What is the appropriate investigation and management for a patient with a suspected Reynolds syndrome, who has a Mirena (levonorgestrel) IUD and is experiencing abdominal cramps?

Reynolds Syndrome Investigation and Management in a Patient with Mirena IUD and Abdominal Cramps

Critical First Step: Rule Out IUD-Related Complications Before Pursuing Reynolds Syndrome Workup

The abdominal cramps in this patient with a Mirena IUD require immediate investigation for IUD-related complications (displacement, perforation, infection, or pregnancy) before attributing symptoms to Reynolds syndrome, as IUD complications are far more common and potentially serious. 1, 2

Immediate Investigation Algorithm

Step 1: Pregnancy Test

• Perform urine pregnancy test immediately, regardless of bleeding patterns, as Mirena is highly effective but not 100% protective 1
• If positive, evaluate urgently for ectopic pregnancy 2

Step 2: Pelvic Examination

• Check for visible IUD strings to assess for displacement 1
• Perform bimanual examination to evaluate for tenderness suggesting pelvic inflammatory disease (PID) or perforation 1

Step 3: Pelvic Ultrasound

• Obtain pelvic ultrasound to locate the IUD and evaluate for displacement, perforation, or new pathologic conditions (fibroids, polyps, endometrial pathology) 1
• If strings are not visible, ultrasound is essential to locate the device 1

Step 4: STI Screening

• Screen for sexually transmitted infections, particularly if there is concern for PID 1, 2

IUD-Related Complications to Exclude

Displacement or Perforation

• Approximately 30% of women with uterine perforation are asymptomatic, while 71% present with mild symptoms including abnormal bleeding or abdominal pain 3
• IUD perforation or migration can cause pelvic/back pain and bleeding, requiring immediate referral for removal via hysteroscopy or laparoscopy 1
• During laparoscopy for perforation, filmy adhesions are found in 30% of patients 3

Pelvic Inflammatory Disease

• PID can present with pain and bleeding 1
• If PID is diagnosed, treat with antibiotics per CDC STD Treatment Guidelines without immediate IUD removal unless clinically indicated 1, 2
• Pelvic actinomycosis associated with IUD use, though rare, can manifest as mild PID symptoms 4

Malposition

• Hysteroscopy can reveal malpositioned IUDs causing symptoms like hemorrhagic discharge, pain, and dyspareugia 5
• Malpositioned IUDs can often be repositioned during hysteroscopy, avoiding unnecessary removal 5

Reynolds Syndrome Investigation (Only After Excluding IUD Complications)

Reynolds syndrome is the rare coexistence of progressive systemic sclerosis (scleroderma) and primary biliary cirrhosis 6. Investigation should include:

Clinical Features to Assess

• Raynaud's phenomenon: Paroxysmal blanching of fingers accompanied by local pain, followed by cyanotic discoloration—this is an early clinical symptom 6
• Skin changes: Obtain skin biopsies if scleroderma is suspected 6
• Hepatomegaly: Perform abdominal ultrasound to evaluate liver size 6

Laboratory Investigations

• Immunoserological profile for progressive systemic sclerosis 6
• Hepatic parameters: Alkaline phosphatase (AP) and gamma-glutamyl transpeptidase (GGTP) 6
• Anti-mitochondrial antibodies (AMA) with M2 specificity for primary biliary cirrhosis 6

Specialized Testing

• Capillary microscopy at proximal nail fold to detect blood vessel alterations 6
• Liver biopsy if primary biliary cirrhosis is suspected 6
• Esophageal evaluation for esophageal sclerosis 6

Management Approach

If IUD Complications Are Found

• Displacement/perforation: Refer for surgical removal 1
• PID: Treat with antibiotics per CDC guidelines; IUD removal not immediately necessary if patient desires continued contraception 1, 2
• Pregnancy: Remove IUD immediately if strings visible and intrauterine pregnancy confirmed 2

If No IUD Complications and Symptoms Persist

• Symptomatic management: Offer NSAIDs (naproxen 500-550 mg orally or ketorolac 20 mg orally with food) for 5-7 days during cramping episodes 2, 7
• Reassurance: Bleeding pattern changes do not correlate with contraceptive failure 8
• Consider removal: If symptoms remain unacceptable despite reassurance and management, discuss early removal with alternative contraception 8

If Reynolds Syndrome Is Confirmed

• Progressive systemic sclerosis: Treat with pentoxifylline and nifedipine to improve rheology, plus physiotherapy 6
• Primary biliary cirrhosis: Treat with ursodeoxycholic acid, which normalizes cholestatic parameters 6

Critical Clinical Pitfalls

• Do not attribute abdominal pain to Reynolds syndrome without first excluding IUD complications, as displacement, perforation, and infection are more common and require urgent intervention 1
• Do not immediately remove the IUD for PID—treat with antibiotics first and reassess 2
• Do not dismiss back pain as unrelated, as it may indicate displacement, perforation, or infection 1
• Do not assume the device is "wearing off" when pathology is more likely 1