Loin pain hematuria syndrome – A case report of a challenging diagnosis for clinicians

Hussam Haider; Haider Ali Omar; Javed Iqbal Wani; Shahid Aziz

doi:10.4103/KKUJHS.KKUJHS_14_20

Users Online: 682

CASE REPORT

Year : 2021 | Volume : 6 | Issue : 2 | Page : 107-109

Loin pain hematuria syndrome – A case report of a challenging diagnosis for clinicians

Hussam Haider¹, Haider Ali Omar², Javed Iqbal Wani², Shahid Aziz²
¹ Department of Medicine, Asir Central Hospital, Abha, Saudi Arabia
² Department of Medicine, College of Medicine, King Khalid University, Saudi Arabia

Date of Submission	26-Sep-2020
Date of Decision	14-Mar-2021
Date of Acceptance	30-Oct-2021
Date of Web Publication	12-Jan-2022

Correspondence Address:
Javed Iqbal Wani
College of Medicine, King Khalid University, Abha
Saudi Arabia

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/KKUJHS.KKUJHS_14_20

Abstract

Loin pain hematuria syndrome (LPHS) is a rare syndrome of loin pain associated with hematuria, which could be either gross or microscopic in nature. The condition causes severe discomfort for patients and can lead to many limitations in activities of daily life. As such, no primary kidney pathology has been associated with this syndrome which makes it a unique entity, to be diagnosed after exclusion of secondary causes of these symptoms. The symptom complex of Loin pain hematuria has been described classically presenting in young females in their third decade and is known to have spontaneous remissions in almost half of the patients, while as rest of the patients continue to have recurrent episodes of pain and hematuria. A high index of suspicion needs to be developed by treating physician in a patient with recurrent loin pain associated with/out hematuria. We present a patient with a similar clinical course where the diagnosis of LPHS was made on clinical grounds after excluding secondary causes of her symptoms.

Keywords: Haematuria, loin pain hematuria syndrome, loin pain

How to cite this article:
Haider H, Omar HA, Wani JI, Aziz S. Loin pain hematuria syndrome – A case report of a challenging diagnosis for clinicians. King Khalid Univ J Health Sci 2021;6:107-9

How to cite this URL:
Haider H, Omar HA, Wani JI, Aziz S. Loin pain hematuria syndrome – A case report of a challenging diagnosis for clinicians. King Khalid Univ J Health Sci [serial online] 2021 [cited 2022 Jun 26];6:107-9. Available from: https://www.kkujhs.org/text.asp?2021/6/2/107/335627

Introduction

We are presenting a case of recurrent loin pain associated with haematuria who had multiple admissions to hospital and was thoroughly investigated for any secondary causes of her symptoms and was finally diagnosed a rare syndrome known as Loin pain haematuria syndrome. The details shall be discussed here.

Case Report

A 27-year-old female presented with recurrent episodes of left loin pain associated with gross hematuria for the past 2 years. Her condition was not preceded by upper respiratory tract infection and was not related to menstruation. She did not have bleeding from any other site. She was evaluated by urologist and gynecologist during this period. A cystoscopy and MRI were done, and there was no evidence of urological or gynecological cause for her presentation. She has no symptoms related to cardiovascular or connective tissue diseases. There was no family history of renal diseases or similar symptoms.

Her physical examination was unremarkable, apart from low BMI (15 kg/m²) and mild tenderness in the left flank and suprapubic area.

Her investigations showed low hemoglobin (10.8 g/dl) and normal white blood counts and platelets and as well as normal coagulation profile. Her urinalysis showed 60–70 red blood cells/f but no proteinuria or any castes. Hemoglobin electrophoresis showed no evidence of sickle cell anemia. Workup for tuberculosis and bilharziasis and connective tissue diseases was unremarkable. Ultrasonography for kidneys, ureter and bladder was normal. Echocardiography was reported to be normal.

During the previous admission, cystoscopy showed hyperemia of the bladder, biopsy was taken, and histopathology was suggestive of chronic interstitial cystitis.

After exclusion of all causes of loin pain and hematuria, she was discharged on L-arginine, but after 3 months, she came back with the same problem.

The cystoscopy was repeated, and this time showed a normal study. Nutcracker syndrome was excluded by Computed tomography (CT) abdomen, [Figure 1] and CT angiography was done to rule out nutcracker syndrome, which was excluded.

Figure 1: Computed tomography scan of the abdomen from the patient showing normal size kidneys, no stones or masses

Click here to view

A renal biopsy [Figure 2] and [Figure 3] which was done without complications and histopathology was reported as normal. Hence, after exclusion of all causes of the above symptoms, she was diagnosed with loin pain hematuria syndrome (LPHS).

Figure 2: Image showing normal renal biopsy on light microscopic examination

Click here to view

Figure 3: High-power microscopic examination of renal biopsy showing normal glomerulus

Click here to view

Discussion

Epidemiology

LPHS is a rare disorder with prevalence of approximately 0.012%.^[1],[2] The syndrome was reported more commonly in females; however, in the last decade, it has been also reported frequently in males as well with predominant age group from fifth to sixth decades. Our patient belongs to a younger category of 27 years. Although patients with LPHS have a typical presentation of recurrent loin pain and haematuria, which is mostly unilateral, however rarely they may become bilateral and occasionally the loin pain may be the only manifestation of this syndrome without any haematuria. The lack of clear understanding of pathophysiology delays its diagnosis and makes it difficult. Although some studies suggest an excellent long-term prognosis for loin pain hematuria syndrome, many will debate this argument.

Pathogenesis

In various studies, the disease has been postulated to have association with many underlying disorders such as use of oral contraceptive pills, association of psychiatric illness such as depression, somatization disorders, underlying infections, and previous history of calculary disease, but the relationship between LPHS and any underlying disorders has not been fully established.^[3],[4],[5]

The laboratory results have been reported normal in almost all cases of LPHS, as was in our patient. In one series, renal angiography has been reported to have changes in small vessels such as widening of bifurcating arteries, tortuosity of peripheral arteries, and some focal areas of avascularity.^[6] However most of other reports of renal angiography have been reported as normal.^[7]

In most studies, renal biopsies performed on LPHS have been described as normal. In some series, the pathogenesis of flank pain associated with LPHS is thought to be due to glomerular capillary hemorrhage resulting in tubular obstruction and interstitial edema. Over time, chronic edema is thought to result in capsular distention, pain, and further tubular obstruction and hemorrhage. A recent data from Spetie et al.^[8] recommended to have renal biopsy in all patients of suspected LPHS because in their study of 43 cases, 21% were diagnosed with IgA nephropathy, and in many patients, there were changes in glomerular basement membrane on electron microscopy. Our patient had undergone a renal biopsy which was reported as normal on light microcopy.

Many authors have postulated underlying coagulopathy with a variety of coagulation test abnormalities as a pathogenic mechanism and proposed aspirin and/or warfarin as a treatment modality.^[9]

Treatment

Medical treatment of LPHS has been variable in different reported series. In the past, low-dose aspirin/warfarin has been used based on the hypothesis of coagulopathy etiology; however, such modality of treatment has not stood the test of time.^[2] The main objective of treatment of LPHS is symptom relief with analgesics, especially NSAIDs and opioids combined with prolonged courses of antibiotics. Psychiatric evaluation combined with pain relief has been shown to improve the outcome.

Overall long-term prognosis of LPHS is excellent with no reports of deterioration of renal functions, and approximately 30% of patients have been reported to get spontaneous remissions over a period of 3–5 years,^[10] so a conservative rather than aggressive approach would be justified awaiting spontaneous remission.

Keeping in view the increased intraglomerular pressure, angiotensin converting enzyme inhibitors have shown a partial response in some cases.

More invasive interventions have been used in many studies, especially renal denervation, regional nerve blocks or transcutaneous electrical nerve stimulation have also shown some success in few patients. Nephrectomy has been used as a modality of treatment in refractory cases,^[11] but high complication rate has outweighed against such treatment modality. Renal autotransplantation has been used in many series with a good symptomatic relief; however, this approach has been advised to be kept limited for patients of refractory pain requiring high doses of opioid analgesics and failure of other nonsurgical modalities.^[10]

Conclusion

LPHS is a poorly understood disorder with many etiological hypotheses, so the diagnosis of this condition remains challenging and needs to be made by exclusion.

A high index of suspicion needs to be developed by the treating physician in a patient with recurrent loin pain associated with/out hematuria.

The management is even more challenging, and the patient should be managed conservatively to give a chance for spontaneous remission. More aggressive modalities should be reserved for refractory cases only.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1.	Tabataba Vakili S, Alam T, Sollinger H. Loin pain hematuria syndrome. Am J Kidney Dis 2014;64:460.
2.	Zubair AS, Salameh H, Erickson SB, Prieto M. Loin pain hematuria syndrome. Clin Kidney J 2016;9:128-34.
3.	Spitz A, Huffman JL, Mendez R. Autotransplantation as an effective therapy for the loin pain-hematuria syndrome: Case reports and a review of the literature. J Urol 1997;157:1554-9.
4.	Lucas PA, Leaker BR, Murphy M, Neild GH. Loin pain and haematuria syndrome: A somatoform disorder. QJM 1995;88:703-9.
5.	Lall R, Mailis A, Rapoport A. Hematuria-loin pain syndrome: Its existence as a discrete clinicopathological entity cannot be supported. Clin J Pain 1997;13:171-7.
6.	Weisberg LS, Bloom PB, Simmons RL, Viner ED. Loin pain haematuria syndrome. Am J Nephrol 1993; 13:229-37.
7.	Bergroth V, Konttinen YT, Nordstorm D, Laasonen L. Loin pain and haematuria syndrome – Possible association with internal arterial spasms. Br Med J (Clin Res Ed) 1987;294:1657.
8.	Spetie DN, Nadasdy T, Nadasdy G, Agarwal G, Mauer M, Agarwal AK, et al. Proposed pathogenesis of idiopathic loin pain-hematuria syndrome. Am J Kidney Dis 2006;47:419-27.
9.	Siegler RL, Brewer ED, Hammod E. Platelet activation and prostacyclin supporting capacity in the loin pain and hematuria syndrome. Am J Kidney Dis 1988;12:156-60.
10.	Sheil A, Chui A, Verran D, Boulas J, Ibels LS. Evaluation of loin pain hematuria syndrome treated by renal autotransplantation or radical nephrectomy. Am J Kidney Dis 1998;32:215-20.
11.	Gibson P, Winney RJ, Masterton G, Fowles RG. Bilateral nephrectomy and haemodialysis for treatment of severe loin pain hematuria syndrome. Nephrol Dial Transplant 1994;9:1640-1.