Introduction
Methods
Iranian Primary Immunodeficiency Registry (IPIDR)
Clinical and Immunologic Diagnoses in COVID-19-Infected PID Patients
Genetic Analysis and Diagnoses in COVID-19-Infected PID Patients
Classification of COVID-19-Infected PID Patients
Results
Primary immunodeficiency categories | Total patients in the registry | Alive patients during the pandemic | Number of COVID-19 patients (%) | Monogenic defects of patients with COVID-19 | Mortality due to COVID-19, N (%) | Mortality rate due to COVID-19 |
---|---|---|---|---|---|---|
Combined immunodeficiencies | 1392 | 630 | 10 (1.5) | – | 6 (60.0) | 0.009 |
Non-syndromic combined immunodeficiencies | 576 | 247 | 6 (2.4) | – | 5 (83.3) | 0.020 |
Severe combined immunodeficiency | 355 | 113 | 5 (4.4) | – | 4 (80) | 0.035 |
Less profound combined immunodeficiencies | 221 | 134 | 1 (0.7) | STK4 | 1 (100) | 0.007 |
Syndromic combined immunodeficiencies | 816 | 383 | 4 (1.0) | – | 1(25) | 0.002 |
Wiskott-Aldrich syndrome | 74 | 59 | 1 (1.7) | WAS | – | – |
Ataxia-telangiectasia | 292 | 86 | 1 (1.1) | ATM | – | – |
Other syndromic combined immunodeficiencies | 450 | 238 | 2 (0.8) | DNMT3B (n = 2) | 1 (50) | 0.004 |
Predominantly antibody deficiencies | 1391 | 1002 | 4 (0.4) | – | – | – |
Agammaglobulinemia | 208 | 147 | 1 (0.6) | BTK | – | – |
Common variable immunodeficiency | 599 | 352 | 1 (0.2) | – | – | – |
Hyper immunoglobulin M syndrome | 102 | 86 | 1 (1.1) | – | – | – |
Selective IgA deficiency | 193 | 185 | 1 (0.1) | – | – | – |
Other antibody deficiencies | 285 | 232 | 0 | – | – | – |
Congenital defects of phagocytes | 782 | 426 | 2 (0.4) | – | – | – |
Chronic granulomatous disease | 385 | 217 | 2 (0.9) | CYBA (n = 1) | – | – |
Other phagocytosis defects | 397 | 209 | 0 | – | – | – |
Diseases of immune dysregulation | 117 | 90 | 2 (2.2) | – | 1 (50) | 0.011 |
Familial hemophagocytic lymphohistiocytosis | 44 | 37 | 1 (2.7) | RAB27A | 1 (100) | 0.027 |
Susceptibility to EBV and lymphoproliferation | 50 | 34 | 1 (2.9) | CD70 | – | – |
Other immune dysregulations | 23 | 19 | 0 | – | – | – |
Autoinflammatory disorders | 734 | 389 | 1 (0.2) | – | 1 (100) | 0.002 |
Non-inflammasome-related conditions | 45 | 40 | 1 | IL1RN | 1 (100) | 0.025 |
Other autoinflammatory disorders | 689 | 549 | 0 | – | – | – |
Other primary immunodeficiencies* | 302 | 217 | 0 | – | – | – |
Total | 4718 | 2754 | 19 (0.68) | – | 8 (42.1) | 0.003 |
Primary immunodeficiency categories | ID | PID diagnosis | Gender | Age of onset (m) | Age of PID diagnosis (m) | Infection | Autoimmunity | Lymphoproliferation | Other PID clinical manifestation | |
---|---|---|---|---|---|---|---|---|---|---|
Combined immunodeficiencies | Non-syndromic combined immunodeficiencies | P1 | SCID | F | 1 | 1 | URI, LRI | – | LAP | Sensitivity to light |
P2 | SCID | M | 6 | 20 | LRI | – | – | – | ||
P3 | SCID | M | 6 | 6 | LRI | – | – | – | ||
P4 | Omenn syndrome | M | 1.5 | 2.5 | BCGosis, LRI | – | LAP | Severe scaling erythematous skin lesions | ||
P5 | CID | M | 4 | 11 | LRI | AIT | LAP | – | ||
P6 | STK4 | M | 108 | 108 | Meningitis, cellulitis | ITP, AIHA | LAP | Seizure, neurological disorders | ||
Syndromic combined immunodeficiencies | P7 | WAS | M | 4 | 4 | LRI | – | – | Chronic diarrhea, microcytic thrombocytopenia | |
P8 | ATM | M | 24 | 108 | URI, LRI | – | – | Ataxic gait, telangiectasia | ||
P18 | DNMT3B | M | 10 | 17 | URI, LRI | – | LAP | Recurrent diarrhea | ||
P19 | DNMT3B | F | 15 | 28 | URI, LRI | – | – | Recurrent diarrhea, bronchiectasis | ||
Predominantly antibody deficiencies | P9 | BTK | M | 36 | 48 | Skin infection, URI, LRI | – | – | Urticaria, erythematous skin lesions | |
P10 | CVID | M | 12 | 240 | OME, LRI | ITP, AIHA, JIA | – | Recurrent diarrhea, bronchiectasis | ||
P11 | HIgM | F | 36 | 72 | OME, LRI, osteomyelitis | – | HSM | FTT, granulomatous inflammatory process in BM | ||
P12 | SIgAD | M | 6 | 84 | OME, LRI, recurrent oral herpes lesions | – | – | Bronchiectasis | ||
Congenital defects of phagocytes | P13 | CGD | F | 36 | 36 | LRI | – | – | FTT | |
P14 | CYBA | M | 1 | 120 | URI, LRI | – | Pulmonary granulomatosis lesion | Bronchiectasis severe pulmonary fibrosis | ||
Diseases of immune dysregulation | P15 | RAB27A | F | 60 | 106 | URI, LRI | AIHA | HSM | Albinism, gray hair, severe anal ulcer, hemophagocytic BM | |
P16 | CD70 | F | 84 | 108 | URI, LRI | Behcet’s disease, alopecia | HSM | HL | ||
Autoinflammatory disorders | P17 | IL1RN | F | 1 | 4 | Cellulitis, dental abscess, gingivitis | UC | HSM | Rash and skin lesions, edema in the right shoulder, chronic diarrhea, severe generalized erythroderma, ascites, anemia, femur swelling |
Primary immunodeficiency categories | ID | PID diagnosis | Age at COVID − 19 infection (m) | Clinical signs and complications after COVID-19 | Organ involvement | Treatment strategy | Medications | Outcome | |
---|---|---|---|---|---|---|---|---|---|
Combined immunodeficiencies | Non-syndromic combined immunodeficiencies | P1 | SCID | 10 | Fever, cough, drop of oxygen saturation, respiratory distress | Lung | Hospitalized, NICU, requiring O2/NIV | Azithromycin, IVIG | Death |
P2 | SCID | 20 | Drop of oxygen saturation | Lung | Hospitalized, NICU | Azithromycin, IVIG | Death | ||
P3 | SCID | 8 | Fever, faintness, respiratory distress | Lung | Hospitalized, NICU | Hydroxychloroquine, vancomycin, meropenem, IVIG | Death | ||
P4 | Omenn syndrome | 6 | Fever, tachypnea, vomiting heart failure, seizure(once), cardiomegaly, a drop of oxygen saturation, respiratory distress | Heart, Lung | Hospitalized, NICU | Isoniazid, rifampin, ethambutol, vitamin B6, cotrimoxazole | Death | ||
P5 | CID | 11 | Respiratory distress, CD4 lymphopenia | Lung | Hospitalized, NICU | Hydroxychloroquine, azithromycin, IVIG | Recovery | ||
P6 | STK4 | 144 | Fever, loss of appetite, jaundice, abdominal pain, bloody diarrhea, cardiac and pulmonary arrest | Gastrointestinal, lung, heart | Hospitalized, requiring O2/NIV | Acyclovir, ceftriaxone, vancomycin, dexamethasone | Death | ||
Syndromic combined immunodeficiencies | P7 | WAS | 5 | Fever, cough, respiratory distress | Lung | Hospitalized requiring O2/NIV | Meropenem. cotrimoxazole, vancomycin, azithromycin, IVIG | Recovery | |
P8 | ATM | 206 | Fever, diarrhea | Gastrointestinal | Hospitalized | Azithromycin, IVIG | Recovery | ||
P18 | DNMT3B | 130 | Fever, cough, respiratory distress | Lung | Hospitalized requiring O2/NIV | Azithromycin, IVIG | Recovery | ||
P19 | DNMT3B | 152 | Fever, dry coughs, loss of appetite, vomiting, seizure, loss of awareness, respiratory distress | Lung, gastrointestinal | Hospitalized, ICU | Azithromycin, ceftriaxone, vancomycin, IVIG | Death | ||
Predominantly antibody deficiencies | P9 | BTK | 430 | Dry cough, fever, sweating, abdominal pain, wheezing | Gastrointestinal, lung | Hospitalized, requiring O2/NIV | Azithromycin, IVIG | Recovery | |
P10 | CVID | 444 | Fever, dry cough, fatigue, shortness of breath, muscular pain, chest pain | Lung | Hospitalized | Hydroxychloroquine, azithromycin, meropenem, IVIG | Recovery | ||
P11 | HIgM | 72 | Fever, cough | Lung | Hospitalized, requiring O2/NIV | Hydroxychloroquine, azithromycin | Recovery | ||
P12 | SIgAD | 96 | Fever, mild clear rhinorrhea, mild suprasternal retraction, tachypnea, mild consolidation, productive cough, clear rhinorrhea | Lung | Hospitalized | Meropenem, clindamycin, hydroxychloroquine, IVIG | Recovery | ||
Congenital defects of phagocytes | P13 | CGD | 108 | Fever, cough, headache | Lung | Hospitalized, requiring O2/NIV | Cotrimoxazole, cefixime, meropenem, vancomycin | Recovery | |
P14 | CYBA | 216 | Respiratory distress, fatigue, dry cough, fever, headache, loss of sense of smell and hearing, eyelid edema, severe cardiac enlargement | Lung, olfactory and auditory, cardiovascular system | Hospitalized | Cotrimoxazole, hydroxychloroquine | Recovery | ||
Diseases of immune dysregulation | P15 | RAB27A | 106 | Fever, vomiting, HM, liver involvement and pitting edema, respiratory distress | Gastrointestinal | Hospitalized, ICU | Hydroxychloroquine, azithromycin | Death | |
P16 | CD70 | 372 | Fatigue, dry cough, sore throat | Lung | Hospitalized, requiring O2/NIV | Hydroxychloroquine, azithromycin, IVIG | Recovery | ||
Autoinflammatory disorders | P17 | IL1RN | 96 | Fever, dry coughs, loss of appetite, vomiting, seizure, loss of awareness, respiratory distress | Lung, gastrointestinal | Hospitalized, ICU | Hydroxychloroquine, azithromycin | Death |