Listing 14.07 Immune Deficiency Disorders Excluding HIV in 2026: How SSA Evaluates CVID, SCID, X-Linked Agammaglobulinemia, Hyper-IgM Syndrome, Chronic Granulomatous Disease, Complement Deficiencies, the Three-Infection Rule, and the Repeated-Manifestations Path
People with primary immune deficiencies live under a different rule set than the rest of us. A cold that would put you in bed for two days can hospitalize them for a week. Sinus infections turn into bronchiectasis. Skin infections turn into bacteremia. And the immune deficiency itself, whether antibody, complement, phagocyte, or combined, does not go away with treatment. It gets managed with lifelong replacement, prophylaxis, or transplant.
Listing 14.07 exists for exactly this. It is the listing for primary and secondary immune deficiencies that are not HIV. HIV goes to 14.11. Everything else that meaningfully impairs immune function comes here.
See If You Qualify
The exact text of Listing 14.07
14.07 Immune deficiency disorders, excluding HIV infection. As described in 14.00E1. With:
A. One or more of the following infections. The infection(s) must either be resistant to treatment or require hospitalization or intravenous treatment three or more times in a 12-month period.
1. Sepsis; or
2. Meningitis; or
3. Pneumonia; or
4. Septic arthritis; or
5. Endocarditis; or
6. Sinusitis documented by appropriate medically acceptable imaging.
OR
B. Stem cell transplantation as described under 14.00E2. Consider under a disability until at least 12 months from the date of transplantation. Thereafter, evaluate any residual impairment(s) under the criteria for the affected body system.
OR
C. Repeated manifestations of an immune deficiency disorder, with at least two of the constitutional symptoms or signs (severe fatigue, fever, malaise, or involuntary weight loss) and one of the following at the marked level:
1. Limitation of activities of daily living.
2. Limitation in maintaining social functioning.
3. Limitation in completing tasks in a timely manner due to deficiencies in concentration, persistence, or pace.
Three paths. Any one is enough. Path A is infection-based. Path B is transplant-based (automatic 12 months). Path C is repeated manifestations plus constitutional symptoms plus one marked functional limitation. Same structure as 14.02, 14.05, 14.06, 14.10, and other autoimmune listings.
Section 14.00E1: how SSA defines immune deficiency
Under 14.00E1, immune deficiency disorders include:
- Humoral (antibody) deficiencies. Common variable immunodeficiency (CVID), X-linked agammaglobulinemia (XLA/Bruton), hyper-IgM syndromes, IgG subclass deficiencies with clinical significance, specific antibody deficiency, transient hypogammaglobulinemia when persistent
- Combined immunodeficiencies. Severe combined immunodeficiency (SCID) all forms, Wiskott-Aldrich syndrome, ataxia-telangiectasia, DiGeorge/22q11.2 deletion syndrome, MHC class II deficiency, ZAP-70 deficiency, purine nucleoside phosphorylase deficiency
- Phagocyte defects. Chronic granulomatous disease (CGD), leukocyte adhesion deficiency, Chediak-Higashi syndrome, cyclic neutropenia, severe congenital neutropenia
- Complement deficiencies. C1q, C1r/s, C2, C3, C4, C5-C9 (membrane attack complex), MBL, ficolin deficiencies
- Innate immunity defects. IRAK-4, MyD88, NEMO, TLR3 pathway defects, IL-12/IFN-gamma axis defects
- Immune dysregulation syndromes. IPEX, ALPS, XLP, familial HLH, chronic mucocutaneous candidiasis
- Secondary immunodeficiencies. Post-transplant immunosuppression, post-chemotherapy, post-splenectomy, protein-losing enteropathies with immunoglobulin loss, nephrotic syndrome with immunoglobulin loss, chronic corticosteroid or biologic therapy for autoimmune disease
Paragraph A: the three-infection rule
Paragraph A requires either:
- An infection resistant to treatment, or
- Three or more hospitalizations or IV treatment courses for qualifying infections within a 12-month period
Only six infection types count under Paragraph A:
- Sepsis
- Meningitis
- Pneumonia
- Septic arthritis
- Endocarditis
- Sinusitis documented by imaging
Other infections (UTIs, cellulitis, wound infections, viral gastroenteritis) do not count under Paragraph A even if they are frequent. If your pattern is recurrent otitis media or cellulitis, Paragraph A does not apply. Paragraph C may still apply.
What counts as IV treatment
Under 14.00E, IV treatment includes hospitalized IV antibiotics, home IV therapy for at least 5 days, and outpatient IV infusions in an infusion center for the treatment of one of the six qualifying infections. Sinusitis is different in that imaging is required (CT sinuses showing acute or chronic sinusitis).
The 12-month window is rolling
Like other three-hospitalization rules, SSA uses a rolling 12-month window. Pick your best window. If your qualifying infections occurred in April, October, and January of the following year, that is under 12 months. If they occurred April, October, and July of the year after that, the April event has aged out of the current 12-month window and no longer counts toward the same set.
Paragraph B: stem cell transplantation
Any hematopoietic stem cell transplant for treatment of immune deficiency automatically qualifies the person for 12 months from the date of transplantation under 14.00E2. Common transplant indications in this space:
- SCID (all forms): standard of care in infancy or as soon as diagnosed
- Wiskott-Aldrich syndrome
- Hyper-IgM syndrome with CD40L deficiency
- Chronic granulomatous disease with severe phenotype
- Severe congenital neutropenia unresponsive to G-CSF
- IPEX syndrome
- Familial HLH
- Some cases of CVID with severe complications
Autologous gene therapy is now approved for some SCID forms (elivaldogene autotemcel, Skysona-adjacent products) and CGD. Gene therapy is evaluated under 14.00E2 the same way as allogeneic transplant.
After 12 months, any residual impairments (graft-versus-host disease, transplant-related lung disease, endocrine dysfunction, secondary malignancies) are evaluated under the affected body system's listings.
Paragraph C: repeated manifestations with marked functional limitation
Paragraph C is the catch-all for patients whose immune deficiency does not fit the infection pattern of Paragraph A but is disabling through chronic disease burden. Requirements:
- Repeated manifestations of the immune deficiency
- At least two constitutional symptoms (severe fatigue, fever, malaise, involuntary weight loss)
- One marked functional limitation from three domains (ADLs, social functioning, CPP)
Repeated manifestations under 14.00C4 can include:
- Recurrent infections that do not meet Paragraph A criteria (recurrent UTIs, otitis media, chronic sinusitis without CT documentation, cellulitis, opportunistic viral infections)
- Chronic diarrhea and malabsorption
- Bronchiectasis (which may also route through 3.07)
- Autoimmune manifestations (CVID with autoimmune cytopenias, thyroiditis, arthritis, enteropathy)
- Granulomatous disease in CVID (GLILD, hepatic granulomas)
- Lymphoproliferative complications
- Ongoing immunoglobulin replacement infusion reactions
Common variable immunodeficiency (CVID) deep dive
CVID is the most common symptomatic primary immunodeficiency in adults, affecting about 1 in 25,000 to 1 in 50,000. Diagnostic criteria under the 2019 ESID definition:
- Age 4 years or older at diagnosis
- IgG less than 2 standard deviations below age-adjusted mean
- At least one of: low IgA, low IgM
- Impaired vaccine response (poor response to pneumococcal polysaccharide, tetanus, or protein-conjugate vaccines)
- Exclusion of secondary causes
- Onset of infections or non-infectious complications
CVID is now understood as a spectrum with multiple genetic subtypes (TACI, ICOS, CD19, CD20, CD21, NFKB1, NFKB2, CTLA-4, LRBA, PIK3CD, PIK3R1). Treatment is lifelong immunoglobulin replacement (IVIG monthly or SCIG weekly) plus management of complications.
CVID complications that support Paragraph C:
- Bronchiectasis with chronic productive cough and recurrent exacerbations
- Granulomatous lymphocytic interstitial lung disease (GLILD)
- Enteropathy resembling celiac disease or IBD
- Autoimmune cytopenias (ITP, AIHA)
- Lymphoproliferative disease (MALT lymphoma risk increased)
Antibody replacement therapy and how SSA views it
IVIG or SCIG replacement does not disqualify you. Just as insulin does not disqualify a diabetic and factor VIII does not disqualify a hemophiliac, immunoglobulin replacement does not disqualify a CVID patient. SSA evaluates the impairment as it exists at baseline plus with treatment. If you still have Paragraph A infections, Paragraph B transplant history, or Paragraph C manifestations while on optimal IgG replacement, you meet the listing.
Standard IgG dosing in 2026 is 400 to 800 mg/kg per month IV every 3 to 4 weeks, or 100 to 200 mg/kg per week subcutaneously. Trough IgG levels above 700 mg/dL are the usual target, with some patients requiring 1000 mg/dL or higher to prevent infections. If your infections continue at target IgG trough, that supports 14.07 strongly.
Chronic granulomatous disease (CGD)
CGD is caused by defects in NADPH oxidase. Neutrophils cannot generate reactive oxygen species and cannot kill catalase-positive organisms (Staphylococcus aureus, Serratia marcescens, Burkholderia cepacia, Aspergillus, Nocardia). Manifestations include:
- Recurrent bacterial and fungal infections (skin, lymph nodes, lung, liver, bone)
- Granulomas that can obstruct the GI tract, urinary tract, or airways
- Colitis resembling Crohn disease
- Failure to thrive in children
Diagnosis is by dihydrorhodamine (DHR) flow cytometry or nitroblue tetrazolium (NBT) test. Treatment is lifelong TMP-SMX prophylaxis, itraconazole prophylaxis, and interferon-gamma injections three times weekly. Bone marrow transplant is potentially curative.
Complement deficiencies
Complement deficiencies produce distinct infection patterns:
- Early classical pathway (C1q, C1r/s, C2, C4): SLE-like syndromes, encapsulated bacteria infections
- C3: bacterial infections including sepsis
- Terminal complement (C5-C9): recurrent Neisseria infections (meningococcemia, gonococcal infections)
- Mannose-binding lectin (MBL): variable severity, often mild
- Alternate pathway (properdin, factor B, factor D): Neisseria infections
Meningococcal disease in a young adult without other explanation should trigger complement testing. Prophylactic meningococcal vaccination (MenACWY plus MenB) is standard.
Worked case 1: Priyanka, 33, Washington, CVID with GLILD
Priyanka was diagnosed with CVID in 2020 (IgG 340 mg/dL, IgA less than 7, IgM 22, absent response to pneumococcal vaccine). She has been on monthly IVIG 500 mg/kg with trough IgG 850 mg/dL. In a 12-month window she had:
- Community-acquired pneumonia with hospitalization: 6 days inpatient in September 2025
- CT-documented pansinusitis requiring IV ceftriaxone and clindamycin for 14 days: home IV January 2026
- Streptococcus pneumoniae bacteremia with sepsis: 8 days inpatient in May 2026
Three qualifying infections in 12 months, meeting Paragraph A pneumonia, sinusitis (CT documented), and sepsis criteria. Her attorney filed under Paragraph A. DDS approved at initial in July 2026.
Worked case 2: Ethan, 8 months, Minnesota, SCID post-transplant
Ethan was diagnosed with X-linked SCID at 2 weeks via newborn screening (TREC assay). He received a matched sibling donor bone marrow transplant at 6 weeks. His parents filed SSI for him at 3 months post-transplant. The attorney filed under Paragraph B (stem cell transplantation). SSI approved at initial with 12-month automatic disability from transplant date. Post-12-month evaluation for any residual impairments.
Worked case 3: Jordan, 27, Texas, CGD with Paragraph C
Jordan has X-linked CGD (DHR flow cytometry negative). He is on TMP-SMX, itraconazole, and interferon-gamma prophylaxis. His pattern is not three sepsis or pneumonia admissions per year, but he has:
- Chronic granulomatous colitis with intermittent hospitalizations for flares, chronic diarrhea, and weight loss
- Recurrent skin abscesses requiring outpatient I&D
- Chronic liver granulomas causing pain and requiring imaging follow-up
- Two Aspergillus pneumonias in prior years, now controlled
Constitutional: severe fatigue documented across 15 immunology visits, involuntary weight loss of 22 pounds over 14 months. Marked functional limitation: functional capacity evaluation showing inability to sustain 6-hour work days due to fatigue and GI symptoms (ADL marked limitation). His attorney filed under Paragraph C. DDS denied at initial. On reconsideration with additional immunology summary letter and FCE, allowance in November 2026.
Documentation checklist
- Immunology or allergy-immunology clinic records for at least 24 months
- Immunoglobulin panel (IgG with subclasses, IgA, IgM, IgE) with dates
- Vaccine response testing (pneumococcal polysaccharide, tetanus, diphtheria, Hib)
- T-cell and B-cell subsets by flow cytometry
- Complement testing (CH50, AH50, individual complement components as clinically indicated)
- DHR flow cytometry or NBT for suspected CGD
- TREC and KREC newborn screening results (for pediatric SCID cases)
- Genetic testing panel if available (primary immunodeficiency panel or WES)
- All hospital admissions with dates, LOS, discharge summaries
- CT sinus reports for sinusitis
- Blood cultures, wound cultures, respiratory cultures with organism identification and susceptibilities
- IgG trough levels on replacement therapy
- Bone marrow biopsy or transplant records if applicable
- Any autoimmune workup (autoimmune cytopenias, autoimmune enteropathy)
- Pulmonary imaging and PFTs for GLILD or bronchiectasis
- Functional assessment (FCE, neuropsych) for Paragraph C
Common denial reasons and how to counter them
"Infections not among the six qualifying types"
Counter: Paragraph A only counts six infection types. If your pattern is otitis, UTIs, or cellulitis, Paragraph A does not apply. Pivot to Paragraph C repeated manifestations with functional limitation, or fall back to RFC analysis.
"IgG replacement should have prevented infections"
Counter: submit IgG trough levels showing adequate replacement, and document infections that occurred despite adequate replacement. This is expected in CVID and does not disqualify you. Get an immunology letter stating that infections continue at target trough.
"Sinusitis not adequately documented"
Counter: CT sinus imaging is required under Paragraph A6. Get repeat CT sinus during any acute sinusitis episode, or archived CT sinus reports showing the disease pattern.
"Diagnosis not established under 14.00E1"
Counter: submit the immunoglobulin panel with values, vaccine response testing, flow cytometry, and genetic testing if available. Get an immunology summary letter identifying the specific immunodeficiency and citing 14.00E1.
What to do this week if you have immune deficiency and think you might qualify
- Pull your complete immunology records. Every immunoglobulin panel, every hospital admission, every infection culture, every CT scan.
- Get an immunology summary letter. Ask your immunologist to identify the specific immunodeficiency, list the qualifying infections (Paragraph A) or the repeated manifestations (Paragraph C), and cite the diagnostic criteria.
- File the strongest path you meet. If you have three qualifying infections in 12 months, Paragraph A is easier than Paragraph C. If you have a stem cell transplant, Paragraph B is automatic for 12 months.
Frequently asked questions
Does IgG replacement therapy disqualify me?
No. Immunoglobulin replacement is standard of care for CVID, XLA, hyper-IgM, and other antibody deficiencies. SSA evaluates the impairment with treatment. If you still have qualifying infections or repeated manifestations while on adequate IgG replacement, you meet 14.07.
What if my only infections are UTIs or cellulitis?
Paragraph A only counts six infection types. UTIs and cellulitis are not among them. However, they can support Paragraph C repeated manifestations if you also have constitutional symptoms and marked functional limitation. Alternatively, you can pursue RFC analysis at Step 5.
Does newborn screening for SCID via TREC assay establish the diagnosis?
TREC is a screening test. Confirmatory testing (T-cell, B-cell, and NK-cell flow cytometry, genetic testing for SCID subtype, ADA activity for ADA-SCID) establishes the diagnosis. Once confirmed, SCID qualifies for immediate transplant listing consideration and, post-transplant, Paragraph B automatic 12-month rule.
Can I qualify with just IgG subclass deficiency and normal total IgG?
Isolated IgG subclass deficiency is controversial. SSA does not have an explicit rule, but if you have documented IgG2 or IgG3 deficiency with clinical significance (impaired vaccine response, recurrent qualifying infections), it can be evaluated under 14.07 with an immunology summary letter tying the deficiency to the infection pattern.
Does gene therapy for immune deficiency count as stem cell transplantation?
Yes under 14.00E2. Autologous gene therapy (elivaldogene autotemcel for CALD, gene therapy for ADA-SCID, and gene therapy for CGD in trials) is evaluated the same as allogeneic transplant. The 12-month automatic disability period applies from the date of infusion.
Can chronic corticosteroid or biologic therapy for autoimmune disease meet 14.07?
Yes as secondary immunodeficiency. If your treatment has produced clinically significant immune suppression with recurrent qualifying infections, 14.07 can apply. Document the medication history, dosage, duration, and infection pattern with an immunology or rheumatology summary letter.
What is the difference between 14.07 and 14.11?
14.11 is specifically for HIV infection. 14.07 covers everything else: primary immunodeficiencies (CVID, SCID, XLA, hyper-IgM, CGD, complement, innate immunity, immune dysregulation) and secondary immunodeficiencies (post-transplant, post-chemotherapy, chronic immunosuppression). The infection criteria differ. 14.07 counts six specific infection types; 14.11 counts any complication of HIV.
See If You Qualify