGNOSIS

INTERPRETATION

The patient had severe hypercalcaemia, acute renal failure and raised amylase, suggesting a diagnosis of acute pancreatitis secondary to hypercalcaemia. The chest radiograph (fig. 2⇑) shows bilateral hilar lymphadenopathy. The computed tomography scan of the chest (fig. 3⇑) shows bilateral hilar and mediastinal lymphadenopathy. Transbronchial biopsy showed noncaseating granulomas (fig. 1⇑). A Ziehl–Neelsen stain for acid-fast bacilli was negative.

Diagnosis: Sarcoidosis with joints and lymph node involvement. Severe hypercalcaemia and hypercalciuria, secondary to sarcoidosis, and contributed to by calcium and vitamin D supplements. Acute renal failure, prerenal; secondary to hypercalcaemia, dehydration. Acute pancreatitis, secondary to hypercalcaemia and sarcoidosis.

TREATMENT AND CLINICAL COURSE

The patient was kept nil per mouth and given intravenous fluids and intravenous proton pump inhibitors. She was managed conservatively for acute pancreatitis and the renal failure improved with intravenous fluids. She was started on oral prednisolone (40 mg·day−1) and all her calcium supplements were stopped. With this, her calcium levels started decreasing and normalised in the next 3 days. She was discharged on oral prednisolone.

A repeat chest radiograph, computed tomography scan of the chest and calcium profile performed 2 months later were normal. The patient was taking prednisolone (15 mg·day−1) and will continue to take steroids to complete 1 yr of therapy; she is presently doing well.

DISCUSSION

Sarcoidosis is a disorder of unknown aetiology characterised by the presence of noncaseating granulomas. Although most patients present with respiratory, skin or ocular involvement, autopsy studies have shown that virtually any organ system in the body can be affected, including joints, kidney, liver, gastrointestinal, heart and nervous system 1. Pulmonary involvement in the form of hilar lymphadenopathy, parenchymal infiltrates and fibrosis occurs in more than 90% of patients, but less than half are symptomatic 2. The diagnosis is often delayed because of the nonspecific nature of the symptoms, as was seen in the current case 3. Hypercalcaemia has been variably reported to occur in 2–63% of patients 4, and pancreatic involvement in 2.1% 4, although these are rarely the presenting manifestations.

Sarcoidosis should be considered as an aetiology in all patients presenting with hypercalcaemia, especially when common conditions have been excluded. Hypercalcaemia may fluctuate in patients with sarcoidosis, depending on the activity of the disease; moreover, hypercalciuria is three times more common than hypercalcaemia 5. Therefore, serum and urine calcium levels should be measured regularly over the whole course of the illness. The mechanism of hypercalcaemia in sarcoidosis is due to excess extrarenal calcitriol production. The alveolar macrophages present within the granulomas contain the enzyme 1 α-hydroxylase, which converts precursor vitamin D to active calcitriol 6. High levels of calcitriol normally cause feedback inhibition of 1 α-hydroxylase; likewise, calcitriol also causes upregulation of 25(OH)D3 24-hydroxylase (where D is vitamin D), which converts 25(OH)D3 to 24,25(OH)2D3, the metabolically inactive form of vitamin D. Both the feedback mechanisms are lost in alveolar macrophages, leading to continuous production of calcitriol 7. Another recently described mechanism is excess production of parathyroid hormone-related peptide 8, which, like parathyroid hormone, causes upregulation of 1 α-hydroxylase. Unlike parathyroid hormone, parathyroid hormone-related peptide is not regulated by calcium but by interleukin-2 and tumour necrosis factor-α, both of which are increased in sarcoidosis 9. It is possible that, when released by alveolar macrophages, these cytokines act in a paracrine fashion to upregulate parathyroid hormone-related peptide production by macrophages 7.

Pancreatic involvement by sarcoidosis is uncommon; in fact, gastrointestinal tract involvement by sarcoidosis is unusual, and the liver is the most frequently involved organ, followed by the stomach 10. Pancreatic sarcoidosis can present as acute pancreatitis, chronic pancreatitis, or nonspecific abdominal pain as a result of granulomatous involvement of the pancreas 11. Acute pancreatitis in sarcoidosis can be either because of active granulomatous pancreatitis or secondary to hypercalcaemia 12. Only eight cases of acute pancreatitis secondary to sarcoidosis have been reported in the literature, and three patients out of the eight had hypercalcaemia 5. Prompt resolution of pancreatitis occurs after treatment with glucocorticoids.

Prednisone is the drug of choice and causes a decrease in circulating calcitriol and serum calcium within 3–5 days. A decrease in urinary calcium excretion rate occurs in 7–10 days. Failure to normalise the serum calcium after 2 weeks should lead the clinician to exclude the possibility of a coexisting disorder, including hyperparathyroidism, myeloma, etc. Chloroquine and hydroxychloroquine can be attempted in patients who develop adverse effects with glucocorticoid therapy 13; however, the role of ketoconazole is not clear 4. Patients should avoid a high calcium diet, calcium supplementation and exposure to sunlight, as even normocalcaemic patients with sarcoidosis may develop hypercalcaemia, renal stones and renal failure 4.

In the current patient, the diagnosis of sarcoidosis was delayed for almost 2 yrs; this is not an uncommon occurrence 3. Moreover the institution of methotrexate also changed the clinical picture in this patient. Once methotrexate was withdrawn and calcium supplements were continued, the sarcoid activity flared up, with presentation as severe hypercalcaemia and pancreatitis.

In conclusion, the current case highlights the need for a high index of suspicion for this condition in patients who present with acute pancreatitis, as steroids, which are generally contraindicated in other forms of pancreatitis, are the treatment of choice. Thus, prompt recognition of this entity is of therapeutic significance.

  • Received June 25, 2005.
  • Accepted September 24, 2005.
  • © ERS Journals Ltd

References

  1. ↵
    Longscope WT, Freiman DG. A study of sarcoidosis. Medicine 1952;31:1–132.
    OpenUrlCrossRefPubMed
  2. ↵
    Hunninghake GW, Costabel U, Ando M, et al. The American Thoracic Society (ATS), the European Respiratory Society (ERS) and the World Association of Sarcoidosis and Other Granulomatous diseases (WASOG) statement on sarcoidosis. Am J Respir Crit Care Med 1999;160:736–755.
    OpenUrlCrossRefPubMedWeb of Science
  3. ↵
    Judson MA, Thompson BW, Rabin DL, et al. The diagnostic pathway to sarcoidosis. Chest 2003;123:406–412.
    OpenUrlCrossRefPubMedWeb of Science
  4. ↵
    Sharma OP. Hypercalcemia in granulomatous disorders: a clinical review. Curr Opin Pulm Med 2000;6:442–447.
    OpenUrlCrossRefPubMed
  5. ↵
    Siavelis HA, Herrmann ME, Aranha GV, Garcia G, Eubanks T, Reyes CV. Sarcoidosis and the pancreas. Surgery 1999;125:456–461.
    OpenUrlPubMedWeb of Science
  6. ↵
    Bell NH, Stern PH, Pantzer E, Sinha TK, De Luca HF. Evidence that increasing circulating 1 alpha, 25-dihydroxyvitamin D is the probable cause for abnormal calcium metabolism in sarcoidosis. J Clin Invest 1979;64:218–225.
  7. ↵
    Conron M, Young C, Beynon HLC. Calcium metabolism in sarcoidosis and its clinical implications. Rheumatology 2000;39:307–313.
    OpenUrlAbstract/FREE Full Text
  8. ↵
    Zeimer HJ, Greenaway TM, Slavin J. Parathyroid-hormone related peptide in sarcoidosis. Am J Med 1998;152:17–21.
  9. ↵
    Muller-Quernheim J. Sarcoidosis: immunopathogenic concepts and their clinical applications. Eur Respir J 1998;12:716–738.
    OpenUrlAbstract
  10. ↵
    Crystal RG. Sarcoidosis. In: Braunwald E, Fauci AS, Kasper DL, Hauser SL, Longo DL, Jameson JL, eds. Harrison's principles of internal medicine. 15th Edn. New York, McGraw Hill, 2001; pp. 1969–1973
  11. ↵
    Siavelis HA, Herrmann ME, Aranha GV, Garcia G, Eubanks T, Reyes CV. Sarcoidosis and the pancreas. Surgery 1999;125:456–461.
  12. ↵
    McCormick PA, Malone D, Fitzgerald MX, Fitzgerald O. Pancreatitis in sarcoidosis. BMJ 1985;290:1472–1473.
  13. ↵
    O'Leary TJ, Jones G, Yip A, Lohnes D, Cohanium M, Yendt ER. The effects of chloroquine on serum 1, 25-dihydroxyvitamin D and calcium metabolism in sarcoidosis. N Engl J Med 1986;315:727–730.
    OpenUrlPubMedWeb of Science
PreviousNext
Back to top
View this article with LENS
Vol 27 Issue 2 Table of Contents
  • Table of Contents
  • Index by author
Email

Thank you for your interest in spreading the word on European Respiratory Society .

NOTE: We only request your email address so that the person you are recommending the page to knows that you wanted them to see it, and that it is not junk mail. We do not capture any email address.

Enter multiple addresses on separate lines or separate them with commas.
A “respiratory” cause of abdominal pain
(Your Name) has sent you a message from European Respiratory Society
(Your Name) thought you would like to see the European Respiratory Society web site.
CAPTCHA
This question is for testing whether or not you are a human visitor and to prevent automated spam submissions.
Print
Citation Tools
A “respiratory” cause of abdominal pain
D. Gupta, R. Agarwal, A. Singh, K. Joshi
European Respiratory Journal Feb 2006, 27 (2) 430-433; DOI: 10.1183/09031936.06.00074105

Citation Manager Formats

  • BibTeX
  • Bookends
  • EasyBib
  • EndNote (tagged)
  • EndNote 8 (xml)
  • Medlars
  • Mendeley
  • Papers
  • RefWorks Tagged
  • Ref Manager
  • RIS
  • Zotero

Share
A “respiratory” cause of abdominal pain
D. Gupta, R. Agarwal, A. Singh, K. Joshi
European Respiratory Journal Feb 2006, 27 (2) 430-433; DOI: 10.1183/09031936.06.00074105
del.icio.us logo Digg logo Reddit logo Technorati logo Twitter logo CiteULike logo Connotea logo Facebook logo Google logo Mendeley logo
Full Text (PDF)

Jump To

  • Article
    • CASE HISTORY
    • INTERPRETATION
    • TREATMENT AND CLINICAL COURSE
    • DISCUSSION
    • References
  • Figures & Data
  • Info & Metrics
  • PDF
  • Tweet Widget
  • Facebook Like
  • Google Plus One

More in this TOC Section

  • A turbulent cause of bilateral pneumonia
  • An incidental finding in a 34-year-old male under investigation for haemoptysis
  • A 61-year-old female patient with right-sided pleuritic chest pain and fatigue
Show more Case for Diagnosis

Related Articles

Navigate

  • Home
  • Current issue
  • Archive

About the ERJ

  • Journal information
  • Editorial board
  • Press
  • Permissions and reprints
  • Advertising

The European Respiratory Society

  • Society home
  • myERS
  • Privacy policy
  • Accessibility

ERS publications

  • European Respiratory Journal
  • ERJ Open Research
  • European Respiratory Review
  • Breathe
  • ERS books online
  • ERS Bookshop

Help

  • Feedback

For authors

  • Instructions for authors
  • Publication ethics and malpractice
  • Submit a manuscript

For readers

  • Alerts
  • Subjects
  • Podcasts
  • RSS

Subscriptions

  • Accessing the ERS publications

Contact us

European Respiratory Society
442 Glossop Road
Sheffield S10 2PX
United Kingdom
Tel: +44 114 2672860
Email: journals@ersnet.org

ISSN

Print ISSN:  0903-1936
Online ISSN: 1399-3003

Copyright © 2023 by the European Respiratory Society