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PubMed This is a summary of 6 peer-reviewed journal articles Updated
Pediatrics · PAMI syndrome

Long-Term Monitoring and Complications

At a Glance

Long-term PAMI care uses individualized checks of blood counts, inflammation, zinc and calprotectin, liver and kidney function, growth, and warning symptoms. Kidney scarring and high blood pressure in the lungs can develop quietly, so improved symptoms or inflammation tests do not end monitoring.

Managing PAMI syndrome is a marathon, not a sprint. Because this condition is lifelong and the immune system remains naturally overactive, “feeling well” is not the only measure of health. Even when skin and joint symptoms are quiet, the body requires regular “under-the-hood” checks to monitor blood counts and organ health [1][2].

The Lifelong Lab Roster

There is no fixed schedule for blood work that fits every child, but doctors typically use a combination of tests to track disease activity and catch complications early [2].

  • Complete Blood Count (CBC): This is the most frequent test. Doctors look for persistent anemia (low red blood cells) and neutropenia (low white blood cells), which occur in nearly all PAMI patients [2][3].
  • Inflammatory Markers (CRP and ESR): These tests measure general inflammation in the body. While they often improve with treatment, they may not always tell the whole story about organ health [3][1].
  • Zinc and Calprotectin (MRP8/14): These are the “signature” markers of PAMI. Tracking their levels can help your team see how well a specific medication is working [4][2].
  • Liver Enzymes: Because the liver can become enlarged (hepatomegaly) and may be affected by both the disease and its treatments, regular checks of liver function are essential [2][1].

Monitoring the “Silent” Organs

One of the most important lessons from recent PAMI research is that some complications can develop quietly, without causing obvious pain or fever. However, screening tests for these conditions are highly individualized and triggered by symptoms or laboratory trends—they are not necessarily done routinely for every patient.

Kidney Health: Watching for FSGS

Recent case reports have shown that children with PAMI can develop kidney issues, such as Focal Segmental Glomerulosclerosis (FSGS), a condition where the kidney’s filtering units become scarred [1].

  • The Risk: Kidney damage can happen even if a child is taking powerful anti-inflammatory drugs like IL-1 inhibitors [1].
  • The Check: Periodic urinalysis (to check for blood or protein in the urine) and blood tests for creatinine (to check how well the kidneys filter) are vital parts of long-term care [1].

Lung Health: Pulmonary Arterial Hypertension (PAH)

Pulmonary hypertension (high blood pressure in the lung’s arteries) is a rare but very serious complication that has been reported in both children and teenagers with PAMI [5][3].

  • The Signs: Because it develops slowly, you should watch for “subtle” changes like your child getting out of breath more easily than their friends, fainting, or complaining of chest pain during play [5].
  • The Check: If these signs appear, your doctor may order an echocardiogram (an ultrasound of the heart) to check the pressure in the lung’s blood vessels [5][3].

Bone Marrow Health: Monitoring for MDS

Because the immune system is constantly “stressing” the bone marrow, there is a small risk that it could develop Myelodysplastic Syndrome (MDS), a condition where the marrow stops making enough healthy blood cells [6]. (The exact causal relationship is still being studied.)

  • The Check: A bone marrow biopsy is not a routine screening test. If a child’s blood counts (like platelets or red blood cells) begin to drop significantly or if the shape of the blood cells looks unusual under a microscope, a bone marrow biopsy may be ordered to ensure the marrow is still healthy [6][3].

Tracking Growth and Development

Inflammation is “expensive” for a child’s body—it uses up energy that should be going toward growth. Growth failure affects more than half of children with PAMI [2].

  • Plotting Progress: Your pediatrician should plot your child’s height and weight on a growth chart at every visit. A “flat” line (where a child stops gaining height or weight) is a major signal that the disease may not be as well-controlled as it seems [2][5].
  • Organ Size: Doctors will also continue to feel your child’s abdomen to check if the spleen or liver is staying the same size or growing, which can happen during periods of high inflammation [2].

Summary of Monitoring Needs

Area of Concern Primary Test Why it Matters
Blood Counts CBC with differential Watches for infection risk (neutropenia) and anemia [2].
Kidney Urinalysis & Creatinine Screens for silent scarring like FSGS [1].
Lungs/Heart Symptom review & Echo Checks for high blood pressure in the lungs (PAH) [5].
Bone Marrow CBC trends & Marrow Biopsy Watches for signs of MDS [6].
Systemic Health Growth Charting Ensures inflammation isn’t stalling development [2].

Common questions in this guide

Which blood tests are used to follow PAMI syndrome over time?
A complete blood count, or CBC, is used to watch for anemia and neutropenia, which means a low level of certain white blood cells. Doctors may also check CRP and ESR, zinc and calprotectin (MRP8/14), and liver enzymes. The timing depends on the child’s disease activity, treatment, and overall health.
How are kidney problems, including FSGS, checked in a child with PAMI?
The care team may use a urinalysis to look for blood or protein and a creatinine blood test to assess kidney filtering. A urine protein-to-creatinine ratio may also be useful. Testing is individualized and may be increased when symptoms or laboratory trends raise concern.
What signs of pulmonary hypertension should parents watch for?
New or worsening shortness of breath during play, fainting, or chest pain with activity should be reported to the child’s doctor. These symptoms can have many causes, but the doctor may order an echocardiogram, an ultrasound of the heart, to check pressure in the lung arteries.
Does every child with PAMI need a bone marrow biopsy?
No. A bone marrow biopsy is not routine screening; it may be considered if blood counts drop significantly or blood cells look unusual under a microscope. The test can help doctors evaluate the bone marrow and investigate concern about myelodysplastic syndrome.
How does PAMI monitoring assess growth and development?
Pediatricians can record height and weight on a growth chart at each visit and assess whether growth is slowing. Ongoing inflammation may contribute to growth failure, so a flat growth pattern can signal that the disease needs closer review.
Can PAMI complications develop even when skin and joint symptoms are controlled?
Yes. Kidney, lung, liver, or blood-related problems may develop with few obvious symptoms, and CRP or ESR results may not fully reflect organ health. The care team should tailor monitoring to the child’s symptoms, examination, and laboratory trends.
Why are zinc and calprotectin levels tracked in PAMI?
Zinc and calprotectin, also called MRP8/14, are signature markers associated with PAMI. Following their levels can help the care team assess how well a medication is working, but results are interpreted alongside other tests and clinical findings.

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References

References (6)
  1. 1

    Kidney Involvement in PSTPIP1 Associated Inflammatory Diseases (PAID): A Case Report and Review of the Literature.

    Borgia P, Papa R, D'Alessandro M, et al.

    Frontiers in medicine 2021; (8()):759092 doi:10.3389/fmed.2021.759092.

    PMID: 34778321
  2. 2

    PSTPIP1-Associated Myeloid-Related Proteinemia Inflammatory (PAMI) Syndrome: A Systematic Review.

    Mejbri M, Renella R, Candotti F, et al.

    Genes 2023; (14(8)) doi:10.3390/genes14081655.

    PMID: 37628706
  3. 3

    Clinical and genetic characteristics of PSTPIP1-associated myeloid-related proteinemia inflammatory syndrome.

    Zhang D, Su G, Liu Y, Lai J

    Pediatric rheumatology online journal 2021; (19(1)):151 doi:10.1186/s12969-021-00636-9.

    PMID: 34620178
  4. 4

    A Case Study of PSTPIP1-Associated Myeloid-Related Proteinemia Inflammatory Syndrome Masquerading as Inflammatory Bowel Disease.

    Rodsaward P, Kiattikunrat K, Tangnuntachai N, et al.

    Inflammatory intestinal diseases 2026; (11(1)):300-306 doi:10.1159/000553249.

    PMID: 42559525
  5. 5

    A very rare cause of pre-capillary pulmonary hypertension: The PAMI syndrome.

    Iseppi M, Savonitto G, Tommasini A, et al.

    Pulmonary circulation 2023; (13(4)):e12300 doi:10.1002/pul2.12300.

    PMID: 37876942
  6. 6

    HSCT is effective in patients with PSTPIP1-associated myeloid-related proteinemia inflammatory (PAMI) syndrome.

    Laberko A, Burlakov V, Maier S, et al.

    The Journal of allergy and clinical immunology 2021; (148(1)):250-255.e1 doi:10.1016/j.jaci.2020.11.043.

    PMID: 33338535

This page is for informational purposes only and does not constitute medical advice. Your child’s PAMI care team should set the monitoring schedule and decide when additional tests are needed.

Guide contents Hyperzincemia and hypercalprotectinemia
Research & Literature
05 Building Your PAMI Care Team